PAH management emphasizing right heart function in pregnancy led to better clinical outcomes

Multidisciplinary and tailored management of pulmonary arterial hypertension in pregnancy that emphasizes optimized right heart function before birth may lead to better clinical outcomes in a referral pulmonary hypertension center.

“Pulmonary hypertension management during pregnancy, as with the management of other cardiovascular disorders in pregnancy, should be aimed at optimizing the central pathophysiology of the condition and providing the greatest degree of achievable physiologic reserve in anticipation of the physiologic stresses inherent to pregnancy, labor and delivery,” Anjali Vaidya, MD, FACC, FASE, FACP, professor of medicine at the Lewis Katz School of Medicine at Temple University and co-director of the Pulmonary Hypertension, Right Heart Failure & CTEPH Program at the Temple Heart and Vascular Institute at Temple University Hospital, and colleagues wrote.

This retrospective, observational study, published in Journal of Cardiovascular Development and Disease, included seven pregnancies in six women with low to high risk PAH aged 21 to 37 years from from 2013 to 2021. Half of these women had preexisting PAH before their pregnancy and two women were diagnosed with PAH during their first pregnancy. One woman had multifetal gestation and one woman was pregnant twice during the study period.

Researchers implemented pulmonary hypertension therapy and serial clinical assessment throughout pregnancy with emphasis on optimizing right heart function in all women. Those not on PAH therapy at the time of presentation had more severe right ventricular dilation, dysfunction, tricuspid valve regurgitation and had evidence of mid-systolic notching of the right ventricular outflow tract Doppler signal. Comparatively, those on PAH therapy before their pregnancy had a less severe right heart dysfunction phenotype with normal to mild right ventricular dysfunction and evidence of lower pulmonary vascular resistance with no notching or a late-notch pattern of the right ventricular outflow tract Doppler signal.

These women delivered between 31 and 40 weeks of gestation; five women required cesarean section delivery. All women received regional anesthesia and were monitored in the pulmonary hypertension intermediate step-down unit after delivery. All deliveries were successful without complications and with excellent outcomes for the mother and child, the researchers wrote.

“A multidisciplinary approach with clear planning and communication is paramount to ensure the safest possible outcomes for this high-risk medical condition,” they wrote.