Anaesthetic Management of a Pregnant Lady with Epstein Anomaly

Abstract

Wilhelm Ebstein first described the clinical and anatomical features of an anomaly of the tricuspid valve in 1866, which occurs in 1 percent of congenital heart defects (1 in 110,000 of the general population). It is characterized by dysplastic abnormalities of both basal and free attachments of the tricuspid valve leaflets, with downward displacement and elongation of the septal and anterior cusp, with resulting tricuspid regurgitation. Congestive heart failure and sudden collapse are the most common causes of death Case Report: A 21 year old primi with 37 wks gestation was referred to our hospital for complicated pregnancy management. She complained of occasional palpitations, and dyspnoea on severe exertion. She had no history of recurrent chest infections, cyanosis or heart failure in the past. On examination she had pansystolic and mid-diastolic murmurs with right parasternal thrill, along with widely split second heart sound. The ECG showed sinus rhythm with right bundle branch block. Chest X-ray showed enlarged right atrium with cardiomegaly, while echocardiogram showed the presence of moderately enlarged right atrium, tricuspid regurgitation with downwardly displaced tricuspid valves, confirming diagnosis of Ebstein’s anomaly. Her blood reports were within normal limits. We kept ready all routine and emergency drugs and equipments. Subsequently she was shifted into the OT, monitors were attached, and given antibiotics for bacterial endocarditis prophylaxis. A sitting epidural was tried at L3-4 interspace. INJ bupivacaine 0.5% 15cc given in graded doses of 5 cc every 5 minutes by monitoring hemodynamic response. Level achieved till T6. Thereafter left lateral tilt and oxygen were continuously administered to the patient. Intraoperatively her vitals were stable and duration of operation was 60 minutes and a female baby of birth weight 2.4kg was extracted. Postoperatively the patient vitals were stable. Conclusion: In conclusion, women with Ebstein’s anomaly may present with a multitude of problems and, should be considered as high risk and cared for in tertiary centres by a multidisciplinary team including obstetricians, cardiologists and obstetric anaesthetists in pregnancy and during delivery. Epidural anaesthesia in a fractionated or ‘graded’ manner provides a haemodynamically stable patient with adequate analgesia, and may be preferred to general anaesthesia in these patients.