Uncorrected total anomalous pulmonary venous drainage in pregnancy

SummaryTotal anomalous pulmonary venous drainage is a rare form of congenital heart disease. It usually presents in the neonatal period, although later presentation, including in adulthood, is known to occur. We could not find any accounts of adult survivors with the undiagnosed disease becoming pregnant. We describe the case of a 19-year-old Bengali primiparous woman who arrived in the UK at 27 weeks’ gestation and needed an urgent caesarean section for intrauterine growth restriction at 34 weeks’ gestation. Uncorrected congenital heart disease was diagnosed at this time although the exact nature of the pathology was not clear. She underwent an uncomplicated caesarean section using a combined spinal-epidural technique with invasive monitoring. Intrathecal 0.5% hyperbaric bupivacaine 0.7 mL and fentanyl 25 μg were sufficient for surgery. She remained cardiovascularly stable throughout the procedure and a female infant was successfully delivered. She underwent corrective cardiac surgery 14 months after delivery. To our knowledge, this is the first report of caesarean section in a patient with uncorrected total anomalous pulmonary venous drainage. In this case, regional anaesthesia was successfully used.