Improved survival in pregnancy and pulmonary hypertension using a multiprofessional approach

Abstract

Objective Pregnancy in women with pulmonary hypertension (PH) is reported to carry a maternal mortality rate of 30-56%. We report our experience of the management of pregnancies using a strategy of early introduction of targeted pulmonary vascular therapy and early planned delivery under regional anaesthesia.Design Retrospective observational study.Setting Specialist quaternary referral pulmonary vascular unit.Population Nine women with PH who chose to proceed with ten pregnancies.Methods A retrospective review of the management of all women who chose to continue with their pregnancy in our unit during 2002-2009. Main outcome measures Maternal and fetal survival.Results All women commenced nebulised targeted therapy at 8-34 weeks of gestation. Four women required additional treatment or conversion to intravenous prostanoid therapy. All women were delivered between 26 and 37 weeks of gestation. Delivery was by planned caesarean section in nine cases. All women received regional anaesthesia and were monitored during the peripartum period in a critical care setting. There was no maternal mortality during pregnancy and all infants were free from congenital abnormalities. One woman died 4 weeks after delivery following patient-initiated discontinuation of therapy. All remaining women and infants were alive after a median of 3.2 years (range, 0.8-6.5 years) of follow-up.Conclusion Although the risk of mortality in pregnant women with PH remains significant, we describe improved outcomes in fully counselled women who chose to continue with pregnancy and were managed with a tailored multiprofessional approach involving early introduction of targeted therapy, early planned delivery and regional anaesthetic techniques.

Pregnancy outcome in women with Eisenmenger’s syndrome: a case series from west China

“…Therefore, ES is regarded as an absolute contraindication for pregnancy [8]. If a woman with ES chooses to continue with pregnancy, oxygen therapy, aggressive use of pulmonary vasodilator therapies, and care by a specialist multidisciplinary team may help to minimize mortality [11]. …”

Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pregnancy in pulmonary hypertension

“…performed a systematic review of all cases of pregnancy-related death in patients with IPAH, congenital heart disease associated with PAH (CHD-PAH), or PAH associated with other diseases (APAH) published between (1997 and 2007) and compared it with data published between 1978 and 1996 to find out whether there has been any progress made in pregnancy outcome among women with PH using newer therapies. [24] The authors observed that mortality remains prohibitively high, though appears to have decreased more recently with the use of new treatments in all three major subgroups (IPAH from 30% to 17%; CHD-PAH 36% to 28%; APAH 56% to 33%), [Table 1]. Most patients died in the 1 st month after delivery in both series.…”

“…There was no fetal or maternal mortality during pregnancy or in early postpartum period. [24] Another multicenter prospective study of 26 pregnancies showed better outcome with 16 (62%) patients delivering normal healthy babies without complications. Three (12%) patients died and one required urgent heart-lung transplantation.…”

Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Perioperative management in patients with pulmonary hypertension

“…[305354] Nevertheless, maternal mortality remains prohibitively high especially in those with uncontrolled PH. [303254] Thus, pregnancy is discouraged and contraception advice becomes critical in women of child bearing potential who have PH.…”

“…[56] The optimal mode of delivery (vaginal versus caesarean section) and best anesthetic approach (epidural versus general) remain controversial. [30325053545557] Although promising, the effects of advanced PH therapies on pregnancy outcomes remain largely unknown. [30] Endothelin receptor antagonists (including bosentan, ambrisentan and macitentan) are contraindicated during pregnancy due to potential teratogenic effects.…”