References

evrazkar

Евразийский Кардиологический Журнал

Eurasian heart journal

2225-16852305-0748

Евразийская ассоциация кардиологов

10.38109/2225-1685-2022-2-6-70

evrazkar-6322

Research Article

КЛИНИЧЕСКИЕ РЕКОМЕНДАЦИИ

CLINICAL GUIDELINES

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

Eurasian guidelines for the diagnosis and treatment of pulmonary hypertension associated with congenital heart defects in adults (2021)

https://orcid.org/0000-0002-1576-4877

Чазова

И. Е.

Chazova

I. E.

Чазова Ирина Евгеньевна (председатель), д.м.н., профессор, академик РАН, заместитель генерального директора по научно-экспертной работе, руководитель отдела гипертонии, НИИ клинической кардиологии им. А.Л. Мясникова

Москва

Irina E. Chazova (chairman), Dr. of Sci. (Med.), Academician of RAS, Deputy General Director for Scientific and Expert Work, Head of Hypertension Department, A.L. Myasnikov Research Institute of Cardiology

Moscow

https://orcid.org/0000-0002-4193-3320

Горбачевский

С. В.

Gorbachevskij

S. V.

Горбачевский Сергей Валерьевич (председатель), д.м.н., профессор, руководитель отделения заболеваний сердца с прогрессирующей легочной гипертензией; профессор кафедры сердечно-сосудистой хирургии

Москва

Sergej V. Gorbachevsky (chairman), Dr. of Sci. (Med.), Professor, Head of the Department of Surgical Treatment of Cardiac Diseases with Progressive Pulmonary Hypertension

Moscow

https://orcid.org/0000-0002-9022-8097

Мартынюк

Т. В.

Martynyuk

T. V.

Мартынюк Тамила Витальевна (зам. председателя), руководитель отдела легочной гипертензии и заболеваний сердца НИИ клинической кардиологии им. А.Л. Мясникова; проф. кафедры кардиологии; д.м.н., председатель секции легочной гипертензии Российского медицинского общества по артериальной гипертонии

121552, Москва, ул. 3-я Черепковская, д. 15а, телефон/факс: +7-495-414-6450

Tamila V. Martynyuk (vice chairman), Dr. of Sci. (Med.), Head of the Department of Pulmonary Hypertension and Cardiac Diseases, A.L. Myasnikov Research Institute of Cardiology

3rd Cherepkovskaya street, 15a, Moscow 121552, Phone/Fax+7-495-414-6450

trukhiniv@mail.ru

https://orcid.org/0000-0001-8937-1796

Шмальц

А. А.

Shmal’c

A. A.

Шмальц Антон Алексеевич (зам. председателя), д.м.н., ведущий научный сотрудник отделения хирургического лечения заболеваний сердца с прогрессирующей легочной гипертензией

Москва

Anton A. Shmalts (vice chairman), Dr. of Sci. (Med.), Leading Research Associate of the Department of Surgical Treatment of Cardiac Diseases with Progressive Pulmonary Hypertension

Moscow

https://orcid.org/0000-0002-9041-3604

Валиева

З. С.

Valieva

Z. S.

Валиева Зарина Солтановна (секретарь), к.м.н., старший научный сотрудник отдела легочной гипертензии и заболеваний сердца, НИИ клинической кардиологии им. А.Л. Мясникова

Москва

Zarina S. Valieva (secretary), Cand. of Sci. (Med.), Senior Researcher of the department of pulmonary hypertension and heart diseases, A.L. Myasnikov Research Institute of Cardiology

Moscow

https://orcid.org/0000-0001-8071-2531

Барышникова

И. Ю.

Baryshnikova

I. Yu.

Барышникова Ирина Юрьевна, к.м.н., научный сотрудник отделения ультразвуковой диагностики

Москва

Irina Yu. Baryshnikova, MD, PhD, Researcher departments of ultrasound diagnostics

Moscow

https://orcid.org/0000-0002-6532-7261

Глушко

Л. А.

Glushko

L. A.

Глушко Людмила Александровна, к.м.н., заведующая группой легочных проб и мониторинга газообмена – врач функциональной диагностики

Москва

Lyudmila A. Glushko, Cand. of Sci. (Med.), Chief of Pulmonary Tests and Gas Exchange Monitoring Group – Functional diagnostician

Moscow

https://orcid.org/0000-0003-3292-0912

Грамович

В. В.

Gramovich

V. V.

Грамович Владимир Владимирович, к.м.н., старший научный сотрудник отдела легочной гипертензии и заболеваний сердца, НИИ клинической кардиологии им. А.Л. Мясникова

Москва

Vladimir V. Gramovich, Cand. of Sci. (Med.), Senior Researcher оf the department of pulmonary hypertension and heart diseases, A.L. Myasnikov Research Institute of Cardiology

Moscow

https://orcid.org/0000-0001-5655-3913

Азизов

В. А.

Azizov

V. A.

Азизов Васадат Алиевич, д.м.н., профессор, заведующий кафедрой внутренних болезней

Баку

Vasadat A. Azizov, Dr. of Sci. (Med.), Professor, Head of the Department of Internal Diseases

Baku

https://orcid.org/0000-0001-8319-3714

Веселова

Т. Н.

Veselova

T. N.

Веселова Татьяна Николаевна, д.м.н., старший научный сотрудник отдела томографии, НИИ клинической кардиологии им. А.Л. Мясникова

Москва

Tatiana N. Veselova, Dr. of Sci. (Med.), Leading Researcher of the tomography department, A.L. Myasnikov Research Institute of Cardiology

Moscow

https://orcid.org/0000-0001-9853-9087

Данилов

Н. М.

Danilov

N. M.

Данилов Николай Михайлович, д.м.н., ведущий научный сотрудник отдела гипертонии НИИ клинической кардиологии им. А.Л. Мясникова

Москва

Nikolaj M. Danilov, Dr. of Sci. (Med.), Leading Researcher Of Hypertension Department, A.L. Myasnikov Research Institute of Cardiology

Moscow

https://orcid.org/0000-0002-6513-6772

Зелвеян

П. А.

Zelveyan

P. A.

Зелвеян Парунак Арутюнович, д.м.н., профессор, директор

Ереван

Parunak A. Zelveyan, Dr. of Sci. (Med.), Professor, Director

Yerevan

https://orcid.org/0000-0001-6439-1774

Иванов

С. Н.

Ivanov

S. N.

Иванов Сергей Николаевич, д.м.н., профессор, ведущий научный сотрудник центра новых хирургических технологий

Новосибирск

Sergei. N. Ivanov, Dr. of Sci. (Med.), Professor, Leading Researcher of THE center of new surgical technologies

Novosibirsk

https://orcid.org/0000-0003-3818-7491

Лазарева

И. В.

Lazareva

I. V.

Лазарева Ирина Валентиновна, к.м.н., заведующая отделением

Минск

Irina V. Lazareva, Cand. of Sci. (Med.)

Minsk

https://orcid.org/0000-0002-0200-852X

Матчин

Ю. Г.

Matchin

Yu. G.

Матчин Юрий Георгиевич, д.м.н., профессор, руководитель лаборатории рентгенэндоваскулярных методов диагностики и лечения в амбулаторных условиях НИИ кардиологии им. А.Л. Мясникова

Москва

Dr. of Sci. (Med.), Professor, Head of the laboratory of x-ray endovascular methods of diagnostics and treatment in outpatient settings, 2nd department of x-ray surgical methods of diagnostics and treatment, A.L. Myasnikov Research Institute of Cardiology

Moscow

https://orcid.org/0000-0003-2854-1952

Мукаров

М. А.

Mukarov

M. A.

Мукаров Мурат Аманжолович, к.м.н., заведующий отделением

Нур-Султан

Murat A. Mukarov, Cand. of Sci. (Med.)

Nur-Sultan

https://orcid.org/0000-0003-1564-7619

Наконечников

С. Н.

Nakonechnikov

S. N.

Наконечников Сергей Николаевич, д.м.н., профессор, директор Евразийской ассоциации кардиологов, проф. кафедры кардиологии

Москва

Sergej N. Nakonechnikov, Dr. of Sci. (Med.), Professor, Director of the, Eurasian Association of Cardiologists, Professor of the Department of Cardiology

Moscow

https://orcid.org/0000-0003-2172-9776

Сарыбаев

А. Ш.

Sarybaev

A. Sh.

Сарыбаев Акпай Шогаибович, д.м.н., профессор, директор

Бишкек

Akpaj Sh. Sarybaev, Dr. of Sci. (Med.), Professor, Director

Bishkek

https://orcid.org/0000-0001-8377-2388

Стукалова

О. В.

Stukalova

O. V.

Стукалова Ольга Владимировна, к.м.н., старший научный сотрудник отдела томографии НИИ кардиологии им. А.Л. Мясникова

Москва

Ol’ga V. Stukalova, Cand. of Sci. (Med.), Senior Researcher of the tomography department, A.L. Myasnikov Research Institute of Cardiology

Moscow

https://orcid.org/0000-0003-2724-4016

Шалаев

С. В.

Shalaev

S. V.

Шалаев Сергей Васильевич, д.м.н., профессор, заведующий кафедрой, заслуженный деятель науки РФ, главный специалист-кардиолог департамента здравоохранения Тюменской области, главный кардиолог Уральского ФО

Тюмень

Sergej V. Shalaev, Dr. of Sci. (Med.), Professor, Head of the Department, Honored Scientist of the Russian Federation, Professor, Chief Cardiologist of the Health Department of the Tyumen Region, Chief Cardiologist of the Ural Federal District

Tyumen

ФГБУ «НМИЦ кардиологии им. акад. Е.И. Чазова» Минздрава РоссииРоссияE.I. Chazov National Medical Research Center Of CardiologyRussian Federation

ФГБУ «НМИЦ ССХ им. А.Н. Бакулева» Минздрава России; ФГБОУ ДПО РМАНПО МЗ РФРоссияA.N. Bakulev National Medical Research Center of Cardiovascular SurgeryRussian Federation

ФГБУ «НМИЦ кардиологии им. акад. Е.И. Чазова» Минздрава России; ФДПО РНИМУ им. Н.И. ПироговаРоссияE.I. Chazov National Medical Research Center Of CardiologyRussian Federation

ФГБУ «НМИЦ ССХ им. А.Н. Бакулева» Минздрава РоссииРоссияA.N. Bakulev National Medical Research Center of Cardiovascular SurgeryRussian Federation

1-й Азербайджанский Медицинский УниверситетАзербайджан1st Azerbaijan Medical UniversityAzerbaijan

Центр превентивной кардиологииАрменияCenter for Preventive CardiologyArmenia

ФГБУ «НМИЦ им. ак. Е.Н. Мешалкина» Минздрава РоссииРоссияE.N. Meshalkin National Medical Research CenterRussian Federation

Республиканский научно-практический центр КардиологияБеларусьScientific and Рractical Сentre of СardiologyBelarus

Национальный научный кардиохирургический центрКазахстанNational Research Cardiac Surgery CenterKazakhstan

ФДПО РНИМУ им. Н.И. ПироговаРоссияPirogov Russian National Research Medical UniversityRussian Federation

Национальный центр кардиологии и терапии им. академика М. Миррахимова при Минздраве Киргизской РеспубликиКыргызстанNational Center for Cardiology and Therapy M.M. MirrakhimovaKyrgyzstan

Департамент здравоохранения Тюменской областиРоссияHealth Department of the Tyumen RegionRussian Federation

2022

28052022

02670

Copyright © Чазова И.Е., Горбачевский С.В., Мартынюк Т.В., Шмальц А.А., Валиева З.С., Барышникова И.Ю., Глушко Л.А., Грамович В.В., Азизов В.А., Веселова Т.Н., Данилов Н.М., Зелвеян П.А., Иванов С.Н., Лазарева И.В., Матчин Ю.Г., Мукаров М.А., Наконечников С.Н., Сарыбаев А.Ш., Стукалова О.В., Шалаев С.В., 2022

2022

Чазова И.Е., Горбачевский С.В., Мартынюк Т.В., Шмальц А.А., Валиева З.С., Барышникова И.Ю., Глушко Л.А., Грамович В.В., Азизов В.А., Веселова Т.Н., Данилов Н.М., Зелвеян П.А., Иванов С.Н., Лазарева И.В., Матчин Ю.Г., Мукаров М.А., Наконечников С.Н., Сарыбаев А.Ш., Стукалова О.В., Шалаев С.В.

Chazova I.E., Gorbachevskij S.V., Martynyuk T.V., Shmal’c A.A., Valieva Z.S., Baryshnikova I.Y., Glushko L.A., Gramovich V.V., Azizov V.A., Veselova T.N., Danilov N.M., Zelveyan P.A., Ivanov S.N., Lazareva I.V., Matchin Y.G., Mukarov M.A., Nakonechnikov S.N., Sarybaev A.S., Stukalova O.V., Shalaev S.V.

Данная работа распространяется под лицензией Creative Commons Attribution 4.0.

This work is licensed under a Creative Commons Attribution 4.0 License.

https://www.heartj.asia/jour/article/view/6322

легочная гипертензиялегочная артериальная гипертензияврожденный порок сердцалегочно-сосудистая болезньхирургическое лечениеатриосептостомияспецифическая терапияклинические рекомендации

pulmonary hypertensionpulmonary arterial hypertensioncongenital heart diseasepulmonary vascular diseasesurgeryatrioseptostomyspecific therapyguidelines

References1

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

Chazova I.E., Martynyuk T.V., Valieva Z.S., et al). EURASIAN CLINICAL GUIDELINES ON DIAGNOSIS AND TREATMENT OF PULMONARY HYPERTENSION. Eurasian heart journal. 2020;(1):78-122. (In Russ.) https://doi.org/10.38109/2225-1685-2020-1-78-122

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

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

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

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

Martynyuk T.V. Pulmonary hypertension: diagnosis and treatment. Moscow: LLC «Medical Information Agency», 2018: 304 р. (In Russ.)

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

Chazova I.E., Arkhipova O.A., Martynyuk T.V. Pulmonary arterial hypertension in Russia: six-year observation analysis of the National Registry // Terapevticheskii arkhiv. 2019; Vol. 91 (1):25-31. (In Russ.) https://doi.org/10.26442/00403660.2019.01.000024

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

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

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

Pulmonary hypertension, including chronic thromboembolic pulmonary hypertension. Russian clinical guidelines, 2020 (In Russ.) http://cr.rosminzdrav.ru/#!/recomend/137

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

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

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

Gorbachevsky S.V., Shmalts A.A. Diagnosis of pulmonary hypertension associated with congenital heart disease. Part 1. Definition, classification and initial examination of patients. Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics). 2021;66(5):28-37. (In Russ.) https://doi.org/10.21508/1027-4065-2021-66-5-28-37

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

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

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

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

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

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.

Bockeria L.A., Gorbacheusky S.V., Shmalts A.A., et al. Hemodinamic correction of the single functional ventricle after prior pulmonary arterial narrowing. Russian Journal of Thoracic and Cardiovascular Surgery. 2008; 5:27-34 (In Russ.)

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

Bockeria L.A., Gorbachevskiy S.V., Shmal’s A.A. Pulmonary arterial hypertension associated with congenital heart disease in adults (clinical guidelines). Grudnaya i Serdechno-Sosudistaya Khirurgiya, Thoracic and Cardiovascular Diseases, Russian journal). Grudnaya i serdechno-sosudistaya khirurgiya (Russian Journal of Thoracic and Cardiovascular Surgery).2017; 59(2):135–147. (In Russ). https://doi.org/10.24022/0236-2791-2017-59-2-135-147

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

Pulmonary hypertension in children. Russian clinical guidelines, 2017 (In Russ). http://cr.rosminzdrav.ru/#!/recomend/901

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

Hypertensive pulmonary vascular disease associated with congenital heart disease in children. Russian clinical guidelines, 2018 (In Russ). http://cr.rosminzdrav.ru/#!/schema/356

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

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

Chazova I.E., Martynyuk T.V. Pulmonary hypertension. Moscow. Practice. 2015, 928 p. (In Russ.)

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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.

Bockeria L.A., Gorbachevskiy S.V., Shmal’s A.A. Pulmonary arterial hypertension associated with congenital heart disease in adults. Methodological manual. Bakoulev SCCS RAMS. 2016. 28 p. ISBN 978-5-7982-0368-0 (In Russ.)

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

Khugaev GA, Shmalts AA. Morphological assessment of pulmonary vessels in pulmonary arterial hypertension associated with congenital heart disease. Arkhiv Patologii. 2021;83(5):49-57. (In Russ.) https://doi.org/10.17116/patol20218305149

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

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

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

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

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

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

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

Sharykin A.S. Congenital heart disease. Guidelines for pediatricians, cardiologists, neonatologists. Moscow: Teremok. 2005:381. (In Russ.)

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

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

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

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

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

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

Esipova I.K. Some questions in pulmonary pathology. The Academy of Sciences of the Soviet Union. 1962. (In Russ.)

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.

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

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

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

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

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

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

Gorbachevsky S.V., Shmalts A.A., Plotnikova L.R. Pulmonary hypertension in children with congenital heart disease. Moscow; OOO «Kedr», 2018; 91. (in Russ.)

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

Pulmonary hypertension in children. Editors L.A. Bockeria, S.V. Gorbachevsky, M.A. Shkolnikova. Moscow: Aktelion farmas’yutikalz, 2013; 416. (in Russ.)

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.

Gorbachevsky S.V., Shmalts A.A. Hypertensive pulmonary vascular disease associated with congenital heart defects. In: L.A. Bockeria, K.V. Shatalov (eds.). Pediatric cardiac surgery. A guide for doctors. Moscow, 2016; 833–850. (in Russ.)

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Chazova I.E., Avdeev S.N., Tsareva N.A., et al. Clinical guidelines for the diagnosis and treatment of pulmonary hypertension. Terapevticheskii arkhiv. 2014; 9:4-23. (In Russ.)

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.

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

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

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

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

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

Ryabykina G.V., Blinova E.V., Sakhnova T.A. Electrovectorcardiographic diagnosis of right ventricular hypertrophy in patients with pulmonary hypertension. Practitioners’ manual. Medical alphabet. 2017;1(8):52-66. (In Russ.)

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

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

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

Yurpol’skaya L.A., Shmalts A.A. Х-ray methods and magnetic resonance imaging in pulmonary hypertension associated with congenital heart disease. Children’s Heart and Vascular Diseases. 2021;18 (2):83–93 (in Russ.) https://doi.org/10.24022/1810-0686-2021-18-2-83-93

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

Tsallagova Z.S., Lazutkina V.K. Possibilities of radiography in assessment of pulmonary arterial hypertension. Meditsinskaya vizualizatsiya. 2001; (4):58–63. (In Russ.)

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

X-ray diagnostics of diseases of the heart and blood vessels. /Ed. M.A. Ivanitskaya. -M., “Medicine”, 1970. (In Russ).

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.

X-ray diagnostics in pediatrics. Guide for doctors in 2 volumes. / Ed. V.F.Baklanova, M.A.Filippkina.-M.: Medicine, -1988.-V.1.-448 P. (In Russ).

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

Pediatric cardiology. Ed. J. Hoffman. Translation from English -M .: Practice. -2006.-543P. (In Russ.)

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

Korobkova I.K., Lazutkina V.K., Nizovtsova L.A., Riden T.V. Radiographic assessment of pulmonary hypertension: Methodical aspects. Journal of radiology and nuclear medicine. 2015;(4):46-53. (In Russ.) https://doi.org/10.20862/0042-4676-2015-0-4-14-21

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

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

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

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

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

Baryshnikova I.Yu., Shmalts A.A. Diagnosis of pulmonary hypertension associated with congenital heart disease. Part 2. Echocardiography. Russian Bulletin of Perinatology and Pediatrics. 2021; 66(6):23–32 (In Russ). 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.

Bokeriya L.A., Baryshnikova I.Yu., Gorbachevsky S.V. Echocardiography in the diagnosis and assessment of pulmonary hypertension. Tutorial. Moscow, 2017; 54 р. (In Russ.)

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

Shariya A.M., Martynyuk T.V., Ternovoy S.K., Shariya M.A. Possibilities of Magnetic Resonance Tomography in Diagnosis of Pulmonary Arterial Hypertension. Kardiologiia. 2021;61(6):97-104. (In Russ.) 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.

L.A. Bockeria, V.N. Makarenko, L.A. Yurpol’skaya. Computer tomography and magnetic resonance imaging in the diagnostic algorithm of congenital heart disease: what, when, who? – “for” and “against”. Russian Journal of Thoracic and Cardiovascular Surgery. 2014; 3: 4-13. (In Russ.)

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.

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

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.

X-ray diagnostics of diseases of the heart and blood vessels. Ed. M.A. Ivanitskaya. M., “Medicine”, 1970 (In Russ).

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.

Makarenko V.N., Yurpolskaya L.A., Zelenikin M.M., Shinkareva T.V. Computed tomography in the evaluation of patients after Fontan surgery. Bulletin of the NCSSH them. A.N. Bakulev RAMS. 2010; 11(1):46-50. (In Russ).

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

Sobolev A.V., Shmalts A.A. Endovascular diagnostics of pulmonary hypertension associated with congenital heart defects. Part 1. Cardiac catheterization and angiography. Russian journal of endovascular surgery. 2021; 8(3):263–71. (In Russ.) 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.

Gorbachevsky S.V., Belkina M.V., Koledinsky D.G., et al. Invasive pulmonary artery pressure monitoring is an objective method for assessing the severity of pulmonary hypertension in children with congenital heart disease. Children’s diseases of the heart and blood vessels. 2006; 4:77–81. (In Russ).

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.

Glushko L.A., Shmalts A.A. Assessment of the state of the cardiorespiratory system in pulmonary hypertension associated with congenital heart disease. Creative cardiology. 2021; 15(2): 167-179. (In Russ).

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

Taran I.N., Valieva Z.S., Martynyuk T.V., et al. Spiroveloergometry in risk stratification of patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. Eurasian Cardiology Journal. 2017; 4:86-92. (In Russ). 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.

Chazova I.E., Martynyuk T.V. et al. Diagnosis and treatment of pulmonary hypertension. Russian recommendations. Cardiovascular Therapy and Prevention 2007; 6: Appendix 2. (In Russ).

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.

Arkhipova O.A., Martynyuk T.V., Samoilenko L.E., et al. Perfusion lung scintigraphy in patients with pulmonary hypertension of various etiologies. Eurasian Cardiology Journal. 2015; 4:21-25. (In Russ).

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.

Gorbachevsky S.V., Shmalts A.A. Palliative atrial and arterial switching in transposition of the main arteries with ventricular septal defect and severe pulmonary vascular disease. Children’s diseases of the heart and blood vessels. 2019; 16(2): 86-94. (In Russ).

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.

Petrosyan K.V., Gorbachevsky S.V., Dadabaev G.M., Pardaev D.B. Stenting of the trunk of the left coronary artery in a patient with pulmonary hypertension. Eurasian Cardiology Journal. 2017; 3:86-87.(In Russ).

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.

Gorbachevsky S.V., Pursanov M.G., Shmalts A.A., et al. Results of atrioseptostomy with stenting in patients with idiopathic and similar forms of pulmonary arterial hypertension. Thoracic and cardiovascular surgery. 2019; 61(2): 100-113..(In Russ).

202

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

Shmalts A.A., Nishonov N.A. Atrioseptostomy in patients with pulmonary hypertension. Thoracic and cardiovascular surgery. 2015; 57(5): 18-25.(In Russ).

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

Jaı ̈s 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

Arkhipova O.A., Martynyuk T.V., Chazova I.Y. Tactics features of conducting and choosing specific therapy for adult patients with pulmonary arterial hypertension associated with congenital heart disease. Eurasian heart journal. 2018;(1):48-59. (In Russ.) 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

Shmalts A.A., Gorbachevskiy S.V.. Evidence base for specific pulmonary vasodilators in children with congenital heart defects. Pediatrics named after G.N. Speransky. 2017; 96 (6): 126-139. (In Russ.) 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

Shmalts AA, Gorbachevsky SV. Evidence base for specific pulmonary vasodilators in adults with congenital heart disease. Terapevticheskii Arkhiv (Ter. Arkh.). 2021; 93(9):1106–1116. (In Russ.) 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

Shmal’ts A.A., Belkina M.V., Gorbachevskiy S.V. Specific pulmonary vasodilators after Fontan operation. Detskie bolezni serdtsa i sosudov (Children’s Heart and Vascular Diseases). 2017; 14 (1): 16–24 (in Russ.) 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=

Tracleer®. Instructions for use of a medicinal product for medical use. LS-002509 (In Russ.) 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=

Bosenex. Instructions for medical use of the use of the medical product. LP-004208 (In Russ.) 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=

Opsumit®. Instructions for use of a medicinal product for medical use. LP-003310. (In Russ.) 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=

Upbravi. Instructions for use of a medicinal product for medical use. LP-005577. (In Russ.) 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

Shmalts A.A., Gorbachevsky S.V. Advantages and limitations of initial combination therapy in pulmonary arterial hypertension patients in Russia. Therapeutic Archive. 2020; 92 (12). (In Russ.) 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.

Chazova I.E., Martynyuk T.V., Nakonechnikov S.N. Endothelin receptor antagonists in pulmonary arterial hypertension: yesterday, today and tomorrow. Russian Journal of Cardiology 2009; 4:73–81. (In Russ.)

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

Shmal’ts A.A., Gorbachevskiy S.V. Riociguat and sildenafil for pulmonary hypertension: similarity and difference. PULMONOLOGIYA. 2016;26(1):85-91. (In Russ.) 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

Valieva Z.S., Gratsianskaya S.E., Martynyuk T.V. The soluble guanylate cyclase stimulator riociguat is the first-line therapy for chronic thromboembolic pulmonary hypertension patients. Kardiologiia. 2020;60(8):115-123. (In Russ.) 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

Eurasian clinical guidelines on diagnosis and treatment of pulmonary hypertension. Eurasian heart journal. 2020;(1):78-122. (In Russ.) https://doi.org/10.38109/2225-1685-2020-1-78-122

296

297

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

298

299

Martynyuk T.V. Pulmonary hypertension: diagnosis and treatment. Ser. Library of the National Medical Research Center for Cardiology of the Ministry of Health of Russia. M.: OOO Medical Information Agency, 2018. 304 p. (in Russ.)

300

Chazova I.E., Arkhipova O.A., Martynyuk T.V. Pulmonary arterial hypertension in Russia: analysis of a six-year follow-up according to the National Register. Therapeutic archive. 2019; 91(1): 24-32 (in Russ.)

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

Pulmonary hypertension, including chronic thromboembolic pulmonary hypertension. Russian clinical guidelines, 2020 (in Russ.)

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

306

Gorbachevsky S.V., Shmalts A.A. Diagnosis of pulmonary hypertension associated with congenital heart disease. Part 1. Definition, classification and initial examination of patients. Russian Bulletin of Perinatology and Pediatrics. 2021; 66(5):28-37 (in Russ.)

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

311

312

Bockeria L.A., Gorbachevsky S.V., Shmalts A.A., et al. Hemodynamic correction of the functionally single ventricle of the heart after previously performed narrowing of the pulmonary artery. Thoracic and cardiovascular surgery. 2008; 5:27-34. (in Russ.)

313

Bockeria L.A., Gorbachevsky S.V., Shmalts A.A. Pulmonary arterial hypertension associated with congenital heart disease in adults (clinical recommendation). Thoracic and cardiovascular surgery. 2017; 59(2): 135-47 (in Russ.)

314

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

Pulmonary hypertension in children. Russian clinical guidelines, 2017 (in Russ.) http://cr.rosminzdrav.ru/#!/recomend/901.

315

Hypertensive pulmonary vascular disease associated with congenital heart disease in children. Russian clinical guidelines, 2018 (in Russ.) http://cr.rosminzdrav.ru/#!/schema/356.

316

317

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

Chazova I.E., Martynyuk T.V. Pulmonary hypertension. Moscow. Practice. 2015, 928 s (in Russ.)

318

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

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

328

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

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

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

Bockeria L.A., Gorbachevsky S.V., Shmalts A.A. Pulmonary arterial hypertension associated with congenital heart disease in adults. Toolkit. NTSSSH them. A. N. Bakuleva RAMS, 2016 (in Russ.)

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.

Khugaev G.A., Shmalts A.A. Morphological assessment of pulmonary vessels in pulmonary hypertension caused by congenital heart defects. Archive of pathology. 2021; 83(5):49–57 (in Russ.)

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

Sharykin A.S. Congenital heart defects. Guidelines for pediatricians, cardiologists, neonatologists.-M.: Teremok.-2005.-381 p (in Russ.)

347

348

349

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

352

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

Esipova I.K. Some questions of lung pathology. AN USSR. 1962. (in Russ.)

353

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

Gorbachevsky S.V., Shmalts A.A., Plotnikova L.R. Pulmonary hypertension in children with congenital heart disease. Moscow. 2018 (in Russ.)

360

Bockeria L.A., Gorbachevsky S.V., Shkolnikova M.A. Pulmonary hypertension in children. Moscow. Actelion Pharmaceuticals; 2013 (in Russ.)

361

Gorbachevsky S.V., Shmalts A.A. Hypertensive pulmonary vascular disease associated with congenital heart disease. In: Bokeria L.A., Shatalov K.V. Children’s cardiac surgery. Guide for doctors. Moscow: NTSSSh im. A.N. Bakulev; 2016: 833– 50. (in Russ.)

362

363

364

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

365

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

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

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

Chazova I. E., Avdeev S. N., Tsareva N. A., et al. Clinical guidelines for the diagnosis and treatment of pulmonary hypertension. Therapeutic archive. 2014; 9:4-23 (in Russ.)

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

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

Ryabykina G.V., Blinova E.V., Sakhnova T.A. Electrovectorcardiographic diagnosis of right ventricular hypertrophy in patients with pulmonary hypertension. Medical alphabet 2017; 8:3-17 (in Russ.)

381

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

Yurpolskaya L.A., Shmalts A.A. Radiography, X-ray computed and magnetic resonance imaging in pulmonary hypertension associated with congenital heart disease. Children’s diseases of the heart and blood vessels. 2021; 18(2):83-93 (in Russ.)

384

Tsallagova Z.S., Lazutkina V.K. Possibilities of traditional radiography in the assessment of pulmonary arterial hypertension. medical imaging. 2001; 4:59-63. (in Russ.)

385

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

X-ray diagnostics of diseases of the heart and blood vessels. /Ed. M.A. Ivanitskaya.-M., “Medicine”, 1970. (in Russ.)

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.

X-ray diagnostics in pediatrics. Guide for doctors in 2 volumes. /Under the editorship of V.F.Baklanova, A.Filippkin. M.: Medicine, -1988.-T.1.-448 S. (in Russ.)

387

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

Children’s cardiology. / Ed. J. Hoffman. Translation from English -M .: Practice. -2006.-543С (in Russ.)

388

Korobkova I.Z., Lazutkina V.K., Nizovtsova L.A. Methodological aspects of radiographic assessment of pulmonary hypertension. Bulletin of radiology and radiology. 2015.4:45-53 (in Russ.)

389

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

393

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

394

395

396

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

397

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

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

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

408

Bockeria LA, Baryshnikova I.Yu., Gorbachevsky S.V. Echocardiography in the diagnosis and evaluation of pulmonary hypertension. Tutorial. Moscow. 2017 (in Russ.)

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

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

414

Sharia A.M., Martynyuk T.V., Ternovoy S.K., Sharia M.A. Possibilities of magnetic resonance imaging in the diagnosis of pulmonary arterial hypertension. Cardiology. 2021;61(6):97-104. (in Russ.)

415

Bockeria L.A., Makarenko V.N., Yurpolskaya L.A. Computed and magnetic resonance imaging in the diagnostic algorithm for congenital heart defects: What? When? To whom? -“for” and “against”. Thoracic and cardiovascular surgery 2014; 3:4-13 (in Russ.)

416

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

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

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

427

428

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

X-ray diagnostics of diseases of the heart and blood vessels. Ed. M.A. Ivanitskaya. M., “Medicine”, 1970. (in Russ.)

429

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

432

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

435

Sobolev A.V., Shmalts A.A. Endovascular diagnosis of pulmonary hypertension associated with congenital heart disease. Part 1. Cardiac catheterization and angiocardiography. Endovascular surgery. 2021; 8(3):263–71 (in Russ.)

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

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

Bockeria L.A., Alekyan B.G. (ed.). Guide to X-ray endovascular surgery of the heart and vessels. Moscow, NTSSSh im. A.N. Bakulev RAMS; 2008 (in Russ.)

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

448

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

449

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

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

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

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

465

466

Chazova I.E., Martynyuk T.V. et al. Diagnosis and treatment of pulmonary hypertension. Russian recommendations. Cardiovascular Therapy and Prevention 2007; 6: Appendix 2. (in Russ.)

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

469

470

471

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

475

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

476

477

478

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

479

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

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

489

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

490

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

493

494

495

496

Shmalts A.A., Nishonov N.A. Atrioseptostomy in patients with pulmonary hypertension. Thoracic and cardiovascular surgery. 2015; 57(5): 18-25 (in Russ.)

497

498

499

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

502

503

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

504

505

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.

Jaı ̈s 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

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

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

519

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

Arkhipova O.A., Martynyuk T.V., Chazova I.E. Features of tactics of management and choice of specific therapy in adult patients with pulmonary arterial hypertension associated with congenital heart disease. Eurasian Cardiology Journal. 2018.1:48-53 (in Russ.)

522

Shmalts A.A., Gorbachevsky S.V. Evidence base for specific pulmonary vasodilators in children with congenital heart disease. Pediatrics. Journal them. G.N. Speransky. 2017: 96(6):126-139 (in Russ.)

523

Shmalts A.A., Gorbachevsky S.V. Evidence base for specific pulmonary vasodilators in adults with congenital heart disease. Therapeutic archive. 2021; 93(9): 1106-1116 (in Russ.)

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.

Shmalts A.A., Belkina M.V., Gorbachevsky S.V. Specific pulmonary vasodilators after Fontan operation. Children’s diseases of the heart and blood vessels. 2017; 14(1): 16-24 (in Russ.)

525

Traclear®. Instructions for use of a medicinal product for medical use. LS-002509. (in Russ.) https://grls.rosminzdrav.ru/Grls_View_v2.aspx?routingGuid=93619b5d-c8c6-4bdb-b201-88aff22c785c&t=

526

Bosenex. Instructions for medical use of the use of the medicinal product. LP-004208. (in Russ.) https://grls.rosminzdrav.ru/Grls_View_v2.aspx?routingGuid=68d79ec9-1045-403c-be20-e88cc33f0aa6&t=

527

Opsumite®. Instructions for use of a medicinal product for medical use. LP-003310. (in Russ.) 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=

Upbravi. Instructions for use of a medicinal product for medical use.LP-005577. (in Russ.) https://grls.rosminzdrav.ru/Grls_View_v2.aspx?routingGuid=094dda8d-ba79-4353-83f9-98ea68b865aa&t=

529

530

531

532

Shmalts A.A., Gorbachevsky S.V. Opportunities and limitations for initial combined specific therapy of pulmonary arterial hypertension in the Russian Federation. Therapeutic archive. 2020; 92(12): 80-85. (in Russ.)

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

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

538

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

542

543

544

545

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

553

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.

Shmalts A.A., Gorbachevsky S.V. Riociguat and sildenafil in the treatment of pulmonary hypertension: similarities and differences. Pulmonology 2016; 26(1): 85-91. (in Russ.)

556

Valieva Z.S., Gratsianskaya S.E., Martynyuk T.V. The use of riociguat for the treatment of patients with chronic thromboembolic pulmonary hypertension. Cardiology. 2020; 60(8):115-23. (in Russ.)

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

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

564

565

566

567

568

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

569

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

572

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

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

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

583

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

587

588

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

589

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.

The authors declare that there are no conflicts of interest present.