Preview

Eurasian heart journal

Advanced search

Vasoreactivity testing in pulmonary hypertension associated with congenital heart disease

https://doi.org/10.38109/2225-1685-2022-3-58-64

Abstract

   In December, 2021 at the IXth All-Russian Congress «Pulmonary Hypertension 2021» the first Eurasian guidelines for the diagnosis and treatment of pulmonary hypertension associated with congenital heart disease in adults were approved. The expert group discussed many contentious issues, including the need for diagnostic tests for vasoreactivity. Invasive registration of the reaction of the pulmonary vessels to vasodilators – a vasoreactivity testing – in idiopathic, hereditary and drug- and toxin-induced pulmonary arterial hypertension (PAH) is used to determine indications for calcium antagonist therapy, in PAH associated with uncorrected congenital heart disease (CHD) in children – to determine the indications for surgical treatment. The use of a vasoreactivity testing to assess the operability of adult patients with congenital heart disease is inappropriate. An overview of the performance technique, specific pulmonary vasodilators and criteria for evaluating the test for vasoreactivity in various subgroups of PH associated with CHD is given.

About the Authors

A. A. Shmalts
A. N. Bakulev Scientific Center for Cardiovascular Surgery; Ministry of the Russian Federation
Russian Federation

Anton A. Shmalts, Dr. of Sci. (Med.), Leading Research Associate, Associate Professor

Department of Cardiovascular Surgery

121552

Rublyevskoe Shosse 135

Russian State Medical Postgraduate Academy

123995

Barrikadnaya str., 2/1

Moscow



S. V. Gorbachevsky
A. N. Bakulev Scientific Center for Cardiovascular Surgery; Ministry of the Russian Federation
Russian Federation

Sergej V. Gorbachevsky, Dr. of Sci. (Med.), Professor, Head of Department, Professor

Department of Cardiovascular Surgery

121552

Rublyevskoe Shosse 135

Russian State Medical Postgraduate Academy

123995

Barrikadnaya str., 2/1

Moscow



T. V. Martynyuk
E. I. Chazov National Medical Research Center of Cardiology; N. I. Pirogov Russian Medical Research Medical University
Russian Federation

Tamila V. Martynyuk, Dr. of Sci. (Med.), Head of the Department, Professor

Department of Cardiology

121552

3rd Cherepkovskaya str., 15A

117997

Ostrovityanova str., 1

Moscow



S. N. Nakonechnikov
N. I. Pirogov Russian Medical Research Medical University, Russian Medical Society for Arterial Hypertension
Russian Federation

Sergej N. Nakonechnikov, Dr. of Sci. (Med.), Professor, Director

Department of Cardiology

117997

Ostrovityanova str., 1

Moscow



References

1. Чазова И. Е. Евразийские клинические рекомендации по диагностике и лечению лёгочной гипертензии, 2019 // Евразийский кардиологический журнал. – 2020. – 1: 78-124. [Chazova I. E., Martynyuk T. V., Valieva Z. S., Azizov V. A., Barbarash O. L., Veselova T. N. et al. Eurasian clinical guidelines for the diagnosis and treatment of pulmonary hypertension, 2019. Eurasian Journal of Cardiology. 2020; 1: 78-124 (In Russ.)]. https://doi.org/10.38109/2225-1685-2020-1-78-122.

2. Лёгочная гипертензия, в том числе хроническая тромбоэмболическая лёгочная гипертензия. Российские клинические рекомендации, 2020. [Pulmonary hypertension, including chronic thromboembolic pulmonary hypertension. Russian clinical guidelines, 2020 (In Russ.)]. Доступно по ссылке: http://disuria.ru/_ld/10/1026_kr20I27MZ.pdf?ysclid=l95a9m6p93127639965.

3. Galié N., Humbert M., Vachiery J. L., Gibbs S., Lang I., Torbicki A. 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 (1): 67-119. https://doi.org/10.1093/eurheartj/ehv317.

4. Лёгочная гипертензия у детей. Российские клинические рекомендации, 2017. [Pulmonary hypertension in children. Russian clinical guidelines, 2017 (In Russ.)]. Доступно по ссылке: https://diseases.medelement.com/disease/%D0%BB%D0%B5%D0%B3%D0%BE%D1%87%D0%BD%D0%B0%D1%8F-%D0%B3%D0%B8%D0%BF%D0%B5%D1%80%D1%82%D0%B5%D0%BD%D0%B7%D0%B8%D1%8F-%D1%83-%D0%B4%D0%B5%D1%82%D0%B5%D0%B9-%D0%BA%D1%80-%D1%80%D1%84-2017/16752?ysclid=l95ad63otd932307996

5. Caicedo L., Hopper R. K., Garcia H., Ivy D. D., Haag D., Fineman J. et al. Acute Vasoreactivity Testing in Pediatric Idiopathic Pulmonary Arterial Hypertension: an international Survey on Current Practice. Pulm Circ. 2019; 9 (4): 2045894019857533. https://doi.org/10.1177/2045894019857533.

6. Гипертензионная сосудистая болезнь легких, ассоциированная с врождёнными пороками сердца, у детей. Российские клинические рекомендации, 2018. [Hypertensive pulmonary vascular disease associated with congenital heart disease in children. Russian clinical guidelines, 2018 (In Russ.)]. Доступно по ссылке: https://serdceplus.ru/wp-content/uploads/2020/03/2018.-%D0%93%D0%B8%D0%BF%D0%B5%D1%80%D1%82%D0%B5%D0%BD%D0%B7%D0%B8%D0%BE%D0%BD%D0%BD%D0%B0%D1%8F-%D1%81%D0%BE%D1%81%D1%83%D0%B4%D0%B8%D1%81%D1%82%D0%B0%D1%8F-%D0%B1%D0%BE%D0%BB%D0%B5%D0%B7%D0%BD%D1%8C-%D0%BB%D0%B5%D0%B3%D0%BA%D0%B8%D1%85-%D0%B0%D1%81%D1%81%D0%BE%D1%86%D0%B8%D0%B8%D1%80%D0%BE%D0%B2%D0%B0%D0%BD%D0%BD%D0%B0%D1%8F-%D1%81-%D0%B2%D1%80%D0%BE%D0%B6%D0%B4%D0%B5%D0%BD%D0%BD%D1%8B%D0%BC%D0%B8-%D0%BF%D0%BE%D1%80%D0%BE%D0%BA%D0%B0%D0%BC%D0%B8-%D1%81%D0%B5%D1%80%D0%B4%D1%86%D0%B0-%D1%83-%D0%B4%D0%B5%D1%82%D0%B5%D0%B9.pdf?ysclid=l95aejyhw9713238467.

7. Горбачевский С. В. Лёгочная гипертензия у детей с врождёнными пороками сердца / С. В. Горбачевский, А. А. Шмальц, Л. Р. Плотникова. – Москва, 2018. [Gorbachevsky S. V., Shmalts A. A., Plotnikova L. R. Pulmonary hypertension in children with congenital heart disease. Moscow; 2018 (In Russ.)].

8. Горбачевский С. В. Гипертензионная сосудистая болезнь лёгких, ассоциированная с врождёнными пороками сердца / С. В. Горбачевский, А. А. Шмальц // В кн.: Бокерия Л. А. Детская кардиохирургия. Руководство для врачей / Л. А. Бокерия, К. В. Шаталов ; под ред. К. В. Шаталова. – Москва: НЦССХ им. А. Н. Бакулева, 2016. – 833–50. [Gorbachevsky S. V., Shmalts A. A. Hypertensive pulmonary vascular disease associated with congenital heart disease. In: Bokeria L. A., Shatalov K. V. (ed.). Children’s cardiac surgery. Guide for doctors. Moscow: Research Center of Cardiovascular Surgery named after A. N. Bakulev; 2016: 833–50 (In Russ.)].

9. Бокерия Л. А. Лёгочная артериальная гипертензия, ассоциированная с врождёнными пороками сердца у взрослых (клиническая рекомендация) / Л. А. Бокерия, С. В. Горбачевский, А. А. Шмальц // Грудная и сердечно-сосудистая хирургия. – 2017. – 59 (2). – 135-147. [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): 135-147 (In Russ.)]. https://doi.org/10.24022/0236-2791-2017-59-2-135-147 .

10. Горбачевский С. В. Инвазивный мониторинг давления в лёгочной артерии – объективный метод оценки степени тяжести лёгочной гипертензии у детей с врождёнными пороками сердца / С. В. Горбачевский [и др.] // Детские болезни сердца и сосудов. – 2006. – 4. – 77–81. [Gorbachevsky S. V., Belkina M. V., Koledinsky D. G., Shmalts A. A., Marasulov Sh. I., Maltsev S. G. 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.)].

11. 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. https://doi.org/10.1136/heartjnl-2014-307340.

12. Kozlik-Feldmann R., Hansmann G., Bonnet D., Schranz D., Apitz C., Michel-Behnke I. 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): ii42–8. https://doi.org/10.1136/heartjnl-2015-308378.

13. Abman S. H., Hansmann G., Archer S. L., Ivy D. D., Adatia I., Chung W. K. et al. Pediatric Pulmonary Hypertension: Guidelines From the American Heart Association and American Thoracic Society. Circulation. 2015; 132 (21): 2037–99. https://doi.org/10.1161/cir.0000000000000329.

14. Hansmann G., Koestenberger M., Alastalo T. P., Apitz C., Austin E. D., Bonnet D. 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.

15. Rosenzweig E. B., Abman S. H., Adatia I, Beghetti M., Bonnet D., Haworth S. 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.

16. Simonneau G., Montani D., Celermajer D. S., Denton C. P., Gatzoulis M. A., Krowka M. et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019; 53 (1): 1801913. https://doi.org/10.1183/13993003.01913-2018.

17. Kaemmerer H., Apitz C., Brockmeier K., Eicken A., Gorenflo M., Hager et al. Pulmonary hypertension in adults with congenital heart disease: Updated recommendations from the Cologne Consensus Conference 2018. Int J Cardiol. 2018; 272S: 79-88. https://doi.org/10.1016/j.ijcard.2018.08.078

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

19. Xi S. B., Wang S. S., Qian M. Y., Xie Y. M., Li J. J., Zhang Z. W. Predictors of operability in children with severe pulmonary hypertension associated with congenital heart disease. Chin Med J (Engl). 2019; 132 (7): 811-818. https://doi.org/10.1097/CM9.0000000000000145.

20. Del Cerro M. J., Moledina S., Haworth S. G., Ivy D., Al Dabbagh M., Banjar H. et al. Cardiac catheterization in children with pulmonary hypertensive vascular disease: consensus statement from the Pulmonary Vascular Research Institute, Pediatric and Congenital Heart Disease Task Forces. Pulm Circ. 2016; 6 (1): 118-25. https://doi.org/10.1086/685102.

21. Hill K. D., Lim D. S., Everett A. D., Ivy D. D., Moore J. D. Assessment of pulmonary hypertension in the pediatric catheterization laboratory: current insights from the Magic registry. Catheter Cardiovasc Interv. 2010; 76 (6): 865-73. https://doi.org/10.1002/ccd.22693.

22. Douwes J. M., van Loon R. L. E., Hoendermis E. S., Vonk-Noordegraaf A., Roofthooft M. T. R. 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.

23. Xi S. B., Wang S. S., Qian M. Y., Xie Y. M., Li J. J., Zhang Z. W. Predictors of operability in children with severe pulmonary hypertension associated with congenital heart disease. Chin Med J (Engl). 2019; 132 (7): 811-818. https://doi.org/10.1097/CM9.0000000000000145.

24. Zhang D. Z., Zhu X. Y., Meng J., Xue H. M., Sheng X. T., Han X. M. et al. Acute hemodynamic responses to adenosine and iloprost in patients with congenital heart defects and severe pulmonary arterial hypertension. Int J Cardiol. 2011; 147: 433–7. https://doi.org/10.1016/j.ijcard.2010.04.093

25. Oliveira E. C., Ribeiro A. L., Amaral C. F. Adenosine for vasoreactivity testing in pulmonary hypertension: a head-to-head comparison with inhaled nitric oxide. Respir Med. 2010; 104: 606–11. https://doi.org/10.1016/j.rmed.2009.11.010.

26. 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.

27. 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

28. Sitbon O., Humbert M., Jais X., Ioos V., Hamid A. M., Provencher S. 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.

29. Budts W., Van Pelt N., Gillyns H., Gewillig M., Van de Werf F., Janssens S. Residual pulmonary vasoreactivity to inhaled nitric oxide in patients with severe obstructive pulmonary hypertension and Eisenmenger syndrome. Heart. 2001; 86 (5): 553–558. https://doi.org/10.1136/heart.86.5.553.

30. Post M. C., Janssens S., Van de Werf F., Budts W. Responsiveness to inhaled nitric oxide is a predictor for mid-term survival in adult patients with congenital heart defects and pulmonary arterial hypertension. Eur Heart J. 2004; 25: 1651–1656. https://doi.org/10.1016/j.ehj.2004.07.005

31. D’Alto M., Romeo E., Argiento P., Santoro G., Sarubbi B., Gaio G. et al. Pulmonary vasoreactivity predicts long-term outcome in patients with Eisenmenger syndrome receiving bosentan therapy. Heart. 2010; 96 (18): 1475–1479. https://doi.org/10.1136/hrt.2010.199661.


Review

For citations:


Shmalts A.A., Gorbachevsky S.V., Martynyuk T.V., Nakonechnikov S.N. Vasoreactivity testing in pulmonary hypertension associated with congenital heart disease. Eurasian heart journal. 2022;(3):58-64. (In Russ.) https://doi.org/10.38109/2225-1685-2022-3-58-64

Views: 2222


ISSN 2225-1685 (Print)
ISSN 2305-0748 (Online)