Background and Goal of Study:
Pregnancy with pulmonary arterial hypertension (PAH) have very high risk of acute right-sided heart failure after delivery and may have a sudden death.(1) Eisenmenger syndrome (ES) carry the greatest risk of maternal mortality due to heart failure.(2) Increasing heart strain and vascular resistance itself may further complicate delivery and increase the right heart strain resulting in emergency Caesarean section (CS) and possible maternal demise. Uterine contractions, especially associated with forceps delivery, also adversely affects on pulmonary circulation.(3) The objective was to identify anesthetic management, management and outcomes as suggestion in similar complicated cases because of absence of accepted recommendation in such high cardiac risk parturient.
Materials and Methods: 
A retrospective study included women with PAH which pregnancy risk  IV according WHO admitted for delivery into VUH Santaros Clinics from 2014 to 2017 was conducted. Anaesthesia method, process and medical treatment have been analyzed due to medical records. There were 4 women 25-35 years of age with severe PAH due to congenital heart disease, 3 of them with ES, 1 with residual PAH after late closure patent ductus arteriosus.  
Results and Discussion: 
Decision was made by multidisciplinary team: 1 parturient - vaginal delivery under epidural analgesia, 3 underwent CS under general anaesthesia (GA). All mothers were managed with oxygen, sildenafil and LMWH, 2-diuretics. Two parturient got iloprost inhalations starting from OR. All 4 women treated in ICU for 3-6 days. In one GA case hemodynamic supported with noradrenaline (40h) and milrinone for 51 hours.
Conclusion(s): Successful outcomes was dependant on an individualized approach and multidisciplinary team (cardiologist, obstetrician, anaesthesiologists) collaboration and efforts in operating field and ICU. The clinical situation determined the application of a specific anaesthetic method and GA with adequate pharmacological therapy was the one method in CS.
Learning points: Lower doses of anesthetics have been used in parturients with severe pulmonary hypertension with more pronounced haemodynamic changes during anesthesia. In the time of anesthesia, after the fetal birth, a specific PH treatment with ilioprost inhalation was applied. Only a multidisciplinary team can successfully overcome such challenges with pulmonary artery hypertension.
References :
1)Weiss BM, Zemp L, Seifert B, Hess OM. Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. J Am Coll Cardiol 1998;31:1650–7.
2)Anthony J, Sliwa K. Decompensated Heart Failure in Pregnancy. CardFailRev. 2016;2(1):20-26. doi:10.15420/cfr.2015:24:2.
3)ESC Guidelines on the management of cardiovasc. dis. dur. pregn. Eur Heart J 2011;32:3147–97



10 / Anesthetic management of parturients with severe pulmonary arterial hypertension (pregnancy risk IV WHO) during childbirt