When pulmonary arterial hypertension and pregnancy meet: a multidisciplinary clinical experts review

Pulmonary hypertension (PH) is a rare condition characterized by elevated pulmonary arterial pressure and pulmonary vascular resistance, potentially leading to right ventricular failure. Pulmonary arterial hypertension (PAH) is the most common type of PH in women of childbearing age and, as per the modified World Health Organization (mWHO) classification of maternal cardiovascular risk, it falls into mWHO class IV and pregnancy is contraindicated. These patients face an exceptionally high risk of maternal mortality and morbidity, with estimated maternal cardiac event rates ranging from 40 to 100% during pregnancy, because physiological changes happening in pregnancy exacerbate the disorder. Despite these recommendations, there is a growing incidence of pregnancy among women with PAH. Early referral to specialized centers, personalized therapies and expert multidisciplinary care involving pulmonary hypertension specialists, obstetricians, critical care specialists, anesthesiologists, and neonatologists are crucial steps to ensure positive outcomes for both mother and fetus. This review aims to examine the current understanding of pregnancy in patients with PAH, drawing on the experience of our center in the multidisciplinary management of pregnant women with this condition. In particular, we want to focus the attention of clinicians on the following aspects: early referral of pregnant patients to specialized centers, detailed counseling on the implications of pregnancy, initiation of therapy in treatment-naive patients and potential adjustment of therapy in non-naive patients, periodic risk assessment, evaluation of the appropriate timing of delivery, multidisciplinary management of the most critical periods, which are delivery and the post-partum phase.