Pulmonary hypertension and pregnancy. Is it time to reconsider recommendations in certain groups? Contemporary outcomes in a tertiary referral centre

Abstract Background and aims Pregnancy is as a major risk scenario for pulmonary hypertension (PH) females. Disease targeted therapy (DTT) has dramatically changed prognosis in certain PH groups, namely: pulmonary arterial hypertension (PAH), chronic thromboembolic PH (CTEPH) or segmental-PH; also facilitating pregnancy management. In spite a more benign course in the responders to calcium channel blockers subgroup (R-CCB), maternal mortality is thought to remain high and current recommendations still consider all PH-patients as very high risk (mWHO IV). We aimed to analyse pregnancy outcomes in PAH, CTEPH and segmental-PH in a contemporary cohort and to specifically assess pregnancy risks in the R-CCB subgroup. Methods and results All pregnant PAH, CTEPH and segmental PH patients and patients transferred within 8 weeks post-delivery between January 2000 and January 2020 to our centre were studied. 28 pregnancies were included. Mean age 28±6 years. Underlying aetiology: 10 (36%) Idiopathic-PAH, 4 (14%) Heritable-PAH (1 of them veno-occlussive disease-POVD-), 2 (7.1%)PAH-connective tissue disease, 9 (32,1%) PAH-congenital heart disease (1 Eisenmenger), 1 (3.6%) CPETH, 2 (7.1%) segmental-HP. 21% were R-CCB. From the overall 28 pregnancies, 32% underwent early termination of pregnancy (ETP). When pregnancy was continued in non-responders, 5 (23%) were admitted from cardiac causes and up to 13% required inotropes. 66.7% of non-responders patients needed uptitration of PH-DTT along pregnancy (19% being discharged on prostacyclins). Considering the whole cohort, two patients (7.1%) died along the pregnancy period, both non-responders PAH (1 IPAH and 1 POVD). One died at ETP and the other one 48 hours after C-section at week 22. No R-CCB presented with maternal complications, and all continued on CCB without needing any change on treatment. Regarding the new-born, average gestational age was 28 weeks and 53% suffered from some neonatal morbidity (only 1 R-CCB), including 1 neonatal death. From a haemodynamic perspective, baseline mean pulmonary artery pressure (mPAP) was 41.9±15 mmHg, pulmonary vascular resistances (PVR) were at 7.1±4 WU. If haemodynamics were also available after pregnancy (53%), no significant increase in PVR was noted, although the required DTT was remarkably higher. Survival-free of death or lung transplantation (Figure 1) was calculated at 78.57% over a median long-term follow-up of 6 years. Conclusions DTT has improved outcomes in pregnant