Title: Anaesthetists’stress test: Pulmonary arterial hypertension (PAH) in pregnancy.

Background: Maternal mortality for pregnant women with PAH remains high. Current guidelines clearly recommend that women with PAH should not become pregnant or terminate pregnancy. However, guidelines for the management of such parturients do not exist. We present the management of two cases at our department.

Case reports:
•	A 40-year-old G2P1 female at 38th week of gestation presented to the emergency room with a complaint of dyspnea at rest. She reported a known asymptomatic mitral valve prolapse and a newly diagnosed mild bronchial asthma. Transthoracic echocardiogram (TTE) showed an estimated pulmonary artery systolic pressure (PASP)=70mmHg. An emergency C-section was decided because of obstetrical reasons and was performed under low dose spinal anaesthesia because of thrombocytopenia. Immediately after delivery the mother developed pulseless electrical activity. Death was confirmed after 90 minutes of unsuccessful advanced life support.
•	A 34-year-old G1P0 female at 34th week  of gestation was admitted to our high risk pregnancy unit due to intrauterine growth restriction and a current history of atrial septal defect, left to right shunt and estimated  PASP=45-50mmHg. Elective C-section was performed successfully under epidural anaesthesia. Admission to the cardiac unit followed uneventfully.
Discussion: Pregnancy in PAH is difficult to deal with. Therapy has to be initiated as soon as possible. Close follow-up by a multidisciplinary team is mandatory. Severity of PAH and functional status of mother definitely affect prognosis. The safest mode of delivery seems to be elective C-section in 32-36 week of gestation. The main anaesthetic goals are avoidance of hypoxaemia, marked reduction in systemic vascular resistance and myocardial depression. Epidural or low-dose combined spinal epidural anaesthesia are preferable to general anaesthesia under meticulous monitoring. The immediate postpartum period is the most common for development of acute right ventricular failure.

References:
•	Olsson KM, Channick R. Pregnancy in pulmonary arterial hypertension.EurRespirRev. 2016 Dec;25(142):431-437.
•	Rex S, Devroe S. Anesthesia for pregnant women with pulmonary hypertension. CurrOpinAnaesthesiol. 2016 Jun;29(3):273-81.

Learning points: Early consultation, diagnosis and therapy are crucial in order to optimize patient’s clinical status. Elective delivery should be performed under regional anaesthesia. Postpartum period posses the highest incidence of mortality.


12 / Anaesthetists’stress test: Pulmonary arterial hypertension (PAH) in pregnancy.