Abstract Background and aims Pregnancy impose physiological changes that may be difficult to accomplish for women with pulmonary arterial hypertension (PAH). This study evaluates the impact that pregnancy carries in a PAH population managed in a timely manner and whether patients responders to calcium channel blockers (CCB) deserve specific recommendations because of a different prognosis. Methods A retrospective, longitudinal cohort study. All PAH pregnant patients managed at our institution between February 2010 and December 2023 were included. Clinical characteristics at baseline were recorded. Mortality, major complications (maternal death and cardiogenic shock requiring mechanical circulatory support -MCS- or inotropes) and minor complications (hospital admissions for cardiac reason, PAH therapy uptitration and oxygen prescription) were evaluated over the pregnancy course. Long-term follow-up was also assessed. Gestational age, neonatal weight and complications in the new-born (invasive and non-invasive ventilation, inotropic support and neonatal death) were studied. Data were compared between vasoreactive and non-vasoreactive patients. Results 29 patients were studied (median age 31.5, 27-32.7). 8 underwent early termination of pregnancy and 21 decided to proceed. From the latter group, 7 were responders to CCB and none presented complications over the pregnancy course. From the remaining 14 non-respo nders who completed their pregnancy, 13 (92.9%) presented minor complications: 11 (79%) were started on the prostacyclin pathway, 5 (35.7%) required inotropic support, 5 (35.7%) oxygen prescription; and 5 (35.7%) experienced major complications: 2 (14.3%) required MCS, 5 (35.7%) inotropic support, and one patient died (7%). Over a median follow-up period of 5 years (1.2-8.5), 2 patients died from the non-responder cohort (1.4%); while no patient responder to CCB experienced complications; moreover, none lost her vasoreactive response. All the new-borns from non-vasoreactive patients were delivered prematurely; while those from vasoreactive patients reached week 37 in the 85,7% of the cases. One of the new-borns from the non-vasoreactive cohort died, and there was a non-significant trend towards a fewer rate of complications in the vasoreactive offspring (42.9 Vs. 84.6, p = 0.054). Conclusions In spite of the continuous advances in PAH management, pregnancy still associates high maternal and neonatal morbidity. Nevertheless, vasoreactive PAH patients presented a completely different maternal and neonatal prognosis which may prompt specific recommendations for this particular subgroupOutcomes by vasoreactivity profilePregnancy course for the 21 PAH patients