SummaryThe Fontan procedure is performed for patients with a hypoplastic right ventricle, and pregnancies following this palliative surgery are likely to increase. We present a parturient with the Fontan physiology who successfully underwent two consecutive caesarean deliveries; the first under general anaesthesia for emergency surgery and the second under regional anaesthesia for elective surgery. We suggest that pregnancy and delivery do not typically adversely affect maternal cardiac status in these patients. Attention must be paid, however, to fetal loss, prematurity, growth retardation and associated cardiac congenital malformations for which insufficient data exist in the literature in this patient population. The 2000-2002 Report of the Confidential Enquiries into Maternal Deaths [1] highlighted cardiac disease as the second most frequent cause of maternal death, and 20% of these were cases of congenital cardiac disease. We present a parturient with the Fontan physiology who successfully completed two consecutive pregnancies and deliveries. Previous individual case reports of pregnancy following the Fontan procedure discuss anaesthetic guidelines [2,3] or focus on potential complications during labour and delivery [3,4]. To date, only one study has included a large series, of 33 Fontan deliveries, with regard to maternal and fetal outcome [5]. The novelty of our case resides not only in two successive pregnancies 1 year apart for the same woman, but also in two different anaesthetic approaches for two different obstetric situations. We describe the Fontan procedure and its anaesthetic implications along with a presentation of our conclusions regarding management of such cases.
Case reportA 25-year-old nulliparous patient presented initially at 12 weeks of gestation in her first pregnancy. She was born with a hypoplastic right ventricle and tricuspid atresia, for which she underwent a Blalock-Taussig shunt at 12 years, a Glenn shunt at 19 years and a superseding Fontan procedure at 22 years. The procedure involved transposition of the pulmonary trunk to the right atrium using a tunnel technique with a Gore-Tex tube graft. Following the Fontan procedure she was well with a normal level of activity (New York Heart Association class I).At initial presentation she had no history of dyspnoea, orthopnoea or arrhythmias. She had clubbing, but no cyanosis. There was no peripheral oedema. Blood pressure was 120 ⁄ 85 mmHg and heart rate regular at 82 beats.min )1 . A loud systolic ejection, pansystolic murmur could be heard over the precordium. Lung fields were clear and arterial oxygen saturation was 95% in room air. Echocardiography demonstrated good superior and inferior vena caval flow to the pulmonary artery, good ventricular function, but moderate to severe mitral regurgitation and severe tricuspid regurgitation. Her body weight was 54 kg. At 24 weeks' gestation she was hospitalised with an abnormal fetal ultrasound showing absent end-diastolic