Pulmonary Arterial Hypertension and Pregnancy: Single Center Experience in Current Era of Targeted Therapy

OBJECTIVES: To report our experiences in pregnant patients with pulmonary arterial hypertension (PAH) who were treated with targeted therapy. METHODS: From 2011 to 2017, women who decided to maintain pregnancies in our PAH clinic were included. Clinical data, management, and outcomes of the mothers and fetuses were reviewed. RESULTS: Nine women with PAH and 10 deliveries were reviewed. The median maternal age was 28 (26-32) years old. The functional status of each patient was New York Heart Association functional class II or III at first visit. Sildenafil was prescribed in advance in 9 cases of delivery. Multidiscipline team approach management and intensive care were performed during the peripartum period. There was no maternal or fetal mortality. Severe cardiac events occurred in 2 patients with Eisenmenger syndrome: cardiac arrest and uncontrolled arrhythmia. Non-cardiac events occurred in 3 cases: postpartum bleeding, urinary tract infection, and pneumonia. The median gestational period at delivery was about 34 (32-38) weeks. Three cases were emergent delivery because of unexpected preterm labor. Intrauterine growth restriction developed in 4 fetuses. CONCLUSIONS: Pregnancy could be maintained by the introduction of targeted therapy rather more safely than the previous era in the case of maintenance of pregnancy. Intensive care and a multidisciplinary team approach can possibly improve the outcomes of the pregnant women with PAH and their babies. However, pregnancy in patients with PAH is still strongly prohibited and it can be tried in expert center where there has sufficient multidisciplinary team approach in case of inevitability.