Евразийские рекомендации по диагностике и лечению легочной гипертензии, ассоциированной с врожденными пороками сердца у взрослых (2021)

легочная гипертензия, легочная артериальная гипертензия, врожденный порок сердца, легочно-сосудистая болезнь, хирургическое лечение, атриосептостомия, специфическая терапия, клинические рекомендации

1. Чазова И.Е., Мартынюк Т.В., Валиева З.С. и соавт. Евразийские клинические рекомендации по диагностике и лечению легочной гипертензии. Евразийский кардиологический журнал. 2020; 1:78-122 https://doi.org/10.38109/2225-1685-2020-1-78-122

2. Baumgartner H., De Backer J., Babu-Naraya S.V., et al. 2020 ESC Guidelines for the management of adult congenital heart disease: The Task Force for the management of adult congenital heart disease of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Adult Congenital Heart Disease (ISACHD). Eur Heart J. 2021; Vol. 42 (6): 563–645. https://doi.org/10.1093/eurheartj/ehaa554

3. Frost A., Badesch D., Simon J.R., et al. Diagnosis of pulmonary hypertension. Eur Respir J. 2019; 53: 1801904. https://doi.org/10.1183/13993003.01904-2018

4. Galie N., Humbert M., Vachiery J.L., et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur. Heart J. 2016; 37: 67–119. https://doi.org/10.1183/13993003.01032-2015

5. Мартынюк Т.В. Легочная гипертензия: диагностика и лечение. Сер. Библиотека ФГБУ «НМИЦ кардиологии» Минздрава России. М.: ООО «Медицинское информационное агентство», 2018. 304 с.

6. Чазова И.Е., Архипова О.А., Мартынюк Т.В. Легочная артериальная гипертензия в России: анализ шестилетнего наблюдения по данным Национального регистра. Терапевтический архив. 2019; 91(1):24-32. https://doi.org/10.26442/00403660.2019.01.000024

7. Dimopoulos K., Wort S.J., Gatzoulis M.A. Pulmonary hypertension related to congenital heart disease: A call for action. Eur Heart J. 2014; 35:691–700. https://doi.org/10.1093/eurheartj/eht437

8. Lowe B.S., Therrien J., Ionescu-Ittu R., et al. Diagnosis of pulmonary hypertension in the congenital heart disease adult population: impact on outcomes. J Am Coll Cardiol. 2011; 58: 538–46. https://doi.org/10.1016/j.jacc.2011.03.033

9. Легочная гипертензия, в том числе хроническая тромбоэмболическая легочная гипертензия. Российские клинические рекомендации, 2020 http://cr.rosminzdrav.ru/#!/recomend/137

10. Kovacs G., Berghold A., Scheidl S., et al. Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review. Eur Respir J. 2009; 34 (4): 888–94. https://doi.org/10.1183/09031936.00145608

11. Kaemmerer H., Apitz C., Brockmeier K., et al. Pulmonary hypertension in adults with congenital heart disease: Updated recommendations from the Cologne Consensus Conference 2018. Int J of Cardiology. 2018; 272: 79–88. https://doi.org/10.1016/j.ijcard.2018.08.078

12. Горбачевский С.В., Шмальц А.А. Диагностика легочной гипертензии, ассоциированной с врожденными пороками сердца. Часть 1. Определение, классификация и первичное обследование пациентов. Российский вестник перинатологии и педиатрии. 2021; 66 (5):28-37 https://doi.org/10.21508/1027-4065-2021-66-5-28-37

13. Rosenzweig E.B., Abman S.H., Adatia I., et al. Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management. Eur Respir J. 2019; 53(1). https://doi.org/10.1183/13993003.01916-2018

14. Simonneau G., Gatzoulis M.A., Adatia I., et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013; 62 (25 Suppl): D34-41. https://doi.org/10.1016/j.jacc.2013.10.029

15. Cantor W.J., Harrison D.A., Moussadji J.S., et al. Determinants of survival and length of survival in adults with Eisenmenger syndrome, Am J. Cardiol. 1999; 84: 677–81. https://doi.org/10.1016/S0002-9149(99)00415-4

16. Galie N., Hoeper M.M., Humbert M., et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J. 2009; 30: 2493-537. https://doi.org/10.1093/eurheartj/ehp297

17. Stout K.K., Daniels C.J., Aboulhosn J.A., et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019; 139: e698-e800. https://doi.org/10.1161/CIR.0000000000000603

18. Бокерия Л.А., Горбачевский С.В., Шмальц А.А., соавт. Гемодинамическая коррекция функционально единственного желудочка сердца после ранее выполненного суживания легочной артерии. Грудная и сердечно-сосудистая хирургия. 2008; 5:27-34.

19. Бокерия Л.А., Горбачевский С.В., Шмальц А.А. Легочная артериальная гипертензия, ассоциированная с врожденными пороками сердца у взрослых (клиническая рекомендация). Грудная и сердечно-сосудистая хирургия. 2017; 59 (2): 135-47. https://doi.org/10.24022/0236-2791-2017-59-2-135-147

20. Легочная гипертензия у детей. Российские клинические рекомендации, 2017. http://cr.rosminzdrav.ru/#!/recomend/901

21. Гипертензионная сосудистая болезнь легких, ассоциированная с врожденными пороками сердца, у детей. Российские клинические рекомендации, 2018. http://cr.rosminzdrav.ru/#!/schema/356

22. Cerro M.J., Abman S., Diaz G., et al. A consensus approach to the classification of pediatric pulmonary hypertensive vascular disease: Report from the PVRI Pediatric Taskforce, Panama 2011. Pulm Circ. 2011; 1(2):286– 98. https://doi.org/10.4103/2045-8932.83456

23. Чазова И.Е., Мартынюк Т.В. Легочная гипертензия. Москва. Практика. 2015, 928 с.

24. Apitz C., Hansmann G., Schranz D. Hemodynamic assessment and acute pulmonary vasoreactivity testing in the evaluation of children with pulmonary vascular disease. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart. 2016; 102 (Suppl 2): ii23–9. http://dx.doi.org/10.1136/heartjnl-2014-307340

25. Moceri P., Kempny A., Liodakis E., et al. Physiological differences between various types of Eisenmenger syndrome and relation to outcome. Int J Cardiol. 2015; 179:455–460. https://doi.org/10.1016/j.ijcard.2014.11.100

26. Kozlik-Feldmann G., Hansmann, D., et al. Pulmonary hypertension in children with congenital heart disease (PAH-CHD, PPHVD-CHD). Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK, Heart 2016; 102 (Suppl 2): i42-ii8. http://dx.doi.org/10.1136/heartjnl-2015-308378

27. van der Linde D., Konings E.E., Slager M.A., et al. Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J Am Coll Cardiol 2011; 58: 2241-47. https://doi.org/10.1016/j.jacc.2011.08.025

28. Liu Y., Chen S., Zuhlke L, et al. Global birth prevalence of congenital heart defects 1970-2017: updated systematic review and meta-analysis of 260 studies. Int J Epidemiol. 2019; 48:455-46. https://doi.org/10.1093/ije/dyz009

29. Lytzen R., Vejlstrup N., Bjerre J., et al. Live-born major congenital heart disease in Denmark: incidence, detection rate, and termination of pregnancy rate from 1996 to 2013. JAMA Cardiol 2018; 3: 829-37. https://doi.org/10.1001/jamacardio.2018.2009

30. Moons P., Bovijn L., Budts W., et al. Temporal trends in survival to adulthood among patients born with congenital heart disease from 1970 to 1992 in Belgium. Circulation. 2010; 122: 2264-72. https://doi.org/10.1161/CIRCULATIONAHA.110.946343

31. MarelliA.J., Ionescu-Ittu R., MackieA.S., et al. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation. 2014; 130: 749-56. https://doi.org/10.1161/CIRCULATIONAHA.113.008396

32. Van de Bruaene A., Delcroix M., Pasquet A., et al. The Belgian Eisenmenger syndrome registry: Implications for treatment strategies? Acta Cardiol. 2009;64: 447–53. https://doi.org/10.2143/AC.64.4.2041608

33. van Riel A.C., Schuuring M.J., van Hessen I.D., et al. Contemporary prevalence of pulmonary arterial hypertension in adult congenital heart disease following the updated clinical classification. Int J Cardiol. 2014; 174: 299-305. https://doi.org/10.1016/j.ijcard.2014.04.072

34. Manes A., Palazzini M., Leci E., et al. Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups. Eur Heart J. 2014; 35: 716-24. https://doi.org/10.1093/eurheartj/eht072

35. McLaughlin V.V., Presberg K.W., Doyle R.L., et al. Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004. 126 (1 Suppl):78S–92S. https://doi.org/10.1378/chest.126.1_suppl.78S

36. Marelli A.J., Mackie A.S., Ionescu-Ittu R., et al. Congenital heart disease in the general population: Changing prevalence and age distribution. Circulation. 2007; 115:163–72. https://doi.org/10.1161/CIRCULATIONAHA.106.627224

37. Duffels M.G., Engelfriet P.M., Berger R.M. et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol. 2007; 120(2):198-204. https://doi.org/10.1016/j.ijcard.2006.09.017

38. Ramjug S., Hussain N., Hurdman J. et al. Pulmonary arterial hypertension associated with congenital heart disease: Comparison of clinical and anatomic-pathophysiologic classification. J Heart Lung Transplant. 2016. 35(5):610-18. https://doi.org/10.1016/j.healun.2015.12.016

39. Diller G.P., Dimopoulos K., Kafka H., et al. Model of chronic adaptation: Right ventricular function in Eisenmenger syndrome. Eur. Heart J. Suppl. 2007; 9:H54–H60. https://doi.org/10.1093/eurheartj/sum019

40. Alonso-Gonzalez R., Lopez-Guarch C.J., Subirana-Domenech M.T., et al. Pulmonary hypertension and congenital heart disease: An insight from the REHAP National Registry. Int J Cardiol. 2015; 184:717-23. https://doi.org/10.1016/j.ijcard.2015.02.031

41. Kempny A., Hjortshøj C.S., Gu H., et al. Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study. Circulation. 2017;135(15):1432-1440. https://doi.org/10.1161/CIRCULATIONAHA.116.023033

42. Morse J.H., Barst R.J., Fotino M. Familial pulmonary hypertension: immunogenetic findings in four Caucasian kindreds. Am Rev Respir Dis. 1992; 145:787–92. https://doi.org/10.1164/ajrccm/145.4_Pt_1.787

43. Roberts K.E., McElroy J.J., Wong W.P., et. al. BMPR2 mutations in pulmonary arterial hypertension with congenital heart disease. Eur. Respir. J. 2004; 24:371–74. https://doi.org/10.1183/09031936.04.00018604

44. Бокерия Л.А., Горбачевский С.В., Шмальц А.А. Легочная артериальная гипертензия, ассоциированная с врожденными пороками сердца у взрослых. Методическое пособие. НЦССХ им. А. Н. Бакулева РАМН, 2016. 28 с. ISBN 978-5-7982-0368-0.

45. Хугаев Г.А., Шмальц А.А. Морфологическая оценка сосудов легких при легочной гипертензии, обусловленной врожденными пороками сердца. Архив патологии. 2021; 83 (5): 49–57. https://doi.org/10.17116/patol20218305149

46. Rabinovitch M. Molecular pathogenesis of pulmonary arterial hypertension. J Clin Invest. 2012; 122 (12):4306-13. https://doi.org/10.1172/JCI60658

47. Iacobazzi D., Suleiman M.S., Ghorbel M., et al. Cellular and molecular basis of RV hypertrophy in congenital heart disease. Heart. 2016; 102(1):12-7. https://doi.org/10.1136/heartjnl-2015-308348

48. Bradford R., Tulloh R. Diagnosis and management of pulmonary hypertension in adult congenital heart disease. Br J Cardiac Nurs. 2008; 3:138–45.

49. Rubens C., Ewert R., Halank M., et al. Big endothelin-1 and endothelin-1 plasma levels are correlated with the severity of primary pulmonary hypertension. Chest. 2001. 120(5):1562-69. https://doi.org/10.1378/chest.120.5.1562

50. Gorbachevsky Sv., Shmalts A.A. What can cause pulmonary vascular disease in functionally single ventricle? Anatomy & Physiology: Current Research. 2016; 6 (1). https://doi.org/10.4172/2161-0940.1000e137

51. Adatia I., Kothari S.S., Feinstein J.A. Pulmonary hypertension associated with congenital heart disease: pulmonary vascular disease: the global perspective. Chest 2010; 137(6):52-61. https://doi.org/10.1378/chest.09-2861

52. Шарыкин А.С. Врожденные пороки сердца. Руководство педиатров, кардиологов, неонатологов. -М.:Теремок.-2005.-381 c. ISBN 5-9711-0011-4.

53. Favoccia C., Constantine A.H., Wort S.J., et al. Eisenmenger syndrome and other types of pulmonary arterial hypertension related to adult congenital heart disease. Expert Review of Cardiovasc Therapy 2019; 17:449-59. https://doi.org/10.1080/14779072.2019.1623024

54. Baumgartner H., Bonhoeffer P., De Groot N.M., et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). European Paediatric Cardiology (AEPC), ESC Committee for Practice Guidelines (CPG). Eur Heart J. 2010; 31(23):2915-57. https://doi.org/10.1093/eurheartj/ehq249

55. Heath D., Edwards J.E. The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects. Circulation. 1958; 4 (Part 1):533-47. https://doi.org/10.1161/01.CIR.18.4.533

56. Yamaki S., Horiuchi T., Miura M., et al. Pulmonary vascular disease in secundum atrial septal defect with pulmonary hypertension. Chest. 1986; 89(5):694-8. https://doi.org/10.1378/chest.89.5.694

57. Heath D., Helmholz H.F. Jr, Burchell H., et al. Relation between structural change in the small pulmonary arteries and the immediate reversibility of pulmonary hypertension following closure of ventricular and atrial septal defects. Circulation. 1958; 18(6):1167-74. https://doi.org/10.1161/01.cir.18.6.1167

58. Есипова И.К. Некоторые вопросы патологии лёгких. АН СССР. 1962.

59. Haworth S.G., Rabinovitch M. Pulmonary circulation. In: Anderson RH, Baker EJ, Penny DJ, Redington AN, Rigby ML, Wernovsky G (eds). Paediatric Cardiology, 3rd edn. Churchill Livingstone, Elsevier, Philadelphia, PA, 2010:117–141.

60. Rabinovitch M., Haworth S.G., Castaneda A.R., et al. Lung biopsy in congenital heart disease: a morphometric approach to pulmonary vascular disease. Circulation. 1978; 58(6):1107-22. https://doi.org/10.1161/01.CIR.58.6.1107

61. Endo M., Yamaki S., Ohmi M., Tabayashi K. Pulmonary vascular changes induced by congenital obstruction of pulmonary venous return. Ann Thorac Surg. 2000; 69 (1):193-7. https://doi.org/10.1016/S0003-4975(99)01079-6

62. Dimopoulos K., Diller G.P., Opotowsky A.R., et al. Definition and Management of Segmental Pulmonary Hypertension. J Am Heart Assoc. 2018;7 (14):e008587. https://doi.org/10.1161/JAHA.118.008587

63. Thiene G., Frescura C., Bini R.M., et al. Histology of pulmonary arterial supply in pulmonary atresia with ventricular septal defect. Circulation. 1979; 60 (5):1066-74. https://doi.org/10.1161/01.cir.60.5.1066

64. Van Dissel A.C., Mulder B.J., Bouma B.J. The Changing Landscape of Pulmonary Arterial Hypertension in the Adult with Congenital Heart Disease. J of Clin Med. 2017; 6(4):40. https://doi.org/10.3390/jcm6040040

65. Горбачевский С.В., Шмальц А.А., Плотникова Л.Р. Легочная гипертензия у детей с врожденными пороками сердца. Москва. 2018.

66. Бокерия Л.А., Горбачевский С.В., Школьникова М.А. Легочная гипертензия у детей. Москва. Актелион фармасьютикалз; 2013.

67. Горбачевский С.В., Шмальц А.А. Гипертензионная сосудистая болезнь легких, ассоциированная с врожденными пороками сердца. В кн.: Бокерия Л.А., Шаталов К.В. Детская кардиохирургия. Руководство для врачей. Москва: НЦССХ им. А.Н. Бакулева; 2016: 833–50.

68. Hansmann G., Koestenberger M., Alastalo T.P., et al. 2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT. J Heart Lung Transplant. 2019; 38(9):879-901. https://doi.org/10.1016/j.healun.2019.06.022

69. Lammers A.E., Apitz C., Zartner P., et al. Diagnostics, monitoring and outpatient care in children with suspected pulmonary hypertension/paediatric pulmonary hypertensive vascular disease. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart. 2016; 102 (Suppl 2):ii1–13. https://doi.org/10.1136/heartjnl-2015-307792

70. Dimopoulos K., Diller G.P. Pulmonary Hypertension in Adult Congenital Heart Disease. Springer; 2017. 368p. https://doi.org/10.1007/978-3-319-46028-4

71. Dimopoulos K., Prapa M., Gatzoulis M. Eisenmenger syndrome and other types of pulmonary arterial hypertension related to congenital heart disease. In: Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care. Springer; 2013. https://doi.org/10.1007/978-1-4471-4619-3_182

72. Ikegami R., Ozaki K., Ozawa T., et al. Percutaneous Coronary Intervention for a Patient with Left Main Coronary Compression Syndrome. Intern Med. 2018; 57 (10):1421-4. https://doi.org/10.2169/internalmedicine.9534-17

73. Butera G., Mansour N.B., De Marco F. Emergency transcatheter coronary intervention for left main compression secondary to pulmonary hypertension in a 4-year-old child. Catheter Cardiovasc Interv. 2019; 93(1):105-7. https://doi.org/10.1002/ccd.27796

74. Mamas M.A., Clarke B., Mahadevan V.S. Embolisation of systemic-to-pulmonary collaterals in patients with the Eisenmenger reaction presenting with haemoptysis. Cardiol Young. 2008; 18 (5):528-31. https://doi.org/10.1017/S1047951108002680

75. Larici A.R., Franchi P., Occhipinti M., et al. Diagnosis and management of hemoptysis. Diagn Interv Radiol. 2014; 20 (4):299-309. https://doi.org/10.5152/dir.2014.13426

76. Broberg C.S., Ujita M., Prasad S. et al. Pulmonary arterial thrombosis in Eisenmenger syndrome is associated with biventricular dysfunction and decreased pulmonary flow velocity. J Am Coll Cardiol. 2007; 50(7):634–42. https://doi.org/10.1016/j.jacc.2007.04.056

77. Placik B., Rodbard S., McMahon J., Swaroop S. Pulmonary artery dissection and rupture in Eisenmenger’s syndrome. Vasc Surg. 1976; 10(2):72-80. https://doi.org/10.1177/153857447601000203

78. Areco D., Pizzano N. Pulmonary artery dissection: echocardiographic findings and diagnosis. Echocardiography. 2003; 20 (4): 375-7. https://doi.org/10.1046/j.1540-8175.2003.03044.x

79. Mikhail G.W., Gibbs J.S., Yacoub M.H. Pulmonary and systemic arterial pressure changes during syncope in primary pulmonary hypertension. Circulation. 2001; 104(11):1326-7. https://doi.org/10.1161/hc3601.095274

80. Moledina S., Hislop A.A., Foster H., et al. Childhood idiopathic pulmonary arterial hypertension: a national cohort study. Heart. 2010; 96(17):1401-6. https://doi.org/10.1136/hrt.2009.182378

81. Чазова И. Е., Авдеев С. Н., Царева Н.А., соавт. Клинические рекомендации по диагностике и лечению легочной гипертонии. Терапевтический архив. 2014; 9:4-23.

82. Kaemmerrer H., Koichiro N., Oechslin E., et al. Pulmonary Arterial Hypertension in Congenital Heart Disease: Eisenmenger’s Syndrome – A Global Perspectiv. 1st edition. Bremen: UNI-MED; 2013.

83. Oechslin E., Mebus S., Schulze-Neick I., et al. The Adult Patient with Eisenmenger Syndrome: A Medical Update after Dana Point Part III: Specific Management and Surgical Aspects. Cur Cardiol Rev. 2010; 6 (4):363–72. https://doi.org/10.2174/157340310793566127

84. Labombarda F., Kerros H., Grollier G. Eisenmenger syndrome due to a large ductus arteriosus. Arch Cardiovasc Dis. 2010; 103 (11-12):623-5. https://doi.org/10.1016/j.acvd.2010.02.004

85. Dimopoulos K. Eisenmenger syndrome in an adult patient with a large patent ductus arteriosus. Eur Respir Rev. 2013; 22(130): 558-64. https://doi. org/10.1183/09059180.00007013

86. Рябыкина Г.В., Блинова Е.В., Сахнова Т.А. Электровекторкардиографическая диагностика гипертрофии правого желудочка у больных легочной гипертензией. Медицинский алфавит 2017; 8:3-17.

87. Kamphuis V.P., Haeck M.L., Wagner G.S., et al. Electrocardiographic detection of right ventricular pressure overload in patients with suspected pulmonary hypertension. J Electrocardiol. 2014; 47(2):175-82. https://doi.org/10.1016/j.jelectrocard.2013.10.010

88. Tongers J., Schwerdtfeger B., Klein G., et al. Incidence and clinical relevance of supraventricular tachyarrhythmias in pulmonary hypertension. Am Heart J. 2007; 153:127-32. https://doi.org/10.1016/j.ahj.2006.09.008

89. Юрпольская Л.А., Шмальц А.А. Рентгенография, рентгеновская компьютерная и магнитно-резонансная томография при легочной гипертензии, ассоциированной с врожденными пороками сердца. Детские болезни сердца и сосудов. 2021; 18(2):83-93. https://doi.org/10.24022/1810-0686-2021-18-2-83-93

90. Цаллагова З.С., Лазуткина В.К. Возможности традиционной рентгенографии в оценке легочной артериальной гипертензии. Медицинская визуализация. 2001; 4:59-63.

91. Рентгенодиагностика заболеваний сердца и сосудов. /Ред. М.А. Иваницкая. -М., «Медицина», 1970.

92. Рентгенодиагностика в педиатрии. Руководство для врачей в 2-х томах. /Под ред. В.Ф.Баклановой, М.А.Филиппкина. -М.: Медицина,-1988.-Т.1.-448 P.

93. Детская кардиология. Под ред. Дж.Хоффмана. Пер.с англ. -М.: Практика.-2006.-543С.

94. Коробкова И.З., Лазуткина В.К., Низовцова Л.А. и др. Методические аспекты рентгенологической оценки легочной гипертензии. Вестник рентгенологии и радиологии. 2015. 4: 45-53 https://doi.org/10.20862/0042-4676-2015-0-4-14-21

95. Dimopoulos K., Giannakoulas G., Bendayan I., et al. Cardiothoracic ratio from postero-anterior chest radiographs: a simple, reproducible and independent marker of disease severity and outcome in adults with congenital heart disease. Int J Cardiol. 2013.166:453-7. https://doi.org/10.1016/j.ijcard.2011.10.125

96. Freedom R.M., Yoo Shi-joon, Mikailian H., Williams W.G. The Natural and Modified History of Congenital Heart Disease. 2008. Wiley-Blackwell-904 p. https://doi.org/10.1002/9780470986905.ch1

97. Freedom R. M., Benson L.N., Smallhorn J.F. Neonatal Heart Disease. Springer-Verlag London 1992, 881p. https://doi.org/10.1007/978-1-4471-1814-5

98. Koestenberger M., Apitz C., Abdul-Khaliq H., Hansmann G. Transthoracic echocardiography for the evaluation of children and adolescents with suspected or confirmed pulmonary hypertension. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and D6PK. Heart. 2016; 102 (Suppl 2): ii14-22. https://doi.org/10.1136/heartjnl-2014-307200

99. Anderson R.H., Baker E.J., Penny D.J., et al. Paediatric cardiology. 3d edition. Elsevier, 2009.

100. Барышникова И.Ю., Шмальц А.А. Диагностика легочной гипертензии, ассоциированной с врожденными пороками сердца. Часть 2. Эхокардиография. Российский вестник перинатологии и педиатрии. 2021; 66 (6): 23–32. https://doi.org/10.21508/1027-4065-2021-66-6-23-32

101. Li W., West C., McGhie J., et al. Consensus recommendations for echocardiography in adults with congenital heart defects from the International Society of Adult Congenital Heart Disease (ISACHD). Int J Cardiol. 2018; 272:77-83. https://doi.org/10.1016/j.ijcard.2018.07.058

102. Augustine D.X., Coates-Bradshaw L.D., Willis J., et al. BSE pulmonary hypertension guideline 2018; 5 (3): G11–G24. https://doi.org/10.1530/ERP-17-0071

103. Rudski LG., Lai W.W., Afi lalo J., et al. Guidelines for the Echocardiographic Assessment of the Right Heart in Adults: A Report from the American Society of Echocardiography Endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. J Am Soc Echocardiogr. 2010; 23: 685-713. https://doi.org/10.1016/j.echo.2010.05.010

104. Zaidi A., Knight D.S., Augustine D.X., et al. Echocardiographic assessment of the right heart in adults: a practical guideline from the British Society of Echocardiography. 2020; 7 (1): G19–G41. https://doi.org/10.1530/ERP-19-0051

105. Amsallem M., Sternbach J.M., Adigopula S., et al. Addressing the controversy of estimating pulmonary arterial pressure by echocardiography. J Am Soc Echocardiogr. 2016; 29: 93–102. https://doi.org/10.1016/j.echo.2015.11.001

106. Fisher M.R., Forfia P.R., Chamera E., et al. Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension. Am J Respir Crit Care Med. 2009; 179 (7): 615-21. https://doi.org/10.1164/rccm.200811-1691OC

107. Rimington H., Chambers J.B. Echocardiography: a practical guide for reporting and interpretation. Third edition. CRC Press; 2016. https://doi.org/10.1201/b19334

108. Lang R.M., Badano L.P., Mor-Avi V., et al. Recommendations for Cardiac Chamber Quantification by Echocardiography in Adults: An Update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Society of Echocardiography. 2015; 28 (1): 1–39. https://doi.org/10.1016/j.echo.2014.10.003

109. Abraham S., Weismann C.G. Left ventricular end-systolic eccentricity index for assessment of pulmonary hypertension in infants. Echocardiography. 2016; 33: 910–15. https://doi.org/10.1111/echo.13171

110. Nagueh S.F., Smiseth O.A., Appleton C.P., et al. Recommendations for the Evaluation of Left Ventricular Diastolic Function by Echocardiography: An Update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr. 2016; 29:277-314. https://doi.org/10.1016/j.echo.2016.01.011

111. Friedberg M., Merten L. Tissue velocities, strain, and strain rate for echocardiographic assessment of ventricular function in congenital heart disease. European J Echocardiography. 2009; 10: 585-93. https://doi.org/10.1093/ejechocard/jep045

112. Lai W.W., Mertens L.L., Cohen M.S., Geva T. Echocardiography in pediatric and congenital heart disease. From Fetus to Adult. Second Edition. Wiley Blackwell; 2016.

113. Moceri P., Li W., Dimopoulos K. Echocardiography in the Diagnosis and Follow-Up of Patients with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease. In: Dimopoulos K, Diller GP (eds). Pulmonary Hypertension in Adult Congenital Heart Disease. Springer; 2017. https://doi.org/10.1007/978-3-319-46028-4_11

114. Бокерия ЛА, Барышникова И.Ю., Горбачевский С.В. Эхокардиография в диагностике и оценке легочной гипертензии. Учебное пособие. Москва. 2017.

115. Mertens L., Friedberg M.K. Systolic ventricular function. In: Echocardiography in Pediatric and Congenital Heart Disease: From Fetus to Adult; 2016, Hoboken, US: Wiley-Blackwell, p.96-131.

116. Gurudevan S.V., Malouf P.J., Kahn A.M., et al. Noninvasive assessment of pulmonary vascular resistance using Doppler tissue imaging of the tricuspid annulus. J Am Soc Echocardiogr. 2007; 20 (10):1167–71. https://doi.org/10.1016/j.echo.2007.02.004

117. Sutherland G.R., Di Salvo G., Claus P., et al. Strain and strain rate imaging: a new clinical approach to quantifying regional myocardial function. J Am Soc Echocardiogr 2004; 17: 788-802. https://doi.org/10.1016/j.echo.2004.03.027

118. D’Alto M., Dimopoulos K., Budts W., et al. Multimodality imaging in congenital heart disease-related pulmonary arterial hypertension. Heart. 2016; 102 (12): 910-18. https://doi.org/10.1136/heartjnl-2015-308903

119. Puchalski M.D., Lui G.K., Miller-Hance W., et al. Guidelines for Performing a Comprehensive Transesophageal Echocardiographic. Journal of the American Society of Echocardiography. 2019; 32 (2): 173–215. https://doi.org/10.1016/j.echo.2018.08.016

120. Шария А.М., Мартынюк Т.В., Терновой С.К., Шария М.А. Возможности магнитно-резонансной томографии в диагностике легочной артериальной гипертензии. Кардиология. 2021;61(6):97-104. https://doi.org/10.18087/cardio.2021.6.n1185

121. Бокерия Л.А., Макаренко В.Н., Юрпольская Л.А. Компьютерная и магнитно-резонансная томография в диагностическом алгоритме врожденных пороков сердца: Что? Когда? Кому?-“за” и “против”. Грудная и сердечно-сосудистая хирургия 2014; 3: 4-13.

122. Latus H., Kuehne T., Beerbaum Ph., et all. Cardiac MR and CT imaging in children with suspected or confirmed pulmonary hypertension/ pulmonary hypertensive vascular disease. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart. 2016; 102: ii30–ii35. https://doi.org/10.1136/heartjnl-2015-308246

123. van Wolferen S.A., Marcus J.T., Boonstra A., et al. Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension. Eur Heart J. 2007. 28:1250-7. https://doi.org/10.1093/eurheartj/ehl477

124. Belokon N.A., Podzolkov V.P., Mitrofanov I.O. Congenital heart disease .-M .: Medicine .-1990.-352p.

125. Brignole M., Auricchio A., Baron-Esquivias G., et al. 2013 ESC Guidelines on cardiac pacing and cardiac resynchronization therapy: the Task Force on cardiac pacing and resynchronization therapy of the European Society of Cardiology (ESC). Developed in collaboration with the European Heart Rhythm Association (EHRA). Eur Heart J. 2013; 34: 2281-329. https://doi.org/10.1093/europace/eut206

126. Radenkovic D., Weingärtner S., Ricketts L., et al. T1 mapping in cardiac MRI. Heart Fail Rev. 2017; 22:415–430. https://doi.org/10.1007/s10741-017-9627-2

127. Salerno M., Sharif B., Arheden H., et al. Recent Advances in Cardiovascular Magnetic Resonance Techniques and Applications. Cir.cCardiovasc.Imaging. 2017; 10 :e003951. https://doi.org/10.1161/CIRCIMAGING.116.003951

128. European Medicines Agency. EMA’s final opinion confirms restrictions on use of linear gadolinium agents in body scans. https://https://www.ema.europa.eu/en/documents/press-release/emas-final-opinion-confirms-restrictions-use-linear-gadolinium-agents-body-scans_en.pdf

129. Odagiri K., Inui N., Hakamata A., et al. Noninvasive evaluation of pulmonary arterial blood flow and wall shear stress in pulmonary arterial hypertension with 3D phase contrast magnetic resonance imaging. Springer Plus 2016; 5: 1071. https://doi.org/10.1186/s40064-016-2755-7

130. Torbicki A. Cardiac magnetic resonance in pulmonary arterial hypertension: a step in the right direction. Eur Heart J 2007; 28: 1187-1189. https://doi.org/10.1093/eurheartj/ehm074

131. Perloff J.K., Marelli A. Perloff’s Clinical Recognition of Congenital Heart Disease: Expert Consult Online and Print, 6e. 6th edition. Philadelphia: Saunders; 2012.

132. de Siqueira M.E.M.., Pozo E., Fernandes V.R., et all. Characterization and clinical significance of right ventricular mechanics in pulmonary hypertension evaluated with cardiovascular magnetic resonance feature tracking. J of Cardiovasc Magn Res-2016; https://doi.org/10.1186/s12968-016-0258-x

133. Tello K., Dalmer A., Vanderpool R., et al. Cardiac Magnetic Resonance Imaging-Based Right Ventricular Strain Analysis for Assessment of Coupling and Diastolic Function in Pulmonary Hypertension. JACC Cardiovasc Imaging. 2019; 12 (1): 2155-64. https://doi.org/10.1016/j.jcmg.2018.12.032

134. Рентгенодиагностика заболеваний сердца и сосудов. Ред. М.А. Иваницкая. М., «Медицина», 1970.

135. Макаренко В.Н., Юрпольская Л.А., Зеленикин М.М., Шинкарева Т.В. Компьютерная томография в оценке пациентов после операции Фонтена. Бюллетень НЦССХ им. А.Н.Бакулева РАМН. 2010; 11 (1): 46-50.

136. Kilner P., Nichol E., Rubens M. The roles of CT and CMR in Adult Congenital Heart Disease. In: ESC Textbook of Cardiovascular Imaging. 2nd ed, Oxford:Oxford University Press; 2015, p. 563-600.

137. Grosse-Wortmann L., Drolet Chr., Dragulescu An., et al. Aortopulmonary collateral flow volume affects early postoperative outcome after Fontan completion: A multimodality study. J. Thorac. Cardiovasc. Surg. 2012; 144:1329-36. https://doi.org/10.1016/j.jtcvs.2012.03.032

138. Habib G., Lancellotti P., Antunes M.J., et al. ESC Scientific Document Group. 2015 ESC Guidelines for the management of infective endocarditis: The Task Force for the Management of Infective Endocarditis of the European Society of Cardiology (ESC). Endorsed by: European Association for Cardio-Thoracic Surgery (EACTS), the European Association of Nuclear Medicine (EANM). Eur Heart J. 2015; 36:3075-3128. https://doi.org/10.1093/eurheartj/ehv319

139. Ohno Y., Koyama H., Lee H.Y., Miura S.t all. Contrast-enhanced CT-and MRI-based perfusion assessment for pulmonary diseases: basics and clinical applications. Diagn Interv Radiol-2016; 22: 407–421. https://doi.org/10.5152/dir.2016.16123

140. Pienn M., Kovacs G., Tscherner M., Avian A. et all. Non-invasive determination of pulmonary hypertension with dynamic contrast-enhanced computed tomography: a pilot study. Eur Radiol.-2014; 24:668–676. https://doi.org/10.1007/s00330-013-3067-8

141. Соболев А.В., Шмальц А.А. Эндоваскулярная диагностика легочной гипертензии, ассоциированной с врожденными пороками сердца. Часть 1. Катетеризация сердца и ангиокардиография. Эндоваскулярная хирургия. 2021; 8 (3): 263–71. https://doi.org/10.24183/2409-4080-2021-8-3-263-271

142. Guillinta P., Peterson K.L., Ben-Yehuda O. Cardiac catheterization techniques in pulmonary hypertension. Cardiol Clin. 2004; 22: 401–15. https://doi.org/10.1016/j.ccl.2004.04.011

143. Peterson K.L., Nicod P. Cardiac catheterization: methods, diagnosis, and therapy. Saunders, Philadelphia; 1997.

144. Barnett C., Ben-Yehuda О. Cardiac Catheterization in the Patient with Pulmonary Hypertension. Textbook of Pulmonary Vascular Disease Springer, Science+Business Media, LLC; 2011. https://doi.org/10.1007/978-0-387-87429-6

145. Горбачевский С.В., Белкина М.В., Колединский Д.Г., соавт. Инвазивный мониторинг давления в легочной артерии — объективный метод оценки степени тяжести легочной гипертензии у детей с врожденными пороками сердца. Детские болезни сердца и сосудов. 2006; 4: 77–81.

146. Wilkinson J.L. The Fontan сirculation. Results, late Follow-up and management. Heart Views. 2004; 4 (4): 73-78.

147. Fredenburg T.B., Johnson T.R., Cohen M.D. The Fontan Procedure: Anatomy, Complications, and Manifestations of Failure. RadioGraphics. 2011;31(2): 453–463. https://doi.org/10.1148/rg.312105027

148. Bergersen L., Foerster S., Marshall A.C., Meadows J. (eds). Congenital heart disease: the catheterization manual. Springer, New York; 2009. https://doi.org/10.1007/978-0-387-77292-9

149. Бокерия Л.А., Алекян Б.Г. (ред.). Руководство по рентгеноэндоваскулярной хирургии сердца и сосудов. Москва, НЦССХ им. А.Н. Бакулева РАМН; 2008.

150. Sitbon O., Humbert M., Jais X., et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation. 2005; 111:3105–11. https://doi.org/10.1161/CIRCULATIONAHA.104.488486

151. Barst R.J. Pharmacologically induced pulmonary vasodilatation in children and young adults with primary pulmonary hypertension. Chest. 1986; 89:497–503. https://doi.org/10.1378/chest.89.4.497

152. Rich S., Kaufmann E., Levy P.S. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med. 1992; 327:76–81. https://doi.org/10.1056/NEJM199207093270203

153. Douwes J.M., van Loon RLE, Hoendermis E.S, et al. Acute pulmonary vasodilator response in paediatric and adult pulmonary arterial hypertension: occurrence and prognostic value when comparing three response criteria. Eur Heart J. 2011; 32(24):3137-46. https://doi.org/10.1093/eurheartj/ehr282

154. Chessa M. Cardiac Catheterization. In: Dimopoulos K, Diller G.P. (eds). Pulmonary Hypertension in Adult Congenital Heart Disease. Springer; 2017.

155. Hoeper M.M., Lee S.H., Voswinckel R., et al. Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J Am Coll Cardiol. 2006; 48:2546–52. https://doi.org/10.1016/j.jacc.2006.07.061

156. Diller G.P., Dimopoulos K., Okonko D. et al. Exercise intolerance in adult congenital heart disease: Comparative severity, correlates, and prognostic implication. Circulation. 2005. 112:828–35. https://doi.org/10.1161/CIRCULATIONAHA.104.529800.

157. Savarese G., Paolillo S., Costanzo P., et al. Do changes of 6-minute walk distance predict clinical events in patients with pulmonary arterial hypertension? А meta-analysis of 22 randomized trials. J Am Coll Cardiol 2012; 60:1192–1201. https://doi.org/10.1016/j.jacc.2012.01.083

158. Gabler N.B., French B., Strom B.L, et al. Validation of 6-minute walk distance as a surrogate end point in pulmonary arterial hypertension trials. Circulation 2012; 126: 349–56. https://doi.org/10.1161/CIRCULATIONAHA.112.105890

159. Глушко Л.А., Шмальц А.А. Оценка состояния кардиореспираторной системы при легочной гипертензии, ассоциированной с врожденными пороками сердца. Креативная кардиология. 2021; 15 (2): 167-179.

160. Arena R., Lavie C.J., Milani R.V., et al. Cardiopulmonary exercise testing in patients with pulmonary arterial hypertension: an evidence-based review. J Heart Lung Transplant. 2010; 29 (2): 159-73. https://doi.org/10.1016/j.healun.2009.09.003

161. Johnson M.K., Thompson S. The role of exercise testing in the modern management of pulmonary arterial hypertension. Diseases. 2014; 2: 120-47. https://doi.org/10.3390/diseases2020120

162. Wasserman K., Hansen J.E., Sue D.Y., et al. Principles of exercise testing and interpretation: including pathophysiology and clinical applications. Fifth edition. Philadelphia: Lippincott Williams & Wilkins; 2012, 572 p.

163. Seeger W., Adir Y., Barbera J.A., et al. Pulmonary hypertension in chronic lung diseases. J Am Coll Cardiol. 2013; 62: D109–16. https://doi.org/10.1016/j.jacc.2013.10.036

164. Johnson J.T., Yetman A.T. Cardiopulmonary exercise testing in adults with congenital heart disease. Prog Pediatr Cardiol. 2012; 34: 47–52.

165. Laveneziana P., Garcia G., Joureau B. et al. Dynamic respiratory mechanics and exertional dyspnoea in pulmonary arterial hypertension. Eur Respir J. 2013. 41:578-587. https://doi.org/10.1183/09031936.00223611

166. Ferrazza A.M., Martolini D., Valli G., Palange P. Cardiopulmonary exercise testing in the functional and prognostic evaluation of patients with pulmonary diseases. Respiration. 2009; 77 (1): 3-17. https://doi.org/10.1159/000186694

167. Mantegazza V., Apostolo A, Hager A. Cardiopulmonary Exercise Testing in Adult Congenital Heart Disease. Ann Am Thorac Soc. 2017; 14 (Suppl.1): S93-S101. https://doi.org/10.1513/AnnalsATS.201611-876FR

168. Diller G.P., Kempny A., Inuzuka R., et al. Survival prospects of treatment naïve patients with Eisenmenger: a systematic review of the literature and report of own experience. Heart. 2014; 100(17): 1366-72. https://doi.org/10.1136/heartjnl-2014-305690

169. Buys R., Cornelissen V., Van De Bruaene A., et al. Measures of exercise capacity in adults with congenital heart disease. Int J Cardiol. 2011; 153 (1): 26-30. https://doi.org/10.1016/j.ijcard.2010.08.030

170. Таран И.Н., Валиева З.С., Мартынюк Т.В., и соавт. Спировелоэргометрия в стратификации риска пациентов с легочной артериальной гипертензией и хронической тромбоэмболической легочной гипертензией. Евразийский кардиологический журнал. 2017; 4: 86-92. https://doi.org/10.38109/2225-1685-2017-4-86-92

171. Zhao Q.H., Wang L., Pudasaini B., et al. Cardiopulmonary exercise testing improves diagnostic specificity in patients with echocardiography-suspected pulmonary hypertension. Clin Cardiol. 2017; 40 (2): 95-101. https://doi.org/10.1002/clc.22635

172. Чазова И.Е., Мартынюк Т.В. и соавт. Диагностика и лечение легочной гипертензии. Российские рекомендации. Кардиоваскулярная терапия и профилактика 2007; 6: Приложение 2.

173. Sun X.G., Hansen J.E., Oudiz R.J, Wasserman K. Pulmonary function in primary pulmonary hypertension. J Am Coll Cardiol 2003; 41:1028–35. https://doi.org/10.1016/s0735-1097(02)02964-9

174. Alonso-Gonzalez R., Borgia F., Diller G.P., et al. Abnormal lung function in adults with congenital heart disease: prevalence, relation to cardiac anatomy, and association with survival. Circulation. 2013; 127 (8): 882-90. https://doi.org/10.1161/CIRCULATIONAHA.112.126755

175. Ferguson G.T., Enright P.L., Buist A.S., Higgins M.W. Office spirometry for lung health assessment in adults: A consensus statement from the National Lung Health Education Program. Chest. 2000;117 (4): 1146-61. https://doi.org/10.1378/chest.117.4.1146

176. Архипова О.А., Мартынюк Т.В., Самойленко Л.Е., соавт. Перфузионная сцинтиграфия легких у больных с легочной гипертензией различной этиологии. Евразийский кардиологический журнал. 2015; 4: 21-25.

177. Tunariu N., Gibbs S.R., Win Z., et al. Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension. J Nucl Med. 2007; 48: 680-84. https://doi.org/10.2967/jnumed.106.039438

178. Albrecht T., Blomley M.J., Cosgrove D.O., et al. Non-invasive diagnosis of hepatic cirrhosis by transit-time analysis of an ultrasound contrast agent. Lancet. 1999; 353: 1579-83. https://doi.org/10.1016/S0140-6736(98)06373-9

179. Perloff J.K. Systemic complications of cyanosis in adults with congenital heart disease. Hematologic derangements, renal function, and urate metabolism. Cardiol Clin 1993; 11: 689-99.

180. Giannakoulas G., Mouratoglou S., Gatzoulis M.A.. et al. Blood biomarkers and their potential role in pulmonary arterial hypertension associated with congenital heart disease. A systematic review. International Journal of Cardiology. 2014; 174 (3): 618–23. https://doi.org/10.1016/j.ijcard.2014.04.156

181. Fenster B.E., Lasalvia L, Schroeder J.D., et al. Cystatin C for pulmonary hypertension. Respirology. 2014. 19: 583-89. https://doi.org/10.1111/resp.12259

182. Scognamiglio G., Kempny A., Price L.C., et al. C-reactive protein in adults with pulmonary arterial hypertension associated with congenital heart disease and its prognostic value. Heart. 2014. 100:1335-1341. https://doi.org/10.1136/heartjnl-2014-305494

183. Dimopoulos K., Okonko D.O., Diller G.P., et al. Abnormal ventilatory response to exercise in adults with congenital heart disease relates to cyanosis and predicts survival. Circulation 2006; 113 (24): 2796-802. https://doi.org/10.1161/CIRCULATIONAHA.105.594218

184. Deanfield J., Thaulow E., Warnes C., et al. Management of grown-up congenital heart disease. Eur Heart J. 2003; 24: 1035-84. https://doi.org/10.1016/s0195-668x(03)00131-3

185. Yang-Ting S., Aboulhosn J., Sun X.G. et al. Effects of pulmonary vasodilator therapy on ventilatory efficiency during exercise in adults with Eisenmenger syndrome. Congenit Heart Dis. 2011; 6 (2): 139-46.

186. Dimopoulos K., Inuzuka R., Goletto S., et al. Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation. 2010; 121: 20-25. https://doi.org/10.1161/CIRCULATIONAHA.109.883876

187. Diller G.P., Korten M.A., Bauer U.M., et al. German Competence Network for Congenital Heart Defects Investigators. Current therapy and outcome of Eisenmenger syndrome: data of the German National Register for congenital heart defects. Eur Heart J 2016; 37: 14491455. https://doi.org/10.1093/eurheartj/ehv743

188. Oster M., Bhatt A., Zaragoza-Macias E., et al. Interventional therapy versus medical therapy for secundum atrial septal defect: a systematic review (part 2) for the 2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019; 139: e000–000. https://doi.org/10.1161/CIR.0000000000000605

189. Shah D., Azhar M., Oakley C.M., et al. Natural history of secundum atrial septal defect in adults after medical or surgical treatment: a historical prospective study. Br Heart J. 1994; 71: 224–27. https://doi.org/10.1136/hrt.71.3.224

190. Cho Y.H., Jun T-G., Yang J-H., et al. Surgical strategy in patients with atrial septal defect and severe pulmonary hypertension. Heart Surg Forum. 2012; 15:E111–5. S4.1.1-21.

191. Kouchoukos N.T., Blackstone E.H., Kirklin J.W. Surgical implications of pulmonary hypertension in congenital heart disease. Adv Cardiol. 1978: S4: 225–31. https://doi.org/10.1159/000401033

192. Yan C., Zhao S., Jiang S., et al. Transcatheter closure of patent ductus arteriosus with severe pulmonary arterial hypertension in adults. Heart. 2007; 93: 514–18. https://doi.org/10.1136/hrt.2006.091215

193. Gamboa R., Rios-Méndez R.E., Mollón F.P., et al. Percutaneous closure of patent ductus arteriosus in adults using different devices. Rev Esp Cardiol. 2010; 63: 726–29. https://doi.org/10.1016/s1885-5857(10)70147-x

194. Jeong Y-H., Yun T-J., Song J-M., et al. Left ventricular remodeling and change of systolic function after closure of patent ductus arteriosus in adults: device and surgical closure. Am Heart J. 2007; 154: 436–40. https://doi.org/10.1016/j.ahj.2007.04.045

195. Zabal C., García-Montes J.A., Buendía-Hernández A., et al. Percutaneous closure of hypertensive ductus arteriosus. Heart. 2010; 96: 625–29. https://doi.org/10.1136/hrt.2009.185025

196. Lim D.S., Forbes T.J., Rothman A., et al. Transcatheter closure of high-risk muscular ventricular septal defects with the CardioSEAL occluder: initial report from the CardioSEAL VSD registry. Catheter Cardiovasc Interv. 2007; 70 (5): 740-44

197. Chessa M., Butera G., Negura D., et al. Transcatheter closure of congenital ventricular septal defects in adult: mid-term results and complications. Int J Cardiol. 2009; 133 (1):70-3. https://doi.org/10.1016/j.ijcard.2007.11.098

198. Горбачевский С.В., Шмальц А.А. Паллиативное предсердное и артериальное переключение при транспозиции магистральных артерий с дефектом межжелудочковой перегородки и тяжелой легочно-сосудистой болезнью. Детские болезни сердца и сосудов. 2019; 16 (2): 86-94.

199. Петросян К.В., Горбачевский С.В., Дадабаев Г.М., Пардаев Д.Б. Стентирование ствола левой коронарной артерии у пациентки с легочной гипертензией. Евразийский кардиологический журнал. 2017; 3: 86-87.

200. Gorbachevsky S.V., Shmalts A.A., Dadabaev G.M., et al. Outcomes of Atrioseptostomy with Stenting in Patients with Pulmonary Arterial Hypertension from a Large Single-Institution Cohort. Diagnostics (Basel). 2020; 10(9): 725. https://doi.org/10.3390/diagnostics10090725

201. Горбачевский С.В., Пурсанов М.Г., Шмальц А.А., соавт. Результаты атриосептостомии со стентированием у больных с идиопатической и схожими формами легочной артериальной гипертензии. Грудная и сердечно-сосудистая хирургия. 2019; 61 (2): 100-113.

202. Шмальц А.А., Нишонов Н.А. Атриосептостомия у больных с легочной гипертензией. Грудная и сердечно-сосудистая хирургия. 2015; 57 (5): 18-25.

203. Christie J.D., Edwards L.B., Kucheryavaya A.Y. et al. The Registry of the International Society for Heart and Lung Transplantation: 29th official adult lung and heart-lung transplant report 2012. J. Heart Lung Transplant. 2012; 31:1073–86. https://doi.org/10.1016/j.healun.2012.08.004

204. Fadel E., Mercier O., Mussot S., et al. Long-term outcome of double-lung and heart-lung transplantation for pulmonary hypertension: a comparative retrospective study of 219 patients. Eur J Cardiothorac Surg. 2010; 38: 277– 84. https://doi.org/10.1016/j.ejcts.2010.02.039

205. Hopkins W.E., Ochoa L.L., Richardson G.W., et al. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. J Heart Lung Transplant. 1996; 15 (1):100-5.

206. Moons P., De Geest S., Budts W. Comprehensive care for adults with congenital heart disease: expanding roles for nurses. Eur J Cardiovasc Nurs. 2002; 1: 23-28. https://doi.org/10.1016/S1474-5151(01)00014-7

207. Baumgartner H., Budts W., Chessa M., et al, Working Group on Grown-up Congenital Heart Disease of the European Society of Cardiology. Recommendations for organization of care for adults with congenital heart disease and for training in the subspecialty of ‘Grown-up Congenital Heart Disease’ in Europe: a position paper of the Working Group on Grown-up Congenital Heart Disease of the European Society of Cardiology. Eur Heart J. 2014; 35: 686-90. https://doi.org/10.1093/eurheartj/ehaa554

208. Thomet C., Moons P., Budts W., et al. ESC Working Group on Grown-up Congenital Heart Disease. Staffing, activities, and infrastructure in 96 specialised adult congenital heart disease clinics in Europe. Int J Cardiol 2019; 292: 100-105. https://doi.org/10.1016/j.ijcard.2019.04.077

209. Popelová J., Oechslin E., Kaemmerer H., Sutton M. Congenital Heart Disease in Adults 2008 Informa UK Ltd.-183p. https://doi.org/10.3109/9780203091685

210. Regitz-Zagrosek V., Roos-Hesselink J.W., Bauersachs J., et al. ESC Scientific Document Group. 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy. Eur Heart J. 2018; 39: 31653241. https://doi.org/10.1093/eurheartj/ehy340

211. Sliwa K., van Hagen I.M., Budts W., et al. Pulmonary hypertension and pregnancy outcomes: data from the Registry Of Pregnancy and Cardiac Disease (ROPAC) of the European Society of Cardiology. Eur J Heart Fail. 2016; 18 (9): 1119–28. https://doi.org/10.1002/ejhf.594

212. Jaı̈s X., Olsson K.M, Barbera J.A., et al. Pregnancy outcomes in pulmonary arterial hypertension in the modern management era. Eur Respir J. 2012; 40: 881–85. https://doi.org/10.1183/09031936.00141211

213. Kovacs A.H., Sears S.F., Saidi A.S. Biopsychosocial experiences of adults with congenital heart disease: review of the literature. Am Heart J 2005; 150: 193-201. https://doi.org/10.1016/j.ahj.2004.08.025

214. Raines D.E., Liberthson R.R., Murray J.R. Anesthetic management and outcome following noncardiac surgery in nonparturients with Eisenmenger’s physiology. J Clin Anesth. 1996; 8: 341–47. https://doi.org/10.1016/0952-8180(96)00084-0

215. Grünig, E., Benjamin, N., Krüger, U., et al. General measures and supportive therapy for pulmonary arterial hypertension: Updated recommendations from the Cologne Consensus Conference 2018. Int J Cardiol. 2018;272S:30-36. https://doi.org/10.1016/j.ijcard.2018.08.085

216. Blanche C., Alonso-Gonzalez R., Uribarri A. et al. Use of intravenous iron in cyanotic patients with congenital heart disease and/or pulmonary hypertension. Int J Cardiol. 2018. 267:79-83. https://doi.org/10.1016/j.ijcard.2018.05.062

217. Cohn J.N. Optimal diuretic therapy for heart failure. Am J Med 2001; 111: 577. https://doi.org/10.1016/s0002-9343(01)00915-9

218. Olsson K.M., Delcroix M., Ghofrani H.A., et al. Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA). Circulation. 2014; 129: 57–65. https://doi.org/10.1161/CIRCULATIONAHA.113.004526

219. Kirchhof P., Benussi S., Kotecha D., et al. 2016 ESC Guidelines for the management of atrial fibrillation developed in collaboration with EACTS. Eur Heart J 2016; 37: 2893-962. 219. https://doi.org/10.1093/eurheartj/ehw210

220. Steffel J., Verhamme P, Potpara T.S., et al. ESC Scientific Document Group. The 2018 European Heart Rhythm Association Practical Guide on the use of non-vitamin K antagonist oral anticoagulants in patients with atrial fibrillation. Eur Heart J. 2018; 39:1330-93. https://doi.org/10.1093/eurheartj/ehy136

221. Pujol C., Niesert A.C., Engelhardt A., et al. Usefulness of direct oral anticoagulants in adult congenital heart disease. Am J Cardiol. 2016; 117: 450-55. https://doi.org/10.1016/j.amjcard.2015.10.062

222. Yang H., Bouma B.J., Dimopoulos K., et al. Non-vitamin K antagonist oral anticoagulants (NOACs) for thromboembolic prevention, are they safe in congenital heart disease? Results of a worldwide study. Int J Cardiol. 2019; 299: 123-30. https://doi.org/10.1016/j.ijcard.2019.06.014

223. Sandoval J., Aguirre J.S., Pulido T., et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med. 2001; 164:1682–87. https://doi.org/10.1164/ajrccm.164.9.2106076

224. Weitzenblum E., Sautegeau A., Ehrhart M., et al. Long-term oxygen therapy can reverse the progression of pulmonary hypertension in patients with chronic obstructive pulmonary disease. Am Rev Respir Dis. 1985; 131: 493-98. https://doi.org/10.1164/arrd.1985.131.4.493

225. van de Veerdonk M.C., Kind T., Marcus J.T., et al. Progressive right ventricular dysfunction in patients with pulmonary arterial hypertension responding to therapy. J Am Coll Cardiol. 2011; 58: 2511-19. https://doi.org/10.1016/j.jacc.2011.06.068

226. Rich S., Seidlitz M., Dodin E., et al. The short-term effects of digoxin in patients with right ventricular dysfunction from pulmonary hypertension. Chest. 1998; 114: 787-92. https://doi.org/10.1378/chest.114.3.787

227. Архипова О.А., Мартынюк Т.В., Чазова И.Е. Особенности тактики ведения и выбора специфической терапии у взрослых пациентов с легочной артериальной гипертензией, ассоциированной с врожденными пороками сердца. Евразийский кардиологический журнал. 2018. 1: 48-53. https://doi.org/10.38109/2225-1685-2018-1-48-53

228. Шмальц А.А., Горбачевский С.В. Доказательная база специфических легочных вазодилататоров у детей с врожденными пороками сердца. Педиатрия. Журнал им. Г.Н. Сперанского. 2017: 96 (6):126-139. https://doi.org/10.24110/0031-403X-2017-96-6-126-139

229. Шмальц А.А., Горбачевский С.В. Доказательная база специфических легочных вазодилататоров у взрослых с врожденными пороками сердца. Терапевтический архив. 2021; 93 (9): 1106-1116. https://doi.org/10.26442/00403660.2021.09.201022

230. Шмальц А.А., Белкина М.В., Горбачевский С.В. Специфические легочные вазодилататоры после операции Фонтена. Детские болезни сердца и сосудов. 2017; 14 (1): 16-24. https://doi.org/10.24022/1810-0686-2017-14-1-16-24

231. Траклир®. Инструкция по применению лекарственного препарата для медицинского применения. ЛС-002509. https://grls.rosminzdrav.ru/Grls_View_v2.aspx?routingGuid=93619b5d-c8c6-4bdb-b201-88aff22c785c&t=

232. Бозенекс. Инструкция по медицинскому применению применению лекарственного препарата. ЛП-004208. https://grls.rosminzdrav.ru/Grls_View_v2.aspx?routingGuid=68d79ec9-1045-403c-be20-e88cc33f0aa6&t=

233. Опсамит®. Инструкция по применению лекарственного препарата для медицинского применения. ЛП-003310. https://grls.rosminzdrav.ru/Grls_View_v2.aspx?routingGuid=b2c51f9d-696d-4b23-881a-8c8d0fec4b96&t=

234. Апбрави. Инструкция по применению лекарственного препарата для медицинского применения. ЛП-005577. https://grls.rosminzdrav.ru/Grls_View_v2.aspx?routingGuid=094dda8d-ba79-4353-83f9-98ea68b865aa&t=

235. Manes A. PAH in Patients with Prevalent Systemic–Pulmonary Shunts and PAH in Patients with Small Cardiac Defects. In: Dimopoulos K, Diller GP (eds). Pulmonary Hypertension in Adult Congenital Heart Disease. Springer; 2017

236. Idorn L., Sondergaard L. Pulmonary Vascular Disease in Patients with Fontan-Type Circulation. In: Dimopoulos K, Diller GP (eds). Pulmonary Hypertension in Adult Congenital Heart Disease. Springer; 2017.

237. Moledina Sh., Theocharis P. Pulmonary Arterial Hypertension in Patients with Previous Reparative Surgery. In: Dimopoulos K, Diller GP (eds). Pulmonary Hypertension in Adult Congenital Heart Disease. Springer; 2017.

238. Шмальц А.А., Горбачевский С.В. Возможности и ограничения для начальной комбинированной специфической терапии легочной артериальной гипертензии в Российской Федерации. Терапевтический архив. 2020; 92 (12): 80-85. https://doi.org/10.26442/00403660.2020.12.000840

239. D’Alto M., Diller G.P. Pulmonary hypertension in adults with congenital heart disease and Eisenmenger syndrome: current advanced management strategies. Heart. 2014; 100 (17):1322-28. https://doi.org/10.1136/heartjnl-2014-305574

240. Чазова И.Е., Мартынюк Т.В., Наконечников С.Н. Антагонисты рецепторов эндотелина при легочной артериальной гипертензии: вчера, сегодня и завтра. Российский кардиологический журнал 2009; 4:73–81.

241. Galie N., Manes A., Palazzini M., et al. Management of pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunts and Eisenmenger’s syndrome. Drugs. 2008. 68:1049–66. https://doi.org/10.2165/00003495-200868080-00004

242. Rubin L.J., Badesch D.B., Barst R.J., et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002; 346: 896–903. https://doi.org/10.1056/NEJMoa012212

243. Galie N., Beghetti M., Gatzoulis M.A., et al. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation. 2006; 114 (1): 48–54. https://doi.org/10.1161/CIRCULATIONAHA.106.630715

244. Gatzoulis M.A., Beghetti M., Galie N., et al. Long-term bosentan therapy improves functional capacity in Eisenmenger syndrome: Results of the BREATHE-5 open-label extension study. Int J Cardiol. 2008; 127 (1): 27-32. https://doi.org/10.1016/j.ijcard.2007.04.078

245. Galiè N., Rubi.n L., Hoeper M., et al. Treatment of patients with symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet. 2008; 371 (9630): 2093-100. https://doi.org/10.1016/S0140-6736(08)60919-8

246. Sitbon O., Beghetti M., Petit J., et al. Bosentan for the treatment of pulmonary arterial hypertension associated with congenital heart defects. Eur J Clin Invest. 2006; 36 Suppl 3: 25-31. https://doi.org/10.1111/j.1365-2362.2006.01685.x

247. Benza R.L., Rayburn B.K., Tallaj J.A., et al. Efficacy of bosentan in a small cohort of adult patients with pulmonary arterial hypertension related to congenital heart disease. Chest. 2006; 129 (4):1009-15. https://doi.org/10.1378/chest.129.4.1009

248. Diller G.P., Dimopoulos K., Kaya M.G., et al. Long-term safety, tolerability and efficacy of bosentan in adults with pulmonary arterial hypertension associated with congenital heart disease. Heart. 2007; 93 (8): 974-76. https://doi.org/10.1136/hrt.2006.089185

249. Durongpisitkul K., Jakrapanichakul D., Sompradikul S. A retrospective study of bosentan in pulmonary arterial hypertension associated with congenital heart disease. J Med Assoc Thai. 2008; 91 (2): 196-202.

250. Duffels M.G., Vis J.C., van Loon R.L, et al. Effect of bosentan on exercise capacity and quality of life in adults with pulmonary arterial hypertension associated with congenital heart disease with and without Down’s syndrome. Am J Cardiol. 2009; 103 (9): 1309-15. https://doi.org/10.1016/j.amjcard.2009.01.021

251. Vis J.C., Duffels M.G., Mulder P., et al. Prolonged beneficial effect of bosentan treatment and 4-year survival rates in adult patients with pulmonary arterial hypertension associated with congenital heart disease. Int J Cardiol. 2013; 164 (1): 64-9. https://doi.org/10.1016/j.ijcard.2011.06.064

252. Baptista R, Castro .G, da Silva A.M., et al. Long-term effect of bosentan in pulmonary hypertension associated with complex congenital heart disease. Rev Port Cardiol. 2013; 32 (2): 123-29. https://doi.org/10.1016/j.repc.2012.02.023

253. Pulido T., Adzerikho I., Channick R.N., et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369: 809– 818. https://doi.org/10.1056/NEJMoa1213917

254. Gatzoulis M.A., Landzberg M., Beghetti M., et al. Evaluation of Macitentan in Patients With Eisenmenger Syndrome. Circulation. 2019; 139(1):51-63. https://doi.org/10.1161/CIRCULATIONAHA.118.033575

255. Galiè N., Ghofrani H.A., Torbicki A., et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med. 2005; 353 :2148–57. https://doi.org/10.1056/NEJMoa050010

256. Lu X.L., Xiong C.M., Shan G.L., et al. Impact of sildenafil therapy on pulmonary arterial hypertension in adults with congenital heart disease. Cardiovasc Ther. 2010; 28 (6): 350-55. https://doi.org/10.1111/j.1755-5922.2010.00213.x

257. Chau E.M., Fan K.Y., Chow W.H. Effects of chronic sildenafil in patients with Eisenmenger syndrome versus idiopathic pulmonary arterial hypertension. Int J Cardiol. 2007; 120 (3): 301-5. https://doi.org/10.1016/j.ijcard.2006.10.018

258. Zeng W.J., Lu X.L, Xiong C.M., et al. The efficacy and safety of sildenafil in patients with pulmonary arterial hypertension associated with the different types of congenital heart disease. Clin Cardiol. 2011; 34 (8): 513-18. https://doi.org/10.1002/clc.20917

259. Iversen K., Jensen A.S., Jensen T.V., et al. Combination therapy with bosentan and sildenafil in Eisenmenger syndrome: a randomized, placebo-controlled, double-blinded trial. Eur Heart J. 31 (9):1124-31. https://doi.org/10.1093/eurheartj/ehq011

260. Ghofrani H. A., Galiè N., Grimminger F., et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med. 2013; 369(4): 330-40. https://doi.org/10.1056/NEJMoa1209655

261. Шмальц А.А., Горбачевский С.В. Риоцигуат и силденафил в лечении легочной гипертензии: сходства и различия. Пульмонология 2016; 26 (1): 85-91 https://doi.org/10.18093/0869-0189-2016-26-1-85-91

262. Валиева З.С., Грацианская С.Е., Мартынюк Т.В. Применение риоцигуата для лечения пациентов с хронической тромбоэмболической легочной гипертензией. Кардиология. 2020; 60 (8): 115-23. https://doi.org/10.18087/cardio.2020.8.n1198

263. Stasch J.P., Pacher P., Evgenov O.V. Soluble Guanylate Cyclase as an Emerging Therapeutic Target in Cardiopulmonary Disease. Circulation 2011; 123: 2263-2274. https://doi.org/10.1161/CIRCULATIONAHA.110.981738

264. Rubin L.J., Galiè N., Grimminger F., et al. Riociguat for the treatment of pulmonary arterial hypertension: a long-term extension study (PATENT-2). Eur Respir J. 2015; 45 (5): 1303-13. https://doi.org/10.1183/09031936.00090614

265. Rosenkranz S., Ghofrani H.A., Beghetti M., et al. Riociguat for pulmonary arterial hypertension associated with congenital heart disease. Heart. 2015;101 (22): 1792-99. https://doi.org/10.1136/heartjnl-2015-307832

266. Cha K.S., Cho K.I., Seo J.S., et al. Effects of inhaled iloprost on exercise capacity, quality of life, and cardiac function in patients with pulmonary arterial hypertension secondary to congenital heart disease (the Eisenmenger syndrome) (from the EIGER Study). Am J Cardiol. 2013; 112 (11):1834-39. https://doi.org/10.1016/j.amjcard.2013.08.003

267. Chon M.K., Cho K.I., Cha K.S., et al. Effects of long-term iloprost treatment on right ventricular function in patients with Eisenmenger syndrome. J Cardiol. 2017; 69 (5):741-46. https://doi.org/10.1016/j.jjcc.2016.07.002

268. Sitbon O., Channick R., Chin K.M., et al. Selexipag for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med. 2015; 373(26):2522-33. https://doi.org/10.1056/NEJMoa1503184

269. Beghetti M., Channick R.N., Chin K.M., et al. Selexipag treatment for pulmonary arterial hypertension associated with congenital heart disease after defect correction: insights from the randomised controlled GRIPHON study. Eur J Heart Fail. 2019; 21 (3): 352-59. https://doi.org/10.1002/ejhf.1375

270. Hascoet S, Fournier E, Jaïs X., et al. Outcome of adults with Eisenmenger syndrome treated with drugs specific to pulmonary arterial hypertension: A French multicentre study. Arch Cardiovasc Dis. 2017;110(5):303-316 https://doi.org/10.1016/j.acvd.2017.01.006

271. Li Q., Kuang H.Y., Wu Y.H., et al. What is the position of pulmonary arterial hypertension-specific drug therapy in patients with Eisenmenger syndrome: A systematic review and meta-analysis. Medicine (Baltimore). 2019; 98(20):e15632. https://doi.org/10.1097/MD.0000000000015632

272. D’Alto M, Romeo E, Argiento P, Sarubbi B, Santoro G, Grimaldi N et al. Bosentan-sildenafil association in patients with congenital heart disease-related pulmonary arterial hypertension and Eisenmenger physiology. Int J Cardiol. 2012; 155 (3):378-82. https://doi.org/10.1016/j.ijcard.2010.10.051

273. Varela D.L., Teleb M., El-Mallah W. Advanced therapies for the management of adults with pulmonary arterial hypertension due to congenital heart disease: a systematic review. Open Heart. 2018; 5(1):e000744. https://doi.org/10.1136/openhrt-2017-000744

274. Brida M., Gatzoulis M.A. Pulmonary arterial hypertension in adult congenital heart disease. Heart. 2018; 104 (19): 1568-74. https://doi.org/10.1136/heartjnl-2017-312106

275. Schuuring M.J., Bouma B.J., Cordina R., et al. Treatment of segmental pulmonary artery hypertension in adults with congenital heart disease. Int J Cardiol. 2013; 164 (1): 106-10. https://doi.org/10.1016/j.ijcard.2011.06.084

276. Hebert A, Mikkelsen UR, Thilen U, et al., Bosentan improves exercise capacity in adolescents and adults after Fontan operation: the TEMPO (Treatment With Endothelin Receptor Antagonist in Fontan Patients, a Randomized, Placebo-Controlled, Double-Blind Study Measuring Peak Oxygen Consumption) study. Circulation. 2014; 130(23):2021-30. https://doi.org/10.1161/CIRCULATIONAHA.113.008441

277. Agnoletti G., Gala S., Ferroni F., et al. Endothelin inhibitors lower pulmonary vascular resistance and improve functional capacity in patients with Fontan circulation. J Thorac Cardiovasc Surg. 2017; 153 (6): 1468-75. https://doi.org/10.1016/j.jtcvs.2017.01.051

278. Schuuring M.J., Vis J.C., van Dijk A.P., et al. Impact of bosentan on exercise capacity in adults after the Fontan procedure: a randomized controlled trial. Eur J Heart Fail. 2013; 15 (6): 690-98. https://doi.org/10.1093/eurjhf/hft017

279. Giardini A., Balducci A., Specchia S., et al. Effect of sildenafil on haemodynamic response to exercise and exercise capacity in Fontan patients. Eur Heart J. 2008; 29 (13): 1681-87. https://doi.org/10.1093/eurheartj/ehn215

280. Van De Bruaene A., La Gerche A., et al. Sildenafil improves exercise hemodynamics in Fontan patients. Circ Cardiovasc Imaging. 2014; 7 (2): 265-73. https://doi.org/10.1161/CIRCIMAGING.113.001243

281. Blok I.M., van Riel A.C.M.J., van Dijk A.P.J., et al. From bosentan to macitentan for pulmonary arterial hypertension and adult congenital heart disease: Further improvement? Int J Cardiol. 2017; 227: 51-52. https://doi.org/10.1016/j.ijcard.2016.11.211

282. Gelatt M., Hamilton R.M., et al. Arrhythmia and mortality after the Mustard procedure: a 30-year single-center experience. J Am Coll Cardiol. 1997; 29: 194-201. https://doi.org/10.1016/s0735-1097(96)00424-x

283. Dearani J.A., Danielson G.K., Puga F.J., et al. Late follow-up of 1095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits. Ann Thorac Surg. 2003;75:399-410. https://doi.org/10.1016/s0003-4975(02)04547-2

284. Giannakoulas G., Gatzoulis M.A. Pulmonary arterial hypertension in congenital heart disease: current perspectives and future challenges. Hellenic J Cardiol 2016; 57:218-22. https://doi.org/10.1016/j.hjc.2016.05.002

285. Van De Bruaene A., Toh N., et al. Pulmonary hypertension in patients with a subaortic right ventricle: prevalence, impact and management. Heart 2019;105:1471-78. https://doi.org/10.1136/heartjnl-2019-314756

286. De Pasquale G., Bonassin Tempesta F, Lopes BS, et al. High prevalence of baffle leaks in adults after atrial switch operations for transposition of the great arteries. Eur Heart J Cardiovasc Imaging. 2017; 18: 531-35. https://doi.org/10.1093/ehjci/jew276

287. Rydman R., Gatzoulis M.A., Ho S.Y., et al. Systemic right ventricular fibrosis detected by cardiovascular magnetic resonance is associated with clinical outcome, mainly new-onset atrial arrhythmia, in patients after atrial redirection surgery for transposition of the great arteries. Circ Cardiovasc Imaging. 2015; 8: e002628. https://doi.org/10.1161/CIRCIMAGING.114.002628

288. Zaragoza-Macias E., Zaidi A.N., Dendukuri N., Marelli A. Medical therapy for systemic right ventricles: a systematic review (part 1) for the 2018 AHA/ ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/ American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2019; 73: 1564-78. https://doi.org/10.1016/j.jacc.2018.08.1030

289. Voet A., Rega F., de Bruaene A.V., et al. Long-term outcome after treatment of isolated pulmonary valve stenosis. Int J Cardiol. 2012; 156: 11-15. https://doi.org/10.1016/j.ijcard.2010.10.038

290. Raissadati A., Nieminen H., Sairanen H., Jokinen E. Outcomes after the Mustard, Senning and arterial switch operation for treatment of transposition of the great arteries in Finland: a nationwide 4-decade perspective. Eur J Cardiothorac Surg. 2017; 52: 573-80. https://doi.org/10.1093/ejcts/ezx107

291. Hernandez-Madrid A., Paul T., Abrams D., et al. ESC Scientific Document Group. Arrhythmias in congenital heart disease: a position paper of the European Heart Rhythm Association (EHRA), Association for European Paediatric and Congenital Cardiology (AEPC), and the European Society of Cardiology (ESC) Working Group on Grown-up Congenital Heart Disease, endorsed by HRS, PACES, APHRS, and SOLAECE. Europace. 2018; 20:1719-53. https://doi.org/10.1093/europace/eux380

292. Redington A.N., Somerville J. Stenting of aortopulmonary collaterals in complex pulmonary atresia. Circulation. 1996; 94: 2479-84. https://doi.org/10.1161/01.cir.94.10.2479.

293. Hsu C.H., Gomberg-Maitland M., Glassner C. et al. The management of pregnancy and pregnancy-related medical conditions in pulmonary arterial hypertension patients. Int J Clin Pract Suppl. 2011; 172: 6-14. https://doi.org/10.1111/j.1742-1241.2011.02711.x

294. Daliento L., Somerville J., Presbitero P., et al. Eisenmenger syndrome. Factors relating to deterioration and death, Eur. Heart J. 1998; 19: 1845–55. https://doi.org/10.1053/euhj.1998.1046

295. Чазова И.Е., Мартынюк Т.В., Валиева З.С. и соавт. Евразийские клинические рекомендации по диагностике и лечению легочной гипертензии. Евразийский кардиологический журнал. 2020; 1:78-122 https://doi.org/10.38109/2225-1685-2020-1-78-122

296. Baumgartner H., De Backer J., Babu-Naraya S.V., et al. 2020 ESC Guidelines for the management of adult congenital heart disease: The Task Force for the management of adult congenital heart disease of the European Society of Cardiology (ESC). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Adult Congenital Heart Disease (ISACHD). Eur Heart J. 2021; Vol. 42 (6): 563–645.

297. Frost A., Badesch D., Simon J.R., et al. Diagnosis of pulmonary hypertension. Eur Respir J. 2019; 53: 1801904.

298. Galie N., Humbert M., Vachiery J.L., et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur. Heart J. 2016; 37: 67–119.

299. Мартынюк Т.В. Легочная гипертензия: диагностика и лечение. Сер. Библиотека ФГБУ «НМИЦ кардиологии» Минздрава России. М.: ООО «Медицинское информационное агентство», 2018. 304 с.

300. Чазова И.Е., Архипова О.А., Мартынюк Т.В. Легочная артериальная гипертензия в России: анализ шестилетнего наблюдения по данным Национального регистра. Терапевтический архив. 2019; 91 (1): 24-32.

301. Dimopoulos K., Wort S.J., Gatzoulis M.A. Pulmonary hypertension related to congenital heart disease: A call for action. Eur Heart J. 2014; 35: 691–700.

302. Lowe B.S., Therrien J., Ionescu-Ittu R., et al. Diagnosis of pulmonary hypertension in the congenital heart disease adult population: impact on outcomes. J Am Coll Cardiol. 2011; 58: 538–46.

303. Легочная гипертензия, в том числе хроническая тромбоэмболическая легочная гипертензия. Российские клинические рекомендации, 2020. http://cr.rosminzdrav.ru/#!/recomend/137

304. Kovacs G., Berghold A., Scheidl S., et al. Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review. Eur Respir J. 2009; 34 (4): 888–94.

305. Kaemmerer H., Apitz C., Brockmeier K., et al. Pulmonary hypertension in adults with congenital heart disease: Updated recommendations from the Cologne Consensus Conference 2018. Int J of Cardiology. 2018; 272: 79–88.

306. Горбачевский С.В., Шмальц А.А. Диагностика легочной гипертензии, ассоциированной с врожденными пороками сердца. Часть 1. Определение, классификация и первичное обследование пациентов. Российский вестник перинатологии и педиатрии. 2021; 66 (5):28-37.

307. Rosenzweig E.B., Abman S.H., Adatia I., et al. Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management. Eur Respir J. 2019; 53(1).

308. Simonneau G., Gatzoulis M.A., Adatia I., et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013; 62 (25 Suppl): D34-41.

309. Cantor W.J., Harrison D.A., Moussadji J.S., et al. Determinants of survival and length of survival in adults with Eisenmenger syndrome, Am J. Cardiol. 1999; 84: 677–81.

310. Galie N., Hoeper M.M., Humbert M., et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J. 2009; 30: 2493-537.

311. Stout K.K., Daniels C.J., Aboulhosn J.A., et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019; 139: e698-e800.

312. Бокерия Л.А., Горбачевский С.В., Шмальц А.А., соавт. Гемодинамическая коррекция функционально единственного желудочка сердца после ранее выполненного суживания легочной артерии. Грудная и сердечно-сосудистая хирургия. 2008; 5: 27-34.

313. Бокерия Л.А., Горбачевский С.В., Шмальц А.А. Легочная артериальная гипертензия, ассоциированная с врожденными пороками сердца у взрослых (клиническая рекомендация). Грудная и сердечно-сосудистая хирургия. 2017; 59 (2): 135-47.

314. Легочная гипертензия у детей. Российские клинические рекомендации, 2017. http://cr.rosminzdrav.ru/#!/recomend/901.

315. Гипертензионная сосудистая болезнь легких, ассоциированная с врожденными пороками сердца, у детей. Российские клинические рекомендации, 2018. http://cr.rosminzdrav.ru/#!/schema/356.

316. Cerro M.J., Abman S., Diaz G., et al. A consensus approach to the classification of pediatric pulmonary hypertensive vascular disease: Report from the PVRI Pediatric Taskforce, Panama 2011. Pulm Circ. 2011; 1 (2): 286–98.

317. Чазова И.Е., Мартынюк Т.В. Легочная гипертензия. Москва. Практика. 2015, 928 с.

318. Apitz C., Hansmann G., Schranz D. Hemodynamic assessment and acute pulmonary vasoreactivity testing in the evaluation of children with pulmonary vascular disease. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart. 2016; 102 (Suppl 2): ii23–9.

319. Moceri P., Kempny A., Liodakis E., et al. Physiological differences between various types of Eisenmenger syndrome and relation to outcome. Int J Cardiol. 2015; 179:455–460.

320. Kozlik-Feldmann G., Hansmann, D., et al. Pulmonary hypertension in children with congenital heart disease (PAH-CHD,PPHVD-CHD). Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK, Heart 2016; 102 (Suppl 2): i42-ii8.

321. van der Linde D., Konings E.E., Slager M.A., et al. Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J Am Coll Cardiol 2011; 58: 2241-47.

322. Liu Y., Chen S., Zuhlke L, et al. Global birth prevalence of congenital heart defects 1970-2017: updated systematic review and meta-analysis of 260 studies. Int J Epidemiol. 2019; 48:455-46.

323. Lytzen R., Vejlstrup N., Bjerre J., et al. Live-born major congenital heart disease in Denmark: incidence, detection rate, and termination of pregnancy rate from 1996 to 2013. JAMA Cardiol 2018; 3: 829-37.

324. Moons P., Bovijn L., Budts W., et al. Temporal trends in survival to adulthood among patients born with congenital heart disease from 1970 to 1992 in Belgium. Circulation. 2010; 122: 2264-72.

325. Marelli A.J., Ionescu-Ittu R., Mackie A.S., et al. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation. 2014; 130: 749-56.

326. Van de Bruaene A., Delcroix M., Pasquet A., et al. The Belgian Eisenmenger syndrome registry: Implications for treatment strategies? Acta Cardiol. 2009;64: 447–53.

327. van Riel A.C., Schuuring M.J., van Hessen I.D., et al. Contemporary prevalence of pulmonary arterial hypertension in adult congenital heart disease following the updated clinical classification. Int J Cardiol. 2014; 174: 299-305.

328. Manes A., Palazzini M., Leci E., et al. Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups. Eur Heart J. 2014; 35: 716-24.

329. McLaughlin V.V., Presberg K.W., Doyle R.L., et al. Prognosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004. 126 (1 Suppl):78S–92S.

330. Marelli A.J., Mackie A.S., Ionescu-Ittu R., et al. Congenital heart disease in the general population: Changing prevalence and age distribution. Circulation. 2007; 115: 163–72.

331. Duffels M.G., Engelfriet P.M., Berger R.M. et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol. 2007; 120(2 ):198-204.

332. Ramjug S., Hussain N., Hurdman J.. et al. Pulmonary arterial hypertension associated with congenital heart disease: Comparison of clinical and anatomic-pathophysiologic classification. J Heart Lung Transplant. 2016. 35(5): 610-18.

333. Diller G.P., Dimopoulos K., Kafka H., et al. Model of chronic adaptation: Right ventricular function in Eisenmenger syndrome. Eur. Heart J. Suppl. 2007; 9: H54–H60.

334. Alonso-Gonzalez R., Lopez-Guarch C.J., Subirana-Domenech M.T., et al. Pulmonary hypertension and congenital heart disease: An insight from the REHAP National Registry. Int J Cardiol. 2015; 184: 717-23.

335. Kempny A., Hjortshøj C.S., Gu H., et al. Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study. Circulation. 2017;135(15):1432-1440.

336. Morse J.H., Barst R.J., Fotino M. Familial pulmonary hypertension: immunogenetic findings in four Caucasian kindreds. Am Rev Respir Dis. 1992; 145:787–92.

337. Roberts K.E., McElroy J.J., Wong W.P., et. al. BMPR2 mutations in pulmonary arterial hypertension with congenital heart disease. Eur. Respir. J. 2004; 24: 371–74.

338. Бокерия Л.А., Горбачевский С.В., Шмальц А.А. Легочная артериальная гипертензия, ассоциированная с врожденными пороками сердца у взрослых. Методическое пособие. НЦССХ им. А. Н. Бакулева РАМН, 2016 г.

339. Хугаев Г.А., Шмальц А.А. Морфологическая оценка сосудов легких при легочной гипертензии, обусловленной врожденными пороками сердца. Архив патологии. 2021; 83 (5): 49–57.

340. Rabinovitch M. Molecular pathogenesis of pulmonary arterial hypertension. J Clin Invest. 2012; 122 (12): 4306-13.

341. Iacobazzi D., Suleiman M.S., Ghorbel M., et al. Cellular and molecular basis of RV hypertrophy in congenital heart disease. Heart. 2016; 102 (1): 12-7.

342. Bradford R., Tulloh R. Diagnosis and management of pulmonary hypertension in adult congenital heart disease. Br J Cardiac Nurs. 2008; 3:138–45.

343. Rubens C., Ewert R., Halank M., et al. Big endothelin-1 and endothelin-1 plasma levels are correlated with the severity of primary pulmonary hypertension. Chest. 2001. 120(5):1562-69.

344. Gorbachevsky Sv., Shmalts A.A. What can cause pulmonary vascular disease in functionally single ventricle? Anatomy & Physiology: Current Research. 2016; 6 (1). doi 10.4172/2161-0940.1000e137

345. Adatia I., Kothari S.S., Feinstein J.A. Pulmonary hypertension associated with congenital heart disease: pulmonary vascular disease: the global perspective. Chest 2010; 137 (6 Suppl): 52S-61S.

346. Шарыкин А.С. Врожденные пороки сердца. Руководство педиатров, кардиологов, неонатологов.-М.:Теремок.-2005.-381 С

347. Favoccia C., Constantine A.H., Wort S.J., et al. Eisenmenger syndrome and other types of pulmonary arterial hypertension related to adult congenital heart disease. Expert Review of Cardiovasc Therapy 2019; 17: 449-59.

348. Baumgartner H., Bonhoeffer P., De Groot N.M., et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). European Paediatric Cardiology (AEPC), ESC Committee for Practice Guidelines (CPG). Eur Heart J. 2010; 31(23): 2915-57.

349. Heath D., Edwards J.E. The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects. Circulation. 1958; 4 (Part 1): 533-47.

350. Yamaki S., Horiuchi T., Miura M., et al. Pulmonary vascular disease in secundum atrial septal defect with pulmonary hypertension. Chest. 1986; 89 (5): 694-8.

351. Heath D., Helmholz H.F. Jr, Burchell H., et al. Relation between structural change in the small pulmonary arteries and the immediate reversibility of pulmonary hypertension following closure of ventricular and atrial septal defects. Circulation. 1958; 18 (6):1167-74.

352. Есипова И.К. Некоторые вопросы патологии лёгких. АН СССР. 1962.

353. Haworth S.G., Rabinovitch M. Pulmonary circulation. In: Anderson RH, Baker EJ, Penny DJ, Redington AN, Rigby ML, Wernovsky G (eds). Paediatric Cardiology, 3rd edn. Churchill Livingstone, Elsevier, Philadelphia, PA, 2010: 117–141.

354. Rabinovitch M., Haworth S.G., Castaneda A.R., et al. Lung biopsy in congenital heart disease: a morphometric approach to pulmonary vascular disease. Circulation. 1978; 58(6): 1107-22.

355. Endo M., Yamaki S., Ohmi M., Tabayashi K. Pulmonary vascular changes induced by congenital obstruction of pulmonary venous return. Ann Thorac Surg. 2000; 69 (1):193-7.

356. Dimopoulos K., Diller G.P., Opotowsky A.R., et al. Definition and Management of Segmental Pulmonary Hypertension. J Am Heart Assoc. 2018;7 (14): e008587.

357. Thiene G., Frescura C., Bini R.M., et al. Histology of pulmonary arterial supply in pulmonary atresia with ventricular septal defect. Circulation. 1979; 60 (5):1066-74.

358. Van Dissel A.C., Mulder B.J., Bouma B.J. The Changing Landscape of Pulmonary Arterial Hypertension in the Adult with Congenital Heart Disease. J of Clin Med. 2017;. 6 (4): 40.

359. Горбачевский С.В., Шмальц А.А., Плотникова Л.Р. Легочная гипертензия у детей с врожденными пороками сердца. Москва. 2018.

360. Бокерия Л.А., Горбачевский С.В., Школьникова М.А. Легочная гипертензия у детей. Москва. Актелион фармасьютикалз; 2013.

361. Горбачевский С.В., Шмальц А.А. Гипертензионная сосудистая болезнь легких, ассоциированная с врожденными пороками сердца. В кн.: Бокерия Л.А., Шаталов К.В. Детская кардиохирургия. Руководство для врачей. Москва: НЦССХ им. А.Н. Бакулева; 2016: 833–50.

362. Hansmann G., Koestenberger M., Alastalo T.P., et al. 2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT. J Heart Lung Transplant. 2019; 38 (9):879-901.

363. Lammers A.E., Apitz C., Zartner P., et al. Diagnostics, monitoring and outpatient care in children with suspected pulmonary hypertension/paediatric pulmonary hypertensive vascular disease. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart. 2016; 102 (Suppl 2): ii1–13.

364. Dimopoulos K., Diller G.P. Pulmonary Hypertension in Adult Congenital Heart Disease. Springer; 2017. 368p.

365. Dimopoulos K., Prapa M., Gatzoulis M. Eisenmenger syndrome and other types of pulmonary arterial hypertension related to congenital heart disease. In: Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care. Springer; 2013.

366. Ikegami R., Ozaki K., Ozawa T., et al. Percutaneous Coronary Intervention for a Patient with Left Main Coronary Compression Syndrome. Intern Med. 2018; 57 (10):1421-4.

367. Butera G., Mansour N.B., De Marco F. Emergency transcatheter coronary intervention for left main compression secondary to pulmonary hypertension in a 4-year-old child. Catheter Cardiovasc Interv. 2019; 93(1):105-7.

368. Mamas M.A., Clarke B., Mahadevan V.S. Embolisation of systemic-to-pulmonary collaterals in patients with the Eisenmenger reaction presenting with haemoptysis. Cardiol Young. 2008; 18 (5):528-31.

369. Larici A.R., Franchi P., Occhipinti M., et al. Diagnosis and management of hemoptysis. Diagn Interv Radiol. 2014; 20 (4):299-309.

370. Broberg C.S., Ujita M., Prasad S. et al. Pulmonary arterial thrombosis in Eisenmenger syndrome is associated with biventricular dysfunction and decreased pulmonary flow velocity. J Am Coll Cardiol. 2007; 50 (7): 634–42.

371. Placik B., Rodbard S., McMahon J., Swaroop S. Pulmonary artery dissection and rupture in Eisenmenger’s syndrome. Vasc Surg. 1976; 10 (2):72-80.

372. Areco D., Pizzano N. Pulmonary artery dissection: echocardiographic findings and diagnosis. Echocardiography. 2003; 20 (4): 375-7.

373. Mikhail G.W., Gibbs J.S., Yacoub M.H. Pulmonary and systemic arterial pressure changes during syncope in primary pulmonary hypertension. Circulation. 2001; 104 (11): 1326-7.

374. Moledina S., Hislop A.A., Foster H., et al. Childhood idiopathic pulmonary arterial hypertension: a national cohort study. Heart. 2010; 96 (17):1401-6.

375. Чазова И. Е., Авдеев С. Н., Царева Н.А., соавт. Клинические рекомендации по диагностике и лечению легочной гипертонии. Терапевтический архив. 2014; 9: 4-23.

376. Kaemmerrer H., Koichiro N., Oechslin E., et al. Pulmonary Arterial Hypertension in Congenital Heart Disease: Eisenmenger’s Syndrome – A Global Perspectiv. 1st edition. Bremen: UNI-MED; 2013.

377. Oechslin E., Mebus S., Schulze-Neick I., et al. The Adult Patient with Eisenmenger Syndrome: A Medical Update after Dana Point Part III: Specific Management and Surgical Aspects. Cur Cardiol Rev. 2010; 6 (4): 363–72.

378. Labombarda F., Kerros H., Grollier G. Eisenmenger syndrome due to a large ductus arteriosus. Arch Cardiovasc Dis. 2010; 103 (11-12): 623-5.

379. Dimopoulos K. Eisenmenger syndrome in an adult patient with a large patent ductus arteriosus. Eur Respir Rev. 2013; 22(130): 558-64.

380. Рябыкина Г.В., Блинова Е.В., Сахнова Т.А. Электровекторкардиографическая диагностика гипертрофии правого желудочка у больных легочной гипертензией. Медицинский алфавит 2017; 8: 3-17.

381. Kamphuis V.P., Haeck M.L., Wagner G.S., et al. Electrocardiographic detection of right ventricular pressure overload in patients with suspected pulmonary hypertension. J Electrocardiol. 2014; 47 (2): 175-82.

382. Tongers J., Schwerdtfeger B., Klein G., et al. Incidence and clinical relevance of supraventricular tachyarrhythmias in pulmonary hypertension. Am Heart J. 2007; 153:127-32.

383. Юрпольская Л.А., Шмальц А.А. Рентгенография, рентгеновская компьютерная и магнитно-резонансная томография при легочной гипертензии, ассоциированной с врожденными пороками сердца. Детские болезни сердца и сосудов. 2021; 18 (2): 83-93.

384. Цаллагова З.С., Лазуткина В.К. Возможности традиционной рентгенографии в оценке легочной артериальной гипертензии. Медицинская визуализация. 2001; 4: 59-63.

385. Рентгенодиагностика заболеваний сердца и сосудов. /Ред. М.А. Иваницкая.-М., «Медицина», 1970.

386. Рентгенодиагностика в педиатрии. Руководство для врачей в 2-х томах. /Под ред.В.Ф.Баклановой, М.А.Филиппкина.-М.: Медицина,-1988.-Т.1.-448 С.

387. Детская кардиология. / Под ред. Дж.Хоффмана. Пер.с англ.-М.: Практика.-2006.-543С

388. Коробкова И.З., Лазуткина В.К., Низовцова Л.А. и др. Методические аспекты рентгенологической оценки легочной гипертензии. Вестник рентгенологии и радиологии. 2015. 4: 45-53

389. Dimopoulos K., Giannakoulas G., Bendayan I., et al. Cardiothoracic ratio from postero-anterior chest radiographs: a simple, reproducible and independent marker of disease severity and outcome in adults with congenital heart disease. Int J Cardiol. 2013.166:453-7.

390. Freedom R.M., Yoo Shi-joon, Mikailian H., Williams W.G. The Natural and Modified History of Congenital Heart Disease. 2008. Wiley-Blackwell-904 p.

391. Freedom R. M., Benson L.N., Smallhorn J.F. Neonatal Heart Disease. Springer-Verlag London 1992, 881p.

392. Koestenberger M., Apitz C., Abdul-Khaliq H., Hansmann G. Transthoracic echocardiography for the evaluation of children and adolescents with suspected or confirmed pulmonary hypertension. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and D6PK. Heart. 2016; 102 (Suppl 2): ii14-22.

393. Anderson R.H., Baker E.J., Penny D.J., et al. Paediatric cardiology. 3d edition. Elsevier, 2009.

394. Барышникова И.Ю., Шмальц А.А. Диагностика легочной гипертензии, ассоциированной с врожденными пороками сердца. Часть 2. Эхокардиография. Российский вестник перинатологии и педиатрии. 2021; 66(6): 23–32.

395. Li W., West C., McGhie J., et al. Consensus recommendations for echocardiography in adults with congenital heart defects from the International Society of Adult Congenital Heart Disease (ISACHD). Int J Cardiol. 2018; 272:77-83.

396. Augustine D.X., Coates-Bradshaw L.D., Willis J., et al. BSE pulmonary hypertension guideline 2018; 5 (3): G11–G24.

397. Rudski LG., Lai W.W., Afi lalo J., et al. Guidelines for the Echocardiographic Assessment of the Right Heart in Adults: A Report from the American Society of Echocardiography Endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. J Am Soc Echocardiogr. 2010; 23: 685-713.

398. Zaidi A., Knight D.S., Augustine D.X., et al. Echocardiographic assessment of the right heart in adults: a practical guideline from the British Society of Echocardiography. 2020; 7 (1): G19–G41.

399. Amsallem M., Sternbach J.M., Adigopula S., et al. Addressing the controversy of estimating pulmonary arterial pressure by echocardiography. J Am Soc Echocardiogr. 2016; 29: 93–102.

400. Fisher M.R., Forfia P.R., Chamera E., et al. Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension. Am J Respir Crit Care Med. 2009; 179 (7): 615-21.

401. Rimington H., Chambers J.B. Echocardiography: a practical guide for reporting and interpretation. Third edition. CRC Press; 2016.

402. Lang R.M., Badano L.P., Mor-Avi V., et al. Recommendations for Cardiac Chamber Quantification by Echocardiography in Adults: An Update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Society of Echocardiography. 2015; 28 (1): 1–39.

403. Abraham S., Weismann C.G. Left ventricular end-systolic eccentricity index for assessment of pulmonary hypertension in infants. Echocardiography. 2016; 33: 910–15.

404. Nagueh S.F., Smiseth O.A., Appleton C.P., et al. Recommendations for the Evaluation of Left Ventricular Diastolic Function by Echocardiography: An Update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr. 2016; 29:277-314.

405. Friedberg M., Merten L. Tissue velocities, strain, and strain rate for echocardiographic assessment of ventricular function in congenital heart disease. European J Echocardiography. 2009; 10: 585-93.

406. Lai W.W., Mertens L.L., Cohen M.S., Geva T. Echocardiography in pediatric and congenital heart disease. From Fetus to Adult. Second Edition. Wiley Blackwell; 2016.

407. Moceri P., Li W., Dimopoulos K. Echocardiography in the Diagnosis and Follow-Up of Patients with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease. In: Dimopoulos K, Diller GP (eds). Pulmonary Hypertension in Adult Congenital Heart Disease. Springer; 2017.

408. Бокерия ЛА, Барышникова И.Ю., Горбачевский С.В. Эхокардиография в диагностике и оценке легочной гипертензии. Учебное пособие. Москва. 2017.

409. Mertens L., Friedberg M.K. Systolic ventricular function. In: Echocardiography in Pediatric and Congenital Heart Disease: From Fetus to Adult; 2016, Hoboken, US: Wiley-Blackwell, p.96-131.

410. Gurudevan S.V., Malouf P.J., Kahn A.M., et al. Noninvasive assessment of pulmonary vascular resistance using Doppler tissue imaging of the tricuspid annulus. J Am Soc Echocardiogr. 2007; 20 (10):1167–71.

411. Sutherland G.R., Di Salvo G., Claus P., et al. Strain and strain rate imaging: a new clinical approach to quantifying regional myocardial function. J Am Soc Echocardiogr 2004; 17: 788-802.

412. D’Alto M., Dimopoulos K., Budts W., et al. Multimodality imaging in congenital heart disease-related pulmonary arterial hypertension. Heart. 2016; 102 (12): 910-18.

413. Puchalski M.D., Lui G.K., Miller-Hance W., et al. Guidelines for Performing a Comprehensive Transesophageal Echocardiographic. Journal of the American Society of Echocardiography. 2019; 32 (2): 173–215.

414. Шария А.М., Мартынюк Т.В., Терновой С.К., Шария М.А. Возможности магнитно-резонансной томографии в диагностике легочной артериальной гипертензии. Кардиология. 2021;61(6):97-104.

415. Бокерия Л.А., Макаренко В.Н., Юрпольская Л.А. Компьютерная и магнитно-резонансная томография в диагностическом алгоритме врожденных пороков сердца: Что? Когда? Кому?-“за” и “против”. Грудная и сердечно-сосудистая хирургия 2014; 3: 4-13.

416. Latus H., Kuehne T., Beerbaum Ph., et all. Cardiac MR and CT imaging in children with suspected or confirmed pulmonary hypertension/ pulmonary hypertensive vascular disease. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK. Heart. 2016; 102: ii30–ii35.

417. van Wolferen S.A., Marcus J.T., Boonstra A., et al. Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension. Eur Heart J. 2007. 28:1250-7.

418. Belokon N.A., Podzolkov V.P., Mitrofanov I.O. Congenital heart disease .-M .: Medicine .-1990.-352p. (in Russian).

419. Brignole M., Auricchio A., Baron-Esquivias G., et al. 2013 ESC Guidelines on cardiac pacing and cardiac resynchronization therapy: the Task Force on cardiac pacing and resynchronization therapy of the European Society of Cardiology (ESC). Developed in collaboration with the European Heart Rhythm Association (EHRA). Eur Heart J. 2013; 34: 2281-329.

420. Radenkovic D., Weingärtner S., Ricketts L., et al. T1 mapping in cardiac MRI. Heart Fail Rev. 2017; 22:415–430,

421. Salerno M., Sharif B., Arheden H., et al. Recent Advances in Cardiovascular Magnetic Resonance Techniques and Applications. Cir.cCardiovasc.Imaging. 2017; 10 :e003951.

422. European Medicines Agency. EMA’s final opinion confirms restrictions on use of linear gadolinium agents in body scans. https://www.ema.europa.eu/en/documents/referral/gadolinium-article-31-referral-emas-final-opinion-confirms-restric

423. Odagiri K., Inui N., Hakamata A., et al. Noninvasive evaluation of pulmonary arterial blood flow and wall shear stress in pulmonary arterial hypertension with 3D phase contrast magnetic resonance imaging. Springer Plus 2016; 5: 1071

424. Torbicki A. Cardiac magnetic resonance in pulmonary arterial hypertension: a step in the right direction. Eur Heart J 2007; 28: 1187-1189.

425. Perloff J.K., Marelli A. Perloff’s Clinical Recognition of Congenital Heart Disease: Expert Consult Online and Print, 6e. 6th edition. Philadelphia: Saunders; 2012

426. de Siqueira M.E.M.., Pozo E., Fernandes V.R., et all. Characterization and clinical significance of right ventricular mechanics in pulmonary hypertension evaluated with cardiovascular magnetic resonance feature tracking. J of Cardiovasc Magn Res-2016; doi 10.1186/s12968-016-0258-x

427. Tello K., Dalmer A., Vanderpool R., et al. Cardiac Magnetic Resonance Imaging-Based Right Ventricular Strain Analysis for Assessment of Coupling and Diastolic Function in Pulmonary Hypertension. JACC Cardiovasc Imaging. 2019;12 (1): 2155-64.

428. Рентгенодиагностика заболеваний сердца и сосудов. Ред. М.А. Иваницкая. М., «Медицина», 1970.

429. Макаренко В.Н., Юрпольская Л.А., Зеленикин М.М., Шинкарева Т.В. Компьютерная томография в оценке пациентов после операции Фонтена. Бюллетень НЦССХ им. А.Н.Бакулева РАМН. 2010; 11 (1): 46-50.

430. Kilner P., Nichol E., Rubens M. The roles of CT and CMR in Adult Congenital Heart Disease. In: ESC Textbook of Cardiovascular Imaging. 2nd ed, Oxford:Oxford University Press; 2015, p. 563-600.

431. Grosse-Wortmann L., Drolet Chr., Dragulescu An., et al. Aortopulmonary collateral flow volume affects early postoperative outcome after Fontan completion: A multimodality study. J. Thorac. Cardiovasc. Surg. 2012; 144: 1329-36.

432. Habib G., Lancellotti P., Antunes M.J., et al. ESC Scientific Document Group. 2015 ESC Guidelines for the management of infective endocarditis: The Task Force for the Management of Infective Endocarditis of the European Society of Cardiology (ESC). Endorsed by: European Association for Cardio-Thoracic Surgery (EACTS), the European Association of Nuclear Medicine (EANM). Eur Heart J. 2015; 36: 3075-3128.

433. Ohno Y., Koyama H., Lee H.Y., Miura S.t all. Contrast-enhanced CT-and MRI-based perfusion assessment for pulmonary diseases: basics and clinical applications. Diagn Interv Radiol-2016; 22: 407–421

434. Pienn M., Kovacs G., Tscherner M., Avian A. et all. Non-invasive determination of pulmonary hypertension with dynamic contrast-enhanced computed tomography: a pilot study. Eur Radiol.-2014; 24:668–676

435. Соболев А.В., Шмальц А.А. Эндоваскулярная диагностика легочной гипертензии, ассоциированной с врожденными пороками сердца. Часть 1. Катетеризация сердца и ангиокардиография. Эндоваскулярная хирургия. 2021; 8 (3): 263–71.

436. Guillinta P., Peterson K.L., Ben-Yehuda O. Cardiac catheterization techniques in pulmonary hypertension. Cardiol Clin. 2004; 22: 401–15.

437. Peterson K.L., Nicod P. Cardiac catheterization: methods, diagnosis, and therapy. Saunders, Philadelphia; 1997

438. Barnett C., Ben-Yehuda О. Cardiac Catheterization in the Patient with Pulmonary Hypertension. Textbook of Pulmonary Vascular Disease Springer, Science+Business Media, LLC; 2011

439. Горбачевский С.В., Белкина М.В., Колединский Д.Г., соавт. Инвазивный мониторинг давления в легочной артерии — объективный метод оценки степени тяжести легочной гипертензии у детей с врожденными пороками сердца. Детские болезни сердца и сосудов. 2006; 4: 77–81.

440. Wilkinson J.L. The Fontan сirculation. Results, late Follow-up and management. Heart Views. 2004; 4 (4): 73-78.

441. Fredenburg T.B., Johnson T.R., Cohen M.D. The Fontan Procedure: Anatomy, Complications, and Manifestations of Failure. RadioGraphics. 2011;31(2): 453–463.

442. Bergersen L., Foerster S., Marshall A.C., Meadows J. (eds). Congenital heart disease: the catheterization manual. Springer, New York; 2009.

443. Бокерия Л.А., Алекян Б.Г. (ред.). Руководство по рентгеноэндоваскулярной хирургии сердца и сосудов. Москва, НЦССХ им. А.Н. Бакулева РАМН; 2008

444. Sitbon O., Humbert M., Jais X., et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation. 2005; 111: 3105–11.

445. Barst R.J. Pharmacologically induced pulmonary vasodilatation in children and young adults with primary pulmonary hypertension. Chest. 1986; 89: 497–503.

446. Rich S., Kaufmann E., Levy P.S. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med. 1992; 327: 76–81.

447. Douwes J.M., van Loon RLE, Hoendermis E.S, et al. Acute pulmonary vasodilator response in paediatric and adult pulmonary arterial hypertension: occurrence and prognostic value when comparing three response criteria. Eur Heart J. 2011; 32 (24): 3137-46.

448. Chessa M. Cardiac Catheterization. In: Dimopoulos K, Diller G.P. (eds). Pulmonary Hypertension in Adult Congenital Heart Disease. Springer; 2017.

449. Hoeper M.M., Lee S.H., Voswinckel R., et al. Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J Am Coll Cardiol. 2006; 48: 2546–52.

450. Diller G.P., Dimopoulos K., Okonko D. et al. Exercise intolerance in adult congenital heart disease: Comparative severity, correlates, and prognostic implication. Circulation. 2005. 112:828–35.

451. Savarese G., Paolillo S., Costanzo P., et al. Do changes of 6-minute walk distance predict clinical events in patients with pulmonary arterial hypertension?: a meta-analysis of 22 randomized trials. J Am Coll Cardiol 2012; 60: 1192–1201.

452. Gabler N.B., French B., Strom B.L, et al. Validation of 6-minute walk distance as a surrogate end point in pulmonary arterial hypertension trials. Circulation 2012; 126: 349–56.

453. Глушко Л.А., Шмальц А.А. Оценка состояния кардиореспираторной системы при легочной гипертензии, ассоциированной с врожденными пороками сердца. Креативная кардиология. 2021; 15 (2): 167-179.

454. Arena R., Lavie C.J., Milani R.V., et al. Cardiopulmonary exercise testing in patients with pulmonary arterial hypertension: an evidence-based review. J Heart Lung Transplant. 2010; 29 (2): 159-73.

455. Johnson M.K., Thompson S. The role of exercise testing in the modern management of pulmonary arterial hypertension. Diseases. 2014; 2: 120-47.

456. Wasserman K., Hansen J.E., Sue D.Y., et al. Principles of exercise testing and interpretation: including pathophysiology and clinical applications. Fifth edition. Philadelphia: Lippincott Williams & Wilkins; 2012, 572 p.

457. Seeger W., Adir Y., Barbera J.A., et al. Pulmonary hypertension in chronic lung diseases. J Am Coll Cardiol. 2013; 62: D109–16.

458. Johnson J.T., Yetman A.T. Cardiopulmonary exercise testing in adults with congenital heart disease. Prog Pediatr Cardiol. 2012; 34: 47–52.

459. Laveneziana P., Garcia G., Joureau B. et al. Dynamic respiratory mechanics and exertional dyspnoea in pulmonary arterial hypertension. Eur Respir J. 2013. 41:578-587.

460. Ferrazza A.M., Martolini D., Valli G., Palange P. Cardiopulmonary exercise testing in the functional and prognostic evaluation of patients with pulmonary diseases. Respiration. 2009; 77 (1): 3-17.

461. Mantegazza V., Apostolo A, Hager A. Cardiopulmonary Exercise Testing in Adult Congenital Heart Disease. Ann Am Thorac Soc. 2017; 14 (Suppl.1): S93-S101.

462. Diller G.P., Kempny A., Inuzuka R., et al. Survival prospects of treatment naïve patients with Eisenmenger: a systematic review of the literature and report of own experience. Heart. 2014; 100(17): 1366-72.

463. Buys R., Cornelissen V., Van De Bruaene A., et al. Measures of exercise capacity in adults with congenital heart disease. Int J Cardiol. 2011; 153 (1): 26-30.

464. Таран И.Н., Валиева З.С., Мартынюк Т.В., и соавт. Спировелоэргометрия в стратификации риска пациентов с легочной артериальной гипертензией и хронической тромбоэмболической легочной гипертензией. Евразийский кардиологический журнал. 2017; 4: 86-92.

465. Zhao Q.H., Wang L., Pudasaini B., et al. Cardiopulmonary exercise testing improves diagnostic specificity in patients with echocardiography-suspected pulmonary hypertension. Clin Cardiol. 2017; 40 (2): 95-101.

466. Чазова И.Е., Мартынюк Т.В. и соавт. Диагностика и лечение легочной гипертензии. Российские рекомендации. Кардиоваскулярная терапия и профилактика 2007; 6: Приложение 2.

467. Sun X.G., Hansen J.E., Oudiz R.J, Wasserman K. Pulmonary function in primary pulmonary hypertension. J Am Coll Cardiol 2003; 41:1028–35.

468. Alonso-Gonzalez R., Borgia F., Diller G.P., et al. Abnormal lung function in adults with congenital heart disease: prevalence, relation to cardiac anatomy, and association with survival. Circulation. 2013; 127 (8): 882-90.

469. Ferguson G.T., Enright P.L., Buist A.S., Higgins M.W. Office spirometry for lung health assessment in adults: A consensus statement from the National Lung Health Education Program. Chest. 2000;117 (4): 1146-61.

470. Архипова О.А., Мартынюк Т.В., Самойленко Л.Е., соавт. Перфузионная сцинтиграфия легких у больных с легочной гипертензией различной этиологии. Евразийский кардиологический журнал. 2015; 4: 21-25. https://doi.org/10.38109/2225-1685-2015-4-21-25

471. Tunariu N., Gibbs S.R., Win Z., et al. Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension. J Nucl Med. 2007; 48: 680-84.

472. Albrecht T., Blomley M.J., Cosgrove D.O., et al. Non-invasive diagnosis of hepatic cirrhosis by transit-time analysis of an ultrasound contrast agent. Lancet. 1999; 353: 1579-83.

473. Perloff J.K. Systemic complications of cyanosis in adults with congenital heart disease. Hematologic derangements, renal function, and urate metabolism. Cardiol Clin 1993; 11: 689-99.

474. Giannakoulas G., Mouratoglou S., Gatzoulis M.A.. et al. Blood biomarkers and their potential role in pulmonary arterial hypertension associated with congenital heart disease. A systematic review. International Journal of Cardiology. 2014; 174 (3): 618–23.

475. Fenster B.E., Lasalvia L, Schroeder J.D., et al. Cystatin C for pulmonary hypertension. Respirology. 2014. 19: 583-89.

476. Scognamiglio G., Kempny A., Price L.C., et al. C-reactive protein in adults with pulmonary arterial hypertension associated with congenital heart disease and its prognostic value. Heart. 2014. 100:1335-1341.

477. Dimopoulos K., Okonko D.O., Diller G.P., et al. Abnormal ventilatory response to exercise in adults with congenital heart disease relates to cyanosis and predicts survival. Circulation 2006; 113 (24): 2796-802.

478. Deanfield J., Thaulow E., Warnes C., et al. Management of grown-up congenital heart disease. Eur Heart J. 2003; 24: 1035-84.

479. Yang-Ting S., Aboulhosn J., Sun X.G. et al. Effects of pulmonary vasodilator therapy on ventilatory efficiency during exercise in adults with Eisenmenger syndrome. Congenit Heart Dis. 2011; 6 (2): 139-46.

480. Dimopoulos K., Inuzuka R., Goletto S., et al. Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation. 2010; 121: 20-25.

481. Diller G.P., Korten M.A., Bauer U.M., et al. German Competence Network for Congenital Heart Defects Investigators. Current therapy and outcome of Eisenmenger syndrome: data of the German National Register for congenital heart defects. Eur Heart J 2016; 37: 14491455.

482. Oster M., Bhatt A., Zaragoza-Macias E., et al. Interventional therapy versus medical therapy for secundum atrial septal defect: a systematic review (part 2) for the 2018 AHA/ACC guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/ American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019; 139: e000–000.

483. Shah D., Azhar M., Oakley C.M., et al. Natural history of secundum atrial septal defect in adults after medical or surgical treatment: a historical prospective study. Br Heart J. 1994; 71: 224–27.

484. Cho Y.H., Jun T-G., Yang J-H., et al. Surgical strategy in patients with atrial septal defect and severe pulmonary hypertension. Heart Surg Forum. 2012; 15:E111–5. S4.1.1-21.

485. Kouchoukos N.T., Blackstone E.H., Kirklin J.W. Surgical implications of pulmonary hypertension in congenital heart disease. Adv Cardiol. 1978: S4: 225–31

486. Yan C., Zhao S., Jiang S., et al. Transcatheter closure of patent ductus arteriosus with severe pulmonary arterial hypertension in adults. Heart. 2007; 93: 514–18.

487. Gamboa R., Rios-Méndez R.E., Mollón F.P., et al. Percutaneous closure of patent ductus arteriosus in adults using different devices. Rev Esp Cardiol. 2010; 63: 726–29.

488. Jeong Y-H., Yun T-J., Song J-M., et al. Left ventricular remodeling and change of systolic function after closure of patent ductus arteriosus in adults: device and surgical closure. Am Heart J. 2007; 154: 436–40.

489. Zabal C., García-Montes J.A., Buendía-Hernández A., et al. Percutaneous closure of hypertensive ductus arteriosus. Heart. 2010; 96: 625–29.

490. Lim D.S., Forbes T.J., Rothman A., et al. Transcatheter closure of high-risk muscular ventricular septal defects with the CardioSEAL occluder: initial report from the CardioSEAL VSD registry. Catheter Cardiovasc Interv. 2007; 70 (5): 740-44.

491. Chessa M., Butera G., Negura D., et al. Transcatheter closure of congenital ventricular septal defects in adult: mid-term results and complications. Int J Cardiol. 2009; 133 (1):70-3.

492. Горбачевский С.В., Шмальц А.А. Паллиативное предсердное и артериальное переключение при транспозиции магистральных артерий с дефектом межжелудочковой перегородки и тяжелой легочно-сосудистой болезнью. Детские болезни сердца и сосудов. 2019; 16 (2): 86-94.

493. Петросян К.В., Горбачевский С.В., Дадабаев Г.М., Пардаев Д.Б. Стентирование ствола левой коронарной артерии у пациентки с легочной гипертензией. Евразийский кардиологический журнал. 2017; 3: 86-87.

494. Gorbachevsky S.V., Shmalts A.A., Dadabaev G.M., et al. Outcomes of Atrioseptostomy with Stenting in Patients with Pulmonary Arterial Hypertension from a Large Single-Institution Cohort. Diagnostics (Basel). 2020; 10(9): 725.

495. Горбачевский С.В., Пурсанов М.Г., Шмальц А.А., соавт. Результаты атриосептостомии со стентированием у больных с идиопатической и схожими формами легочной артериальной гипертензии. Грудная и сердечно-сосудистая хирургия. 2019; 61 (2): 100-113.

496. Шмальц А.А., Нишонов Н.А. Атриосептостомия у больных с легочной гипертензией. Грудная и сердечно-сосудистая хирургия. 2015; 57 (5): 18-25.

497. Christie J.D., Edwards L.B., Kucheryavaya A.Y. et al. The Registry of the International Society for Heart and Lung Transplantation: 29th official adult lung and heart-lung transplant report 2012. J. Heart Lung Transplant. 2012; 31:1073–86.

498. Fadel E., Mercier O., Mussot S., et al. Long-term outcome of double-lung and heart-lung transplantation for pulmonary hypertension: a comparative retrospective study of 219 patients. Eur J Cardiothorac Surg. 2010; 38: 277–84.

499. Hopkins W.E., Ochoa L.L., Richardson G.W., et al. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. J Heart Lung Transplant. 1996; 15 (1):100-5.

500. Moons P., De Geest S., Budts W. Comprehensive care for adults with congenital heart disease: expanding roles for nurses. Eur J Cardiovasc Nurs. 2002; 1: 23-28.

501. Baumgartner H., Budts W., Chessa M., et al, Working Group on Grown-up Congenital Heart Disease of the European Society of Cardiology. Recommendations for organization of care for adults with congenital heart disease and for training in the subspecialty of ‘Grown-up Congenital Heart Disease’ in Europe: a position paper of the Working Group on Grown-up Congenital Heart Disease of the European Society of Cardiology. Eur Heart J. 2014; 35: 686-90.

502. Thomet C., Moons P., Budts W., et al. ESC Working Group on Grown-up Congenital Heart Disease. Staffing, activities, and infrastructure in 96 specialised adult congenital heart disease clinics in Europe. Int J Cardiol 2019; 292: 100-105.

503. Popelová J., Oechslin E., Kaemmerer H., Sutton M. Congenital Heart Disease in Adults 2008 Informa UK Ltd.-183p.

504. Regitz-Zagrosek V., Roos-Hesselink J.W., Bauersachs J., et al. ESC Scientific Document Group. 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy. Eur Heart J. 2018; 39: 31653241.

505. Sliwa K., van Hagen I.M., Budts W., et al. Pulmonary hypertension and pregnancy outcomes: data from the Registry Of Pregnancy and Cardiac Disease (ROPAC) of the European Society of Cardiology. Eur J Heart Fail. 2016; 18 (9): 1119–28.

506. Jaı̈s X., Olsson K.M, Barbera J.A., et al. Pregnancy outcomes in pulmonary arterial hypertension in the modern management era. Eur Respir J. 2012; 40: 881–85.

507. Kovacs A.H., Sears S.F., Saidi A.S. Biopsychosocial experiences of adults with congenital heart disease: review of the literature. Am Heart J 2005; 150: 193-201.

508. Raines D.E., Liberthson R.R., Murray J.R. Anesthetic management and outcome following noncardiac surgery in nonparturients with Eisenmenger’s physiology. J Clin Anesth. 1996; 8: 341–47.

509. Grünig, E., Benjamin, N., Krüger, U., et al. General measures and supportive therapy for pulmonary arterial hypertension: Updated recommendations from the Cologne Consensus Conference 2018.

510. Blanche C., Alonso-Gonzalez R., Uribarri A. et al. Use of intravenous iron in cyanotic patients with congenital heart disease and/or pulmonary hypertension. Int J Cardiol. 2018. 267:79-83.

511. Cohn J.N. Optimal diuretic therapy for heart failure. Am J Med 2001; 111: 577.

512. Olsson K.M., Delcroix M., Ghofrani H.A., et al. Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA). Circulation. 2014; 129: 57–65.

513. Kirchhof P., Benussi S., Kotecha D., et al. 2016 ESC Guidelines for the management of atrial fibrillation developed in collaboration with EACTS. Eur Heart J 2016; 37: 2893-962.

514. Steffel J., Verhamme P,. Potpara T.S., et al. ESC Scientific Document Group. The 2018 European Heart Rhythm Association Practical Guide on the use of non-vitamin K antagonist oral anticoagulants in patients with atrial fibrillation. Eur Heart J. 2018; 39:1330-93.

515. Pujol C., Niesert A.C., Engelhardt A., et al. Usefulness of direct oral anticoagulants in adult congenital heart disease. Am J Cardiol. 2016; 117: 450-55.

516. Yang H., Bouma B.J., Dimopoulos K., et al. Non-vitamin K antagonist oral anticoagulants (NOACs) for thromboembolic prevention, are they safe in congenital heart disease? Results of a worldwide study. Int J Cardiol. 2019; 299: 123-30.

517. Sandoval J., Aguirre J.S., Pulido T., et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med. 2001; 164:1682–87.

518. Weitzenblum E., Sautegeau A., Ehrhart M., et al. Long-term oxygen therapy can reverse the progression of pulmonary hypertension in patients with chronic obstructive pulmonary disease. Am Rev Respir Dis. 1985; 131: 493-98.

519. van de Veerdonk M.C., Kind T., Marcus J.T., et al. Progressive right ventricular dysfunction in patients with pulmonary arterial hypertension responding to therapy. J Am Coll Cardiol. 2011; 58: 2511-19.

520. Rich S., Seidlitz M., Dodin E., et al. The short-term effects of digoxin in patients with right ventricular dysfunction from pulmonary hypertension. Chest. 1998; 114: 787-92.

521. Архипова О.А., Мартынюк Т.В., Чазова И.Е. Особенности тактики ведения и выбора специфической терапии у взрослых пациентов с легочной артериальной гипертензией, ассоциированной с врожденными пороками сердца. Евразийский кардиологический журнал. 2018. 1: 48-53.

522. Шмальц А.А., Горбачевский С.В. Доказательная база специфических легочных вазодилататоров у детей с врожденными пороками сердца. Педиатрия. Журнал им. Г.Н. Сперанского. 2017: 96 (6):126-139.

523. Шмальц А.А., Горбачевский С.В. Доказательная база специфических легочных вазодилататоров у взрослых с врожденными пороками сердца. Терапевтический архив. 2021; 93 (9): 1106-1116.

524. Шмальц А.А., Белкина М.В., Горбачевский С.В. Специфические легочные вазодилататоры после операции Фонтена. Детские болезни сердца и сосудов. 2017; 14 (1): 16-24.

525. Траклир®. Инструкция по применению лекарственного препарата для медицинского применения. ЛС-002509. https://grls.rosminzdrav.ru/Grls_View_v2.aspx?routingGuid=93619b5d-c8c6-4bdb-b201-88aff22c785c&t=

526. Бозенекс. Инструкция по медицинскому применению применению лекарственного препарата. ЛП-004208. https://grls.rosminzdrav.ru/Grls_View_v2.aspx?routingGuid=68d79ec9-1045-403c-be20-e88cc33f0aa6&t=

527. Опсамит®. Инструкция по применению лекарственного препарата для медицинского применения. ЛП-003310. https://grls.rosminzdrav.ru/Grls_View_v2.aspx?routingGuid=b2c51f9d-696d-4b23-881a-8c8d0fec4b96&t=

528. Апбрави. Инструкция по применению лекарственного препарата для медицинского применения.ЛП-005577. https://grls.rosminzdrav.ru/Grls_View_v2.aspx?routingGuid=094dda8d-ba79-4353-83f9-98ea68b865aa&t=

529. Manes A. PAH in Patients with Prevalent Systemic–Pulmonary Shunts and PAH in Patients with Small Cardiac Defects. In: Dimopoulos K, Diller GP (eds). Pulmonary Hypertension in Adult Congenital Heart Disease. Springer; 2017

530. Idorn L., Sondergaard L. Pulmonary Vascular Disease in Patients with Fontan-Type Circulation. In: Dimopoulos K, Diller GP (eds). Pulmonary Hypertension in Adult Congenital Heart Disease. Springer; 2017.

531. Moledina Sh., Theocharis P. Pulmonary Arterial Hypertension in Patients with Previous Reparative Surgery. In: Dimopoulos K, Diller GP (eds). Pulmonary Hypertension in Adult Congenital Heart Disease. Springer; 2017.

532. Шмальц А.А., Горбачевский С.В. Возможности и ограничения для начальной комбинированной специфической терапии легочной артериальной гипертензии в Российской Федерации. Терапевтический архив. 2020; 92 (12): 80-85.

533. D’Alto M., Diller G.P. Pulmonary hypertension in adults with congenital heart disease and Eisenmenger syndrome: current advanced management strategies. Heart. 2014; 100 (17):1322-28.

534. Чазова И.Е., Мартынюк Т.В., Наконечников С.Н. Антагонисты рецепторов эндотелина при легочной артериальной гипертензии: вчера, сегодня и завтра. Российский кардиологический журнал 2009; 4:73–81.

535. Galie N., Manes A., Palazzini M., et al. Management of pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunts and Eisenmenger’s syndrome. Drugs. 2008. 68:1049–66.

536. Rubin L.J., Badesch D.B., Barst R.J., et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002; 346: 896–903.

537. Galie N., Beghetti M., Gatzoulis M.A., et al. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation. 2006; 114 (1): 48–54.

538. Gatzoulis M.A., Beghetti M., Galie N., et al. Long-term bosentan therapy improves functional capacity in Eisenmenger syndrome: Results of the BREATHE-5 open-label extension study. Int J Cardiol. 2008; 127 (1): 27-32.

539. Galiè N., Rubi.n L., Hoeper M., et al. Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet. 2008; 371 (9630): 2093-100.

540. Sitbon O., Beghetti M., Petit J., et al. Bosentan for the treatment of pulmonary arterial hypertension associated with congenital heart defects. Eur J Clin Invest. 2006; 36 Suppl 3: 25-31.

541. Benza R.L., Rayburn B.K., Tallaj J.A., et al. Efficacy of bosentan in a small cohort of adult patients with pulmonary arterial hypertension related to congenital heart disease. Chest. 2006; 129 (4):1009-15.

542. Diller G.P., Dimopoulos K., Kaya M.G., et al. Long-term safety, tolerability and efficacy of bosentan in adults with pulmonary arterial hypertension associated with congenital heart disease. Heart. 2007; 93 (8): 974-76.

543. Durongpisitkul K., Jakrapanichakul D., Sompradikul S. A retrospective study of bosentan in pulmonary arterial hypertension associated with congenital heart disease. J Med Assoc Thai. 2008; 91 (2): 196-202.

544. Duffels M.G., Vis J.C., van Loon R.L, et al. Effect of bosentan on exercise capacity and quality of life in adults with pulmonary arterial hypertension associated with congenital heart disease with and without Down’s syndrome. Am J Cardiol. 2009; 103 (9): 1309-15.

545. Vis J.C., Duffels M.G., Mulder P., et al. Prolonged beneficial effect of bosentan treatment and 4-year survival rates in adult patients with pulmonary arterial hypertension associated with congenital heart disease. Int J Cardiol. 2013; 164 (1): 64-9.

546. Baptista R, Castro .G, da Silva A.M., et al. Long-term effect of bosentan in pulmonary hypertension associated with complex congenital heart disease. Rev Port Cardiol. 2013; 32 (2): 123-29.

547. Pulido T., Adzerikho I., Channick R.N., et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369: 809–818.

548. Gatzoulis M.A., Landzberg M., Beghetti M., et al. Evaluation of Macitentan in Patients With Eisenmenger Syndrome. Circulation. 2019; 139 (1): 51-63.

549. Galiè N., Ghofrani H.A., Torbicki A., et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med. 2005; 353 :2148–57.

550. Lu X.L., Xiong C.M., Shan G.L., et al. Impact of sildenafil therapy on pulmonary arterial hypertension in adults with congenital heart disease. Cardiovasc Ther. 2010; 28 (6): 350-55.

551. Chau E.M., Fan K.Y., Chow W.H. Effects of chronic sildenafil in patients with Eisenmenger syndrome versus idiopathic pulmonary arterial hypertension. Int J Cardiol. 2007; 120 (3): 301-5.

552. Zeng W.J., Lu X.L, Xiong C.M., et al. The efficacy and safety of sildenafil in patients with pulmonary arterial hypertension associated with the different types of congenital heart disease. Clin Cardiol. 2011; 34 (8): 513-18.

553. Iversen K., Jensen A.S., Jensen T.V., et al. Combination therapy with bosentan and sildenafil in Eisenmenger syndrome: a randomized, placebo-controlled, double-blinded trial. Eur Heart J. 31 (9):1124-31.

554. Ghofrani H. A., Galiè N., Grimminger F., et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med. 2013; 369(4): 330-40.

555. Шмальц А.А., Горбачевский С.В. Риоцигуат и силденафил в лечении легочной гипертензии: сходства и различия. Пульмонология 2016; 26 (1): 85-91.

556. Валиева З.С., Грацианская С.Е., Мартынюк Т.В. Применение риоцигуата для лечения пациентов с хронической тромбоэмболической легочной гипертензией. Кардиология. 2020; 60 (8): 115-23.

557. Stasch J.P., Pacher P., Evgenov O.V. Soluble Guanylate Cyclase as an Emerging Therapeutic Target in Cardiopulmonary Disease. Circulation 2011; 123: 2263-2274.

558. Rubin L.J., Galiè N., Grimminger F., et al. Riociguat for the treatment of pulmonary arterial hypertension: a long-term extension study (PATENT-2). Eur Respir J. 2015; 45 (5): 1303-13.

559. Rosenkranz S., Ghofrani H.A., Beghetti M., et al. Riociguat for pulmonary arterial hypertension associated with congenital heart disease. Heart. 2015;101 (22): 1792-99.

560. Cha K.S., Cho K.I., Seo J.S., et al. Effects of inhaled iloprost on exercise capacity, quality of life, and cardiac function in patients with pulmonary arterial hypertension secondary to congenital heart disease (the Eisenmenger syndrome) (from the EIGER Study). Am J Cardiol. 2013; 112 (11):1834-39.

561. Chon M.K., Cho K.I., Cha K.S., et al. Effects of long-term iloprost treatment on right ventricular function in patients with Eisenmenger syndrome. J Cardiol. 2017; 69 (5):741-46.

562. Sitbon O., Channick R., Chin K.M., et al. Selexipag for the Treatment of Pulmonary Arterial Hypertension. N Engl J Med. 2015; 373 (26): 2522-33.

563. Beghetti M., Channick R.N., Chin K.M., et al. Selexipag treatment for pulmonary arterial hypertension associated with congenital heart disease after defect correction: insights from the randomised controlled GRIPHON study. Eur J Heart Fail. 2019; 21 (3): 352-59.

564. Hascoet S, Fournier E, Jaïs X., et al. Outcome of adults with Eisenmenger syndrome treated with drugs specific to pulmonary arterial hypertension: A French multicentre study. Arch Cardiovasc Dis. 2017;110(5):303-316

565. Li Q., Kuang H.Y., Wu Y.H., et al. What is the position of pulmonary arterial hypertension-specific drug therapy in patients with Eisenmenger syndrome: A systematic review and meta-analysis. Medicine (Baltimore). 2019; 98 (20): e15632.

566. D’Alto M, Romeo E, Argiento P, Sarubbi B, Santoro G, Grimaldi N et al. Bosentan-sildenafil association in patients with congenital heart disease-related pulmonary arterial hypertension and Eisenmenger physiology. Int J Cardiol. 2012;155(3):378-82

567. Varela D.L., Teleb M., El-Mallah W. Advanced therapies for the management of adults with pulmonary arterial hypertension due to congenital heart disease: a systematic review. Open Heart. 2018; 5 (1): e000744.

568. Brida M., Gatzoulis M.A. Pulmonary arterial hypertension in adult congenital heart disease. Heart. 2018; 104 (19): 1568-74.

569. D’Alto M, Romeo E, Argiento P, Sarubbi B, Santoro G, Grimaldi N et al. Bosentan-sildenafil association in patients with congenital heart disease-related pulmonary arterial hypertension and Eisenmenger physiology. Int J Cardiol. 2012;155(3):378-82

570. Schuuring M.J., Bouma B.J., Cordina R., et al. Treatment of segmental pulmonary artery hypertension in adults with congenital heart disease. Int J Cardiol. 2013; 164 (1): 106-10.

571. Hebert A, Mikkelsen UR, Thilen U, et al., Bosentan improves exercise capacity in adolescents and adults after Fontan operation: the TEMPO (Treatment With Endothelin Receptor Antagonist in Fontan Patients, a Randomized, Placebo-Controlled, Double-Blind Study Measuring Peak Oxygen Consumption) study. Circulation. 2014; 130(23):2021-30.

572. Agnoletti G., Gala S., Ferroni F., et al. Endothelin inhibitors lower pulmonary vascular resistance and improve functional capacity in patients with Fontan circulation. J Thorac Cardiovasc Surg. 2017; 153 (6): 1468-75.

573. Schuuring M.J., Vis J.C., van Dijk A.P., et al. Impact of bosentan on exercise capacity in adults after the Fontan procedure: a randomized controlled trial. Eur J Heart Fail. 2013; 15 (6): 690-98.

574. Giardini A., Balducci A., Specchia S., et al. Effect of sildenafil on haemodynamic response to exercise and exercise capacity in Fontan patients. Eur Heart J. 2008; 29 (13): 1681-87.

575. Van De Bruaene A., La Gerche A., et al. Sildenafil improves exercise hemodynamics in Fontan patients. Circ Cardiovasc Imaging. 2014; 7 (2): 265-73.

576. Blok I.M., van Riel A.C.M.J., van Dijk A.P.J., et al. From bosentan to macitentan for pulmonary arterial hypertension and adult congenital heart disease: Further improvement? Int J Cardiol. 2017; 227: 51-52.

577. Gelatt M., Hamilton R.M., et al. Arrhythmia and mortality after the Mustard procedure: a 30-year single-center experience. J Am Coll Cardiol. 1997; 29: 194-201.

578. Dearani J.A., Danielson G.K., Puga F.J., et al. Late follow-up of 1095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits. Ann Thorac Surg. 2003;75:399-410.

579. Redington AN, Somerville J. Stenting of aortopulmonary collaterals in complex pulmonary atresia. Circulation 1996;94:2479-84.

580. Giannakoulas G., Gatzoulis M.A. Pulmonary arterial hypertension in congenital heart disease: current perspectives and future challenges. Hellenic J Cardiol 2016; 57:218-22.

581. Van De Bruaene A., Toh N., et al. Pulmonary hypertension in patients with a subaortic right ventricle: prevalence, impact and management. Heart 2019;105:1471-78.

582. De Pasquale G., Bonassin Tempesta F, Lopes BS, et al. High prevalence of baffle leaks in adults after atrial switch operations for transposition of the great arteries. Eur Heart J Cardiovasc Imaging. 2017; 18: 531-35.

583. Rydman R., Gatzoulis M.A., Ho S.Y., et al. Systemic right ventricular fibrosis detected by cardiovascular magnetic resonance is associated with clinical outcome, mainly new-onset atrial arrhythmia, in patients after atrial redirection surgery for transposition of the great arteries. Circ Cardiovasc Imaging. 2015; 8: e002628.

584. Zaragoza-Macias E., Zaidi A.N., Dendukuri N., Marelli A. Medical therapy for systemic right ventricles: a systematic review (part 1) for the 2018 AHA/ACC Guideline for the management of adults with congenital heart disease: a report of the American College of Cardiology/ American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2019; 73: 1564-78.

585. Voet A., Rega F., de Bruaene A.V., et al. Long-term outcome after treatment of isolated pulmonary valve stenosis. Int J Cardiol. 2012; 156: 11-15.

586. Raissadati A., Nieminen H., Sairanen H., Jokinen E. Outcomes after the Mustard, Senning and arterial switch operation for treatment of transposition of the great arteries in Finland: a nationwide 4-decade perspective. Eur J Cardiothorac Surg. 2017; 52: 573-80.

587. Hernandez-Madrid A., Paul T., Abrams D., et al. ESC Scientific Document Group. Arrhythmias in congenital heart disease: a position paper of the European Heart Rhythm Association (EHRA), Association for European Paediatric and Congenital Cardiology (AEPC), and the European Society of Cardiology (ESC) Working Group on Grown-up Congenital Heart Disease, endorsed by HRS, PACES, APHRS, and SOLAECE. Europace. 2018; 20:1719-53.

588. Redington A.N., Somerville J. Stenting of aortopulmonary collaterals in complex pulmonary atresia. Circulation. 1996; 94: 2479-84.

589. Hsu C.H., Gomberg-Maitland M., Glassner C. et al. The management of pregnancy and pregnancy-related medical conditions in pulmonary arterial hypertension patients. Int J Clin Pract Suppl. 2011; 172: 6-14.

590. Neutze J.M., Ishikawa T., Clarkson P.M., et al. Assessment and follow-up of patients with ventricular septal defect and elevated pulmonary vascular resistance. Am J Cardiol 1989; 63: 327-31.

591. Daliento L., Somerville J., Presbitero P., et al. Eisenmenger syndrome. Factors relating to deterioration and death, Eur. Heart J. 1998; 19: 1845–55.

592. Oya H., Nagaya N., Satoh T., et al. Haemodynamic correlates and prognostic significance of serum uric acid in adult patients with Eisenmenger syndrome. Heart. 2000; 84: 53–58.