Management and Outcomes of Pregnancy in Patients with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease

Wen Zhang, Mengyuan Yang, Mei Peng, Yiling Ding

Department of Obstetrics and Gynecology, the Second XiangYa Hospital of Central South University, Changsha, China

ABSTRACT

Background: Pregnant women with pulmonary arterial hypertension associated with congenital heart diseases (PAH-CHD) have a high incidence of mortality and adverse outcomes for mother and child.

Methods: We retrospectively examined the treatment strategies and analyzed the outcomes of pregnancy in patients with pulmonary arterial hypertension managed at a single clinical hospital from 2009 to 2018.

Results: Analysis of all 102 patients with PAH-CHD in pregnancy showed that maternal and newborn death from the disease was low (<3%, 3/102) compared to rates previously reported. Although patients with mild pulmonary hypertension can deliver safely, those with moderate to severe pulmonary artery pressure (PAP), and high functional class tend to have a high risk of heart failure. Medications were selectively administered to patients with more severe disease, and it was, therefore, challenging to make a universal statement on their benefit, but they appear having some benefits in improving birth outcomes for mother and child. While some treatments such as anticoagulant therapy during pregnancy, and oxytocin after delivery, did not improve the health outcome of pregnant women but seemed to provide some benefits to the newborns.

Conclusion: Our retrospective analysis of existing clinical data provides preliminary results for further studies to formally evaluate the efficacy of clinical management of patients with pulmonary arterial hypertension.

KEYWORDS

Congenital heart disease, pulmonary arterial hypertension, pregnant outcome

How to cite this article:

Zhang W, Yang M, Peng M, Ding Y. Management and Outcomes of Pregnancy in Patients with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease. Glob Clin Transl Res. 2020;2(2):29-37.DOI: 10.36316/gcatr.02.0028.

References

1. Task Force on the Management of Cardiovascular Diseases During Pregnancy of the European Society of C. Expert consensus document on the management of cardiovascular diseases during pregnancy. Eur Heart J. 2003;24(8):761-81.

2. D'Alto M, Mahadevan VS. Pulmonary arterial hypertension associated with congenital heart disease. European Respiratory Review. 2012;21(126):328-37.

3. Duffels MG, Engelfriet PM, Berger RM, van Loon RL, Hoendermis E, Vriend JW, et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol. 2007; 120(2):198-204.

4. Engelfriet PM, Duffels MG, Moller T, Boersma E, Tijssen JG, Thaulow E, et al. Pulmonary arterial hypertension in adults born with a septal heart defect: the Euro Heart Survey on adult congenital heart disease. Heart. 2007;93(6):682-7.

5. Weiss BM, Zemp L, Seifert B, Hess OM. The outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. J Am Coll Cardiol. 1998; 31 (7):1650-7.

6. Rubin LJ. Primary pulmonary hypertension. N Engl J Med. 1997;336(2):111-7.

7. Frost AE, Badesch DB, Barst RJ, Benza RL, Elliott CG, Farber HW, et al. The changing picture of patients with pulmonary arterial hypertension in the United States: how REVEAL differs from historic and non-US Contemporary Registries. Chest. 2011;139(1):128-37.

8. European Society of Gynecology, Association for European Paediatric Cardiology, German Society for Gender Medicine, Regitz-Zagrosek V, Blomstrom Lundqvist C, Borghi C, et al. ESC Guidelines on the management of cardiovascular diseases during pregnancy: the Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC). Eur Heart J. 2011;32(24):3147-97.

9. Macchia A, Marchioli R, Marfisi R, Scarano M, Levantesi G, Tavazzi L, et al. A meta-analysis of trials of pulmonary hypertension: a clinical condition looking for drugs and research methodology. Am Heart J. 2007;153(6):1037-47.

10. McLaughlin VV, McGoon MD. Pulmonary arterial hypertension. Circulation. 2006;114(13):1417-31.

11. Stewart R, Tuazon D, Olson G, Duarte AG. Pregnancy and primary pulmonaryhypertension: successful outcome with epoprostenol therapy. Chest. 2001;119(3):973-5.

12. Kraemer HC. A comment on "Beyond p-value: the rigor and power of study." Glob Clin Transl Res. 2020;2(1):7-9. DOI:10.36316/gcatr.02.0022.

13. Bonnin M, Mercier FJ, Sitbon O, Roger-Christoph S, Jais X, Humbert M, et al. Severe pulmonary hypertension during pregnancy: mode of delivery and anesthetic management of 15 consecutive cases. Anesthesiology. 2005;102(6):1133-7; discussion 5A-6A.

14. 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): 256-65.

15. Pitts JA, Crosby WM, Basta LL. Eisenmenger's syndrome in pregnancy: does heparin prophylaxis improve the maternal mortality rate? Am Heart J. 1977;93(3):321-6.

16. Khairy P, Ouyang DW, Fernandes SM, Lee-Parritz A, Economy KE, Landzberg MJ. Pregnancy outcomes in women with congenital heart disease. Circulation. 2006;113(4):517-24.

17. Curry RA, Fletcher C, Gelson E, Gatzoulis MA, Woolnough M, Richards N, et al. Pulmonary hypertension and pregnancy--a review of 12 pregnancies in nine women. BJOG. 2012; 119(6):752-61.

18. McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53(17):1573-619.

19. Duarte AG, Thomas S, Safdar Z, Torres F, Pacheco LD, Feldman J, et al. Management of pulmonary arterial hypertension during pregnancy: a retrospective, multicenter experience. Chest. 2013;143(5):1330-6.

20. Roach MK, Abramovici A, Tita AT. Dose and duration of oxytocin to prevent postpartum hemorrhage: a review. Am J Perinatol. 2013;30(7):523-8.

21. Price LC, Forrest P, Sodhi V, Adamson DL, Nelson-Piercy C, Lucey M, et al. Use of vasopressin after Caesarean section in idiopathic pulmonary arterial hypertension. Br J Anaesth. 2007;99(4):552-5.

22. Jonsson M, Hanson U, Lidell C, Norden-Lindeberg S. ST depression at the cesarean section and the relation to oxytocin dose. A randomized controlled trial. BJOG. 2010; 117(1):76-83.

23. Svanstrom MC, Biber B, Hanes M, Johansson G, Naslund U, Balfors EM. Signs of myocardial ischemia after injection of oxytocin: a randomized, double-blind comparison of oxytocin and methylergometrine during Caesarean section. Br J Anaesth. 2008;100(5):683-9.

24. Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation. 1999;99(14):1858-65.

25. Galie N, Beghetti M, Gatzoulis MA, Granton J, Berger RM, Lauer A, et al. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation. 2006; 114(1) :48-54.