Abstract

Introduction Occurrence of single ventricle accounts for 0.5–1.5% of the congenital heart disease in whom survival to adulthood, without surgical intervention, is difficult. Hemodynamic changes during pregnancy poses a challenge, resulting in increased risk of maternal and fetal complications such as heart failure, arrhythmias, miscarriage, low birth weight, complications secondary to premature delivery etc. We report the experience of management of women with single ventricle and compare them with concurrent controls. Material & Methods We collected demographic, medical and obstetric details of women with diagnosis of uncorrected single ventricle admitted to a regional tertiary care center in south India during the time period from 2011 to 2017. We also collected the details of 4:1 age matched controls without diagnosis of single ventricle. Outcomes assessed were mode of delivery, maternal mortality, neonatal death and low birth weight. Comparisons were done using Mann–Whitney test or Chi-square test as appropriate. Results We compared 12 pregnancies in 5 women with uncorrected single ventricle to controls. Two women were on Sildenafil for severe pulmonary hypertension (PAH). Sudden death occurred in a primigravida lady at 26 weeks with Complete AV canal defect with severe PAH following delivery. One patient developed heart failure in third trimester. Cesarean Section (12.5% vs 17.5%, p = 0.729) were similar. Pre-term delivery (30% vs 5%, p = 0.014), low birth weight (50% vs 10%, p = 0.002) and perinatal death (25% vs 2.5%, p = 0.002) were higher compared to the controls. Conclusion Women with single ventricle are at higher risk of maternal or fetal adverse events during pregnancy and peri-partum period. Care under multi-disciplinary team can help optimize the outcome of pregnancy if the women wish to continue pregnancy after thorough counselling.

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