Abstract
The outcome of the treatment of the majority of patients with pulmonary hypertension largely depends on the adequacy of the assessment of the patient's condition and the correctness of the chosen treatment tactics. The course of many diseases is accompanied by impaired pulmonary blood flow, which leads to the need not only to diagnose the pathology itself, but also to a thorough study of the state of hemodynamics.
Modern advances in cardiac surgery and related professions such as pediatric cardiology, anesthesiology, perfusion and intensive care, have recently significantly expanded the indications for surgery for congenital heart diseases with high pulmonary hypertension. However, the cornerstone of the problem remains a discussion of the identity of such concepts as the operationability of patients, the reversibility of pulmonary hypertension and the curability of the patient, assuming that the latter includes both of these concepts.
References
- Arrillaga F.C. Scle’rose de l’arte`re pulmonaire secondaire a certains e’tats pulmonaires chroniques. Arch. Mal. Coeur. 1913; 6: 518–29.
- Brenner O. Pathology of the vessels of the pulmonary circulation. Arch. Intern. Med. 1935; 56: 211–37. DOI: 10.1001/ archinte.1935.03920020003001
- Mazzei J.A., Mazzei M.E. A tribute: Abel Ayerza and pulmonary hypertension. Eur. Respir. Rev. 2011; 20 (122): 220–1. DOI: 10.1183/09059180.00006811
- Eisenmenger V. Die angeborenens Defect der Kammerschieidewand des Herzen. Z. Klin. Med. Suppl. 1897; 32: 1–28.
- Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br. Med. J. 1958; 2 (5099): 755–62.
- Forssmann W. Die Sondierung des rechten Herzens. Klein. Wochnschr. 1929; 8: 2085–7. DOI: 10.1007/bf01875120
- Richards D.W. Right heart catheterisation: its contributions to physiology and medicine. Science. 1957; 125 (3259): 1181–5. DOI: 10.1126/science.125.3259.1181
- Burakovsky V.I., Bukharin V.A., Plotnikova L.R. Pulmonary hypertension with congenital heart disease. Moscow; 1975 (in Russ.).
- Manes A., Palazzini M., Leci E., Bacchi Reggiani M.L., Branzi A., Galié N. 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 (11): 716–24. DOI: 10.1093/eurheartj/ eht072. Epub 2013 Mar 1.
- 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. DOI: 10.1093/eurheartj/ehv317
- 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). pii: 1801916. DOI: 10.1183/13993003.01916-2018
- Hansmann G., Apitz C., Abdul-Khaliq H., Alastalo T.P., Beerbaum P., Bonnet D. et al. Executive summary. 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.): ii86–100. DOI: 10.1136/heartjnl-2015-309132
- Gorbachevsky S.V., Shmalts A.A., Plotnikova L.R. Pulmonary hypertension in children with congenital heart disease. Moscow; 2018 (in Russ.).
- Lopes A.A., Barst R.J., Haworth S.G., Rabinovitch M., Dabbagh M.A., del Cerro M.J. et al. Repair of congenital heart disease with associated pulmonary hypertension in children: what are the minimal investigative procedures? Consensus statement from the Congenital Heart Disease and Pediatric Task Forces, Pulmonary Vascular Research Institute (PVRI). Pulm. Circ. 2014; 4 (2): 330–41. DOI: 10.1086/675995
- Cerro M.J., Abman S., Diaz G., Freudenthal A.H., Freudenthal F., Harikrishnan S. 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. DOI: 10.4103/2045- 8932.83456
- Ivy D.D., Abman S.H., Barst R.J., Berger R.M., Bonnet D., Fleming T.R. et al. Pediatric pulmonary hypertension. J. Am. Coll. Cardiol. 2013; 62 (25 Suppl.): D117–26. DOI: 10.1016/j.jacc.2013.10.028
- 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. DOI: 10.1161/cir.0000000000000329
- Diller G.P., Dimopoulos K., Broberg C.S., Kaya M.G., Naghotra U.S., Uebing A. et al. Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case-control study. Eur. Heart J. 2006; 27 (14): 1737–42. DOI: 10.1093/eurheartj/ehl116
- Hopkins W.E., Waggoner A.D. Severe pulmonary hypertension without right ventricular failure: the unique hearts of patients with Eisenmenger syndrome. Am. J. Cardiol. 2002; 89: 34–8. DOI: 10.1016/s0002-9149(01)02159-2 20. Chin K.M., Kim N.H., Rubin L.J. The right ventricle in pulmonary hypertension. Coron. Artery Dis. 2005; 16 (1): 13–8. DOI: 10.1097/00019501-200502000-00003
- Daliento L., Somerville J., Presbitero P., Menti L., BrachPrever S., Rizzoli G. et al. Eisenmenger syndrome. Factors relating to deterioration and death. Eur. Heart J. 1998; 19 (12): 1845–55. DOI: 10.1053/euhj.1998.1046
- Sandoval J., Gomez-Arroyo J., Gaspar J., Pulido-Zamudio T. Interventional and surgical therapeutic strategies for pulmonary arterial hypertension: Beyond palliative treatments. J. Cardiol. 2015; 66 (4): 304–14. DOI: 10.1016/ j.jjcc.2015.02.001
- Hansmann G., Apitz C. Treatment of children with 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 DGPK. Heart. 2016; 102 (Suppl. 2): ii67-85. DOI: 10.1136/heartjnl-2015-309103
About the authors
Sergey V. Gorbachevskiy, Dr. Med. Sci., Professor, Head of Department;
ORCID