CLINICAL FEATURES OF PULMONARY ARTERIAL HYPERTENSION ASSOCIATED WITH CONGENITAL SYSTEMIC-TO-PULMONARYSHUNTS
https://doi.org/10.38109/2225-1685-2017-4-28-39
Abstract
Pulmonary arterial hypertension (PAH), associated with congenital heart disease (CHD) (PAH-CHD), despite the similarities of histological lesions, is а different from other forms of PAH. PAH-CHD is represented by a heterogeneous patient population with varying defect locations, concomitant diseases, indications and contraindications to surgical correction; also, some patients have a history of a defect closure. According to the European guidelines for the diagnosis and treatment of pulmonary hypertension 2015, there are four main groups in the clinical classification of PAH-CHD: 1) Eisenmenger’s syndrome, 2) PAH associated with prevalent systemic-to-pulmonary shunts, 3) PAH with small/concidental defects, 4) PAH after defects correction. The evolution of the guidelines for the surgical correction of CHD has seen in recent years. Risk stratification has a great importance for further prognosis, response to treatment and timing of surgical correction. For optimal treatment carefulness and expertise in diagnosis and differential diagnosis should be exercised. A better understanding of clinical features, risk stratification and the assessment of the impact of a genetic background will help to determine the best clinical management, which is associated with better quality of life and improved survival in patients with PAH-CHD.
About the Authors
S. Ye. Gratsianskaya
National Medical Research Centre of cardiology of Ministry of Health
Russian Federation
Russian Federation
Postgraduate student of the department of pulmonary hypertension and heart disease
121552, Moscow, 3rd Street Cherepkovskaya, 15 a
tel. 8-495-414-68-42
O. A. Arkhipova
National Medical Research Centre of cardiology of Ministry of Health
Russian Federation
Russian Federation
PhD, Researcher of department of pulmonary hypertension and heart disease
121552, Moscow, 3rd Street Cherepkovskaya, 15 a
tel. 8-495-414-68-33
A. V. Zorin
National Medical Research Centre of cardiology of Ministry of Health
Russian Federation
Russian Federation
PhD, Researcher of the department of pulmonary hypertension and heart disease
121552, Moscow, 3rd Street Cherepkovskaya, 15 a
tel.: 8-495-414-68-42
T. V. Martynyuk
National Medical Research Centre of cardiology of Ministry of Health
Russian Federation
Russian Federation
MD, leading researcher, Head of the department of pulmonary hypertension and heart disease
121552, Moscow, 3rd Street Cherepkovskaya, 15 a,
tel. 8-495-414-64-50
I. Ye. Chazova
National Medical Research Centre of cardiology of Ministry of Health
Russian Federation
Russian Federation
Academician of Russian Academy of Science, MD, Director
121552, Moscow, 3rd Street Cherepkovskaya, 15 a
tel. 8-495-414-63-05
References
1. 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;58:538–546. doi: 10.1016/j.jacc.2011.03.033.
2. 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: 163–172. doi: 10.1161/CIRCULATIONAHA.106.627224.
3. Eisenmenger V. Die angeboren defects des kammerscheidewand des herzen. Klin Med. 1897; 32:1-28.
4. Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J. 1958; 2: 701-709.
5. 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; 30:2493-2537.
6. 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.
7. Morse J.H., Barst R.J., Fotino M. Familial pulmonary hypertension: immunogenetic findings in four Caucasian kindreds. Am Rev Respir Dis. 1992; 145:787–792.
8. 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. doi: 10.1093/eurheartj/eht437.
9. Van de Bruaene A., Delcroix M., Pasquet A., et al. The Belgian Eisenmenger syndrome registry: Implications for treatment strategies? Acta Cardiol. 2009; 64: 447-453. doi: 10.2143/AC.64.4.204160.
10. Olbinskaya L.I., Privalova E.V. Residual pulmonary hypertension. Cardiovascular therapy and prevention. 2003; 6:4-9 [in Russian].
11. 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; 24:371-374. doi: 10.1183/09031936.04.00018604.
12. Arkhipova O.A., Martynyuk T.V., Nakonechnikov S.N., Chazova I.Ye. Comparative assessment of demographic characteristics and functional abilities of patients with PAH in the Russian register and the major foreign registers. Book: abstracts of the III Russian Congress of Pulmonary hypertension. 2015. P. 35-36 [in Russian].
13. 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. doi: 10.1093/eurheartj/eht437.
14. Korten M.A., Helm P.C., Abdul-Khaliq H. et al. Competence Network for Congenital Heart Defects Investigators Eisenmenger syndrome and long-term survival in patients with Down syndrome and congenital heart disease. Heart. 2016; 102: 1552–1557. doi: 10.1136/heartjnl-2016-309437.
15. Сhazova I.Ye., Martynyuk T.V. Pulmonary hypertension. M: Practice, 2015 [in Russian].
16. 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; 50:634-642.
17. Van Dissel A.C., Mulder B.J.M., Bouma B.J. The Changing Landscape of Pulmonary Arterial Hypertension in the Adult with Congenital Heart Disease. Journal of Clinical Medicine, 2017; 6(4), 40. http://doi.org/10.3390/jcm6040040.
18. Blok I.M., van Riel A.C., Schuuring M.J. et al. The role of cystatin C as a biomarker for prognosis in pulmonary arterial hypertension due to congenital heart disease. Int. J. Cardiol. 2016; 209:242–247. doi: 10.1016/j.ijcard.2016.02.003
19. Talwar S., Choudhary S.K., Saxena A. et al. Unidirectional valved patches for closure of septal defects in patients with severe pulmonary hypertension. Ann. Pediatr. Cardiol. 2008; 1: 114–119. doi: 10.4103/0974-2069.43876.
20. D’Alto M., Romeo E., Argiento P. et al. Hemodynamics of patients developing pulmonary arterial hypertension after shunt closure. Int. J. Cardiol.2013; 168:3797–3801. doi: 10.1016/j.ijcard.2013.06.036.
21. Gatzoulis M.A., Beghetti M., Landzberg M.J., Galie N. Pulmonary arterial hypertension associated with congenital heart disease: Recent advances and future directions. Int. J. Cardiol. 2014; 177: 340–347. doi: 10.1016/j.ijcard.2014.09.024.
22. Clavé M.M., Maeda N.Y., Castro C.R. et al. Factors influencing outcomes in patients with Eisenmenger syndrome: a nineyear follow-up study. Pulm Circ. 2017; 7 (3): 635-642. doi: 10.1177/2045893217721928.
23. Schuijt M.T.U., Blok I.M., Zwinderman A.H. et al. Mortality in pulmonary arterial hypertension due to congenital heart disease: Serial changes improve prognostication. Int J Cardiol. 2017; 243:449-453. doi: 10.1016/j.ijcard.2017.05.101.
24. 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: 716–724. doi: 10.1093/eurheartj/eht072.
Review
For citations:
Gratsianskaya S.Ye., Arkhipova O.A., Zorin A.V., Martynyuk T.V., Chazova I.Ye. CLINICAL FEATURES OF PULMONARY ARTERIAL HYPERTENSION ASSOCIATED WITH CONGENITAL SYSTEMIC-TO-PULMONARYSHUNTS. Eurasian heart journal. 2017;(4):28-39. https://doi.org/10.38109/2225-1685-2017-4-28-39
Views:
872
Email this article 
