Pulmonary arterial hypertension and pregnancy

Česká verzia

Authors: Hutyra M. ¹; Přeček J. ¹; Navrátil K. ²
Authors place of work: I. interní klinika – kardiologická, LF UP a FN Olomouc 2 Interní oddělení, Vojenská nemocnice Olomouc ¹
Published in the journal: Kardiol Rev Int Med 2018, 20(4): 282-285

Summary

Pulmonary arterial hypertension (PAH) is a disease of the small pulmonary arteries that is characterised by vascular proliferation and remodelling. It results in a progressive increase in pulmonary vascular resistance and, ultimately, right ventricular failure and death. Despite recent major improvements in symptomatic treatments, no current treatment cures this devastating condition. Significant advances in the treatment of PAH have been achieved over the past decades. Given the high mortality rate of patients with PAH, pregnancy is not recommended. Patients who do become pregnant and wish to carry on with the pregnancy are treated with PDE-5 inhibitors, prostanoids, and supplemental therapy as needed. Other treatments such as endothelin receptor antagonists are contraindicated because they cause foetal malformations.

Key words:

pulmonary arterial hypertension – pregnancy – delivery – endothelin receptor antagonists – sildenafil – prostacyclin analogues

Zdroje

1. Galiè N, Humbert M, Vachiery JL 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 J2016; 37(1): 67– 119. doi: 10.1093/ eurheartj/ ehv317.

2. Benza RL, Miller DP, Gomberg-Maitland M et al. Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). Circulation 2010; 122(2): 164– 172. doi: 10.1161/ CIRCULATIONAHA.109.898122.

3. Humbert M, Sitbon O, Chaouat A et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 2010; 122(2): 156– 163. doi: 10.1161/ CIRCULATIONAHA.109.911818.

4. Peacock AJ, Murphy NF, McMurray JJ et al. An epidemiological study of pulmonary arterial hypertension. Eur Respir J 2007; 30(1): 104– 109. doi: 10.1183/ 09031936.00092306.

5. Bendayan D, Hod M, Oron G et al. Pregnancy outcome in patients with pulmonary arterial hypertension receiving prostacyclin therapy. Obstet Gynecol 2005; 106(5 Pt 2): 1206– 1210. doi: 10.1097/ 01.AOG.0000164074.64137.f1.

6. Jais X, Olsson KM, Barbera JA et al. Pregnancy outcomes in pulmonary arterial hypertension in the modern management era. Eur Respir J 2012; 40(4): 881– 885. doi: 10.1183/ 09031936.00141211.

7. Duarte AG, Thomas S, Safdar Z et al. Management of pulmonary arterial hypertension during pregnancy: A retrospective, multicenter experience. Chest 2013; 143(5): 1330– 1336. doi: 10.1378/ chest.12-0528.

8. Mandalenakis Z, Rosengren A, Skoglund K et al. Survivorship in children and young adults with congenital heart disease in Sweden. JAMA Intern Med 2017; 177(2): 224– 230. doi: 10.1001/ jamainternmed.2016.7765.

9. Sliwa K, van Hagen IM, 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– 1128. doi: 10.1002/ ejhf.594.

10. Bedard E, Dimopoulos K, Gatzoulis MA. Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension? Eur Heart J 2009; 30(3): 256– 265. doi: 10.1093/ eurheartj/ ehn597.

11. Galiè N, Corris PA, Frost A. Updated treatment algorithm of pulmonary arterial hypertension. J Am Coll Cardiol 2013; 62 (25 Suppl): D60– D72. doi: 10.1016/ j.jacc.2013.10.031.

12. Pulido T, Adzerikho I, Channick RN et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med 2013; 369(9): 809– 818. doi: 10.1056/ NEJMoa1213917.

13. Ghofrani HA, D'Armini AM, Grimminger F et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med 2013, 25; 369(4): 319– 329. doi: 10.1056/ NEJMoa1209657.

14. Sitbon O, Humbert M, Nunes H et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol 2002; 40(4): 780– 788.

15. Sitbon O, Channick R, Chin KM et al. Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med 2015; 373(26): 2522– 2533. doi: 10.1056/ NEJMoa1503184.

16. Galiè N, Barberà JA, Frost AE et al. Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. N Engl J Med 2015; 373(9): 834– 844. doi: 10.1056/ NEJMoa1413687.

17. Xiang Y, Li J, Sun Y. A case report of pulmonary arterial hypertension in pregnancy and complications of anticoagulation therapy. Medicine 2018; 97(32): e11810. doi: 10.1097/ MD.0000000000011810.

18. Regitz-Zagrosek V, Roos-Hesselink JW, Bauersachs Jet al. ESC Scientific Document Group; 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy. Eur Heart J 2018; 39(34): 3165– 3241. doi: 10.1093/ eurheartj/ ehy340.

19. Rokyta R, Hutyra M, Jansa P. 2014 ESC Guidelines on the diagnosis and management of acute pulmonary embolism. Summary document prepared by the Czech Society of Cardiology. Cor Vasa 2015; 57(4): e257– 296. doi: 10.1016/ j.crvasa.2015.05.009.