The multidisciplinary management of pulmonary arterial hypertension patients in pregnancy and postpartum: A case series

Abstract

Study objective: Pregnancy has been contraindicated in the setting of pulmonary arterial hypertension (PAH) due to elevated maternal and fetal risk. We review our center's successful experience with pregnant PAH patients in this case series.

Study design: This is a retrospective case series.

Participants: Six patients with PAH with seven pregnancies delivered at Tampa General Hospital from 2014 to 2024.

Interventions: All cases involved a multidisciplinary team to optimize antenatal and intrapartum management. The REVEAL 2.0 risk assessment tool also guided treatment planning by determining risk status.

Results: Almost all of the patients in our study were diagnosed with PAH by right heart catheterization at our institution pre- or during pregnancy. All patients were either on a phosphodiesterase inhibitor or prostacyclin during their pregnancies. One mother was admitted at 34 weeks 1 day for decompensated heart failure without prior PAH treatment. All patients with outpatient follow-up had serial 6 min walk distances, brain natriuretic peptide levels, echocardiograms, and right heart catheterizations, and were able to achieve a low risk REVEAL 2.0 score during the pregnancy. All patient cases delivered via cesarean section with shared decision making and 5/7 cases were preterm. There was a 100 % maternal and fetal survival rate in our case series within the 1 year postpartum period.

Conclusion: Although pregnancy is a relative contraindication in patients with PAH, our case series demonstrates that a multidisciplinary approach to care at a specialized center for pulmonary vascular disease with close, personalized ante- and postnatal management can result in successful outcomes.

Keywords: Pregnancy; Pulmonary arterial hypertension; Pulmonary hypertension; REVEAL.

Fig. 1

Echocardiogram of Patient 6 at initial presentation to TGH. Right ventricle cavity size appears markedly dilated. Systolic function is reduced. The estimated peak right ventricular systolic pressure was 81 mmHg. Peak Tricuspid Regurgitation Velocity is 4.50 m/s. Right atrium is massively dilated.

Fig. 2

Echocardiogram of Patient 1 at initial evaluation (a & b) showing a parasternal short axis view with normal LV function, no signs of RV dilatation, and no interventricular septal bowing or flattening. Echocardiogram of Patient 1 one year postpartum (c & d) showing a parasternal short axis view with normal LV function, interventricular septal flattening and bowing, a dilated right ventricle, and a small pericardial effusion.