Unlabelled: Background The presence ofpulmonary arterial hypertension (PAH) in pregnant women increases mortality up to 12- 30% and up to 50% when PAH is associated with Eisenmenger syndrome. Due to low prevalence of PAH in pregnancy many aspects ofperioperative management are still unclear.

The Aim: To summarize our approaches to the anesthesia and intensive care in pregnant women with PAH.

Materials And Methods: 21 pregnant women with PAH (systolic pulmonary artery pressure (SPAP) higher than 60 mm Hg)-who underwent delivery by Caesarean section in 2010 - 2015 were included in the one-centre retrospective study. Data are presented as median (25th, 75th percentile).

Results: The median age was 27 (23; 29) years. Among the patients, there were 4 (19%) cases of idiopathic PAH and in 17 (81%) women PAH was associated with congenital heart disease (CHD); 12 (57%) patients'demonstrated Eisenmenger syndrome. Baseline SPAP was 90 (82; 103) mm Hg. SpO2 90 (85,95)%. All women taken PAH-specific therapy (sildenafil) before delivery. Caesarean section (CS) were performed at 32 (28; 34) weeks. In 20 cases CS was perfofined under epidural anesthesia and in one case under general anesthesia due thrombocytopenia. Inhaled nitric oxide (NO) was administered intraoperative to all women in a dose of 40-60 ppm. Postoperative period was uncomplicated in five women (23?8%). Decompensation with PAP rise, acute right ventricular failure and hypoxemia developed in 16 (76,2%) cases 30 (24, 40) h after abdominal delivery. These patients required combined PAH-specific therapy (NO, sldenafil, iloprost) and inotropic agents, additionallyrespiratory support was used in four patients. The median ICU stay was 13 (9; 22) days. 3 patients died (14?2%); mortality in Eisenmenger syndrome cases was 25% (3/12). 18 healthy babies.

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