Repair of neonates and young infants with Ebstein's anomaly and related disorders.
BACKGROUND
Severely symptomatic neonates and young infants with Ebstein's anomaly usually die without surgical intervention. The relative risks and benefits of single-ventricle palliation versus a two-ventricle repair are uncertain. In a recent series, 69% early survival with single-ventricle palliation was reported in 16 neonates with Ebstein's anomaly. Our institutional bias has been to do a two-ventricle repair in all such patients.


METHODS
We reviewed our entire surgical experience with a two-ventricle repair in the severely symptomatic neonate (n = 22) and young infant (n = 5). The indications for operation were ventilator dependence, severe cardiac failure, prostaglandin-dependent circulation, and gross cardiomegaly.


RESULTS
Between 1994 and 2006, 27 consecutive patients with Ebstein's anomaly underwent operation. Associated comorbidities included anatomic or functional pulmonary atresia (n = 18), ventricular septal defects (n = 3), small left ventricle (n = 3), hypoplastic branch pulmonary arteries (n = 3), previous cardiac surgery (n = 4), significant intracranial hemorrhage (n = 3), hepatic necrosis and renal insufficiency (n = 3), and malignant tachyarrhythmias (n = 4). Operations consisted of tricuspid valve repair (n = 23) or valve replacement (n = 2), Blalock-Taussig shunt only (n = 1), and bilateral pulmonary arterioplasty with bidirectional Glenn (n = 1). Hospital survival was 74%, and there have been no late deaths during a median follow-up period of 5.4 years (range, 0.2 to 12 years). Three patients required tricuspid valve replacement during the follow-up period. Late arrhythmia requiring medication is present in 1 patient. All patients are currently in New York Heart Association functional class I.


CONCLUSIONS
Two-ventricle repair currently has similar early survival compared with single-ventricle palliation. The advantages of a better physiologic repair can be anticipated for a longer follow-up period.
Knott-Craig
Christopher J
CJ
Department of Thoracic and Cardiovascular Surgery, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA. ckc@uab.edu
Goldberg
Steven P
SP
Overholt
Edward D
ED
Colvin
Edward V
EV
Kirklin
James K
JK
D004437:Ebstein Anomaly / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D006330:Heart Defects, Congenital / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D006343:Heart Septal Defects / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D020300:Intracranial Hemorrhages / Q000453:epidemiology; D008099:Liver / Q000473:pathology; D009336:Necrosis; D018579:Patient Selection; D012121:Respiration, Artificial; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome; D014261:Tricuspid Valve / Q000601:surgery