Perioperative Management for Surgical Repair of Ebstein's Anomaly.

Abstract

Background: Ebstein's anomaly is a rare and complex heart defect that affects the tricuspid valve and is accountable for around 1% of congenital cardiac abnormalities. It is one of the most common congenital causes of tricuspid valve regurgitation. Ebstein's anomaly often is diagnosed prenatally, due to its severe cardiomegaly. Some individuals with this anomaly do not experience complications until adulthood and even then, they have mostly minor complaints like exercise intolerance. An atrial septal defect is most commonly (70-90%) associated with Ebstein's anomaly. However, ventricular septal defect (VSD) can be associated with 2-6% of the cases.
 Case presentation: This report presents a case of surgical intervention for a 38-year-old female with Ebstein's anomaly symptomatic with moderate-severe pulmonary regurgitation and foramen ovale apertum.
 Conclusions: Ebstein anomaly is a complicated form of congenital heart disease with variable clinical presentations. The anesthetic plan must also focus on maintenance of RV function and avoidance of increase in PVR. Reversible causes of increased PVR, such as acidemia, hypoxemia, and hypercarbia must be avoided. Agents that lower PVR, such as nitrates, and nitric oxide may be beneficial in patients with severe pulmonary hypertension.
 With a sound knowledge of the cardiac anatomy, accurate scheming of surgical outcomes, routine follow-ups, multidisciplinary team approach, and better management, an experienced center can ultimately improve the prognosis of such patients.