Pulmonary arterial hypertension in pregnancy-a systematic review of outcomes in the modern era

Ting-Ting Low; Nita Guron; Robin Ducas; Kenichiro Yamamura; Pradeepkumar Charla; John Granton; Candice K Silversides

doi:10.1177/20458940211013671

Pulmonary arterial hypertension in pregnancy-a systematic review of outcomes in the modern era

Pulm Circ. 2021 May 14;11(2):20458940211013671. doi: 10.1177/20458940211013671. eCollection 2021 Apr-Jun.

Authors

Ting-Ting Low^{1

2}, Nita Guron^{2

3}, Robin Ducas^{2

4}, Kenichiro Yamamura^{2

5}, Pradeepkumar Charla^{2

6}, John Granton⁷, Candice K Silversides^{2

8}

Affiliations

¹ Department of Cardiology, National University Heart Centre Singapore, Singapore.
² Division of Cardiology, Toronto General Hospital, University Health Network, University of Toronto, Toronto, Canada.
³ Department of Cardiology, Libin Cardiovascular Institute of Alberta, Calgary, Canada.
⁴ Section of Cardiology, University of Manitoba, Winnipeg, Canada.
⁵ Department of Paediatrics, Kyushu University, Fukuoka, Japan.
⁶ Harrington Heart & Vascular Institute, University Hospitals, Cleveland, OH, USA.
⁷ Division of Respirology, Department of Medicine, University Health Network, Sinai Health System, University of Toronto, Toronto, Canada.
⁸ Division of Cardiology, University of Toronto Pregnancy and Heart Disease Program, Mount Sinai Hospital and Univeristy Health Network, Toronto, Canada.

Abstract

Pregnancy is hazardous with pulmonary arterial hypertension, but maternal mortality may have fallen in recent years. We sought to systematically evaluate pulmonary arterial hypertension and pregnancy-related outcomes in the last decade. We searched for articles describing outcomes in pregnancy cohorts published between 2008 and 2018. A total of 3658 titles were screened and 13 studies included for analysis. Pooled incidences and percentages of maternal and perinatal outcomes were calculated. Results showed that out of 272 pregnancies, 214 pregnancies advanced beyond 20 gestational weeks. The mean maternal age was 28 ± 2 years, mean pulmonary artery systolic pressure on echocardiogram was 76 ± 19 mmHg. Etiologies include idiopathic pulmonary arterial hypertension (22%), congenital heart disease (64%), and others (15%). Majority (74%) had good functional class I/II. Only 48% of women received pulmonary arterial hypertension-specific therapy. Premature deliveries occur in 58% of pregnancies at mean of 34 ± 1 weeks, most (76%) had Cesarean section. Maternal mortality rate was 12% overall (n = 26); even higher for idiopathic pulmonary arterial hypertension etiology alone (20%). Reported causes of death included right heart failure, cardiac arrest, pulmonary arterial hypertension crises, pre-eclampsia, and sepsis; 61% of maternal deaths occur at 0-4 days postpartum. Stillbirth rate was 3% and neonatal mortality rate was 1%. In conclusion, pulmonary arterial hypertension in pregnancy continues to be perilous with high maternal mortality rate. Continued prospective studies are needed.

Keywords: heart failure; maternal risks; pulmonary hypertension; survival.

Publication types

Review