[Severe idiopathic pulmonary arterial hypertension in pregnancy: a review of 10 cases].

OBJECTIVE

To study the impact of severe idiopathic pulmonary arterial hypertension (IPAH) on pregnancy outcome and to investigate the effect of multidisciplinary approach during pregnancy on the pregnancy outcome in pregnant woman with severe IPAH.

METHODS

Between March 2007 and November 2013, 10 pregnant women with severe IPAH undergoing treatment in Beijing Anzhen Hospital were studied retrospectively.Hemodynamic measurements, medical therapy, manner of delivery, anesthetic administration, multidisciplinary management and outcomes were assessed.

RESULTS

All 10 cases were first diagnosed at the mean of (24 ± 3) weeks during the pregnancy. The systolic pulmonary artery pressure (sPAP) estimated by transthoracic echocardiography was (95.6 ± 1.3) mmHg (1 mmHg = 0.133 kPa) .Seven cases of class III and 3 cases of class IV were recorded by World Health Organization functional class.One patient underwent pregnancy termination at gestational age of 21 weeks with no maternal death or complications, nine patients continued pregnancy and all the patients underwent cesarean section. The mean pregnancy length was (31 ± 5) weeks.Nine had cesarean deliveries during continuous epidural anesthesia, and one during general anesthesia. There were three maternal deaths in hospital (5, 2, 3 days postpartum), and seven patients were alive, and the average hospitalization days was (8 ± 4) days.One fetus lost with cesarean section. Two were term delivery, and seven cases were premature delivery. The average weight is (1 948 ± 731) g and nine were alive and no malformation.

CONCLUSIONS

Because of maternal mortality in patients with severe IPAH remains prohibitively high, patients should continue to be counseled to avoid pregnancy.Women with severe IPAH who become pregnancy should be followed by multidisciplinary approach, and cesarean deliveries during continuous epidural anesthesia are a relatively safe way for pregnancy termination in patients with severe IPAH.