Management of Pulmonary Arterial Hypertension in Pregnancy: Experience from a Nationally Accredited Center

Abstract

(1) Background: In pulmonary arterial hypertension (PAH), pregnancy is regarded a contraindication due to high maternal and fetal morbidity and mortality. We report our experience in the management of pregnancies in PAH. (2) Methods: retrospective observational study in a nationally accredited pulmonary hypertension (PH) center from 2013 to 2021. (3) Results: seven pregnancies in six women with PAH, ranging from low to high risk and 21 to 37 years old. Half had known pre-existing PAH before pregnancy. One had a multifetal gestation, and one was pregnant twice under our care. PH medical therapy and serial clinical assessment throughout pregnancy were implemented with focused attention on optimizing right heart function. Delivery was planned by a multidisciplinary team involving PH cardiology, maternal fetal medicine, and obstetric anesthesiology. Patients delivered between 31 and 40 weeks of gestation; five of the seven were via cesarean section. All received regional anesthesia and were monitored in the PH intermediate step-down unit after delivery until discharge. In all cases, delivery was without complications with excellent outcomes for the mother and child. (4) Conclusions: Multidisciplinary and tailored management of PAH in pregnancy, emphasizing optimized right heart function prior to delivery, can result in excellent clinical outcomes in a referral PH center.

Keywords: cardio-obstetrics; maternal fetal medicine; pregnancy; pulmonary hypertension; right heart failure; right ventricular dysfunction.

Figure 1

Echocardiogram from Patient 3 prior to (a,b) and after (c,d) PAH medication optimization. (a) Apical 4 Chamber view of enlarged RA and enlarged and hypertrophied RV with small and underfilled LV, LA; (c) Apical 4 Chamber view of normalized RA and RV size and function on PAH therapy; (b) Parasternal short axis view of severe systolic septal flattening, RV enlargement and hypertrophy, and pericardial effusion; (d) Parasternal short axis view of resolution of systolic septal flattening and pericardial effusion with smaller RV size.

Figure 2

Algorithm Management of PAH during pregnancy. Figure created with Biorender. mPA = mean pulmonary artery; PAWP = pulmonary artery wedge pressure; PVR = pulmonary vascular resistance; WU = Wood units; BNP = brain natriuretic peptide; NT-proBNP = N-terminal pro brain natriuretic peptide; PAH = pulmonary arterial hypertension; JVP = jugular venous pressure; RV = right ventricular; OR = operating room; MFM = maternal fetal medicine; PH = pulmonary hypertension; HF = heart failure.