Pregnancy in PAH associated with congenital heart disease: 6-year clinical cases series from the referral center

Introduction: The presence of PAH associated with CHD in pregnant increases mortality up to 30% (50% in Eisenmenger syndrome (sEis) [1]. Due to low prevalence management of these patients are still unclear and each series of clinical cases is of great importance for further analysis.Methods: 18 pregnant with PAH (systolic PAP (SPAP) > 60 mm Hg) who were delivered in 2010 - 2016 were included in the one-centre study. In all cases the main cause of the PAH was the CHD. Data are presented as median (25th, 75th percentile).Results: The age median was 25 (21;28) yr, 12 (66,7%) patients had sEis. At the time of admission SPAP was 85 (70;93) mm Hg, SpO2 - 93 (85; 96)% (in sEis - 87 (78;93)%), 16 females recived PAH therapy before delivery. There were 1 labor at 36 weeks and 17 caesarean section (CS) at 32 (28;34) weeks. Epidural anaesthesia was used in 16 cases and general anesthesia in one CS. NO was inhaled intraoperative to all pregnant. Labor and abdominal deliveries were hemodynamically stable. Decompensation with PAP rise, acute RV failure and hypoxemia developed in all cases at 29 (23;40) h after delivery. All patients required combined PAH therapy (NO, sildenafil, iloprost) and inotropic agents; respiratory support was used in 4 females. The ICU stay was 12,5 (9;24) days. Three patients died (16.7%), mortality in sEis subgroup was 25% (3/12). 15 healthy babies (83.3%) were discharged from the hospital.Collectively, our data show that the strategy we developed in the reffe...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary Circulation and Pulmonary Vascular Disease Source Type: research