Complete Repair of Ebstein Anomaly in Neonates and Young Infants: A 16-Year Follow-Up

Study Questions:

What is the long-term outcome for patients with Ebstein anomaly who underwent repair as a neonate or young infant?


This was a retrospective study of 32 symptomatic patients (23 neonates and 9 infants) of whom 29 underwent biventricular ventricle repair for Ebstein anomaly between March 1994 and May 2010. Primary outcomes included early and late survival, freedom from reoperation, durability of tricuspid valve repair, and functional status.


Median age at the time of operation was 15 days, with a mean weight of 3.9 ± 2 kg. A total of 29 patients underwent biventricular repair, of whom 26 required tricuspid valve repair and two required replacement. Early survival was 78.1%, with an estimated 15-year survival estimate of 74 ± 8%. Early survival was less with pulmonary atresia (PA) (60%, 9/15) than without PA (94.1%, 16/17), p = 0.05. Mean follow-up was 5.9 ± 4.5 years (range, 0.1-16 years). Biventricular repair was achieved in 90.6%, with a 15-year freedom from reoperation of 74 ± 10%. Survivors were all in New York Heart Association class I or II.


In select symptomatic neonates, a biventricular repair is feasible, with reasonable early and late survival, using a variety of techniques including valve repair and replacement. The results are durable, with good late functional status.


Symptomatic neonates with Ebstein anomaly are a very challenging patient population. Surgical options depend greatly on the specific anatomy of the tricuspid valve, size of the functional right ventricle, as well as associated pulmonary atresia. In select patients, biventricular repair is feasible. This diagnosis is associated with a very high early mortality in excess of 20% regardless of single versus biventricular repair.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Interventions, CHD and Pediatrics and Quality Improvement, Interventions and Structural Heart Disease

Keywords: Survivors, Infant, Newborn, Follow-Up Studies, Heart Defects, Congenital, Boston, Ebstein Anomaly, Cardiac Surgical Procedures, New York

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