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Ann Thorac Surg 2006;82:146-152
© 2006 The Society of Thoracic Surgeons

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Original article: Cardiovascular

Analysis of Surgical Outcome in Complex Double-Outlet Right Ventricle With Heterotaxy Syndrome or Complete Atrioventricular Canal Defect

^a Department of Cardiac Surgery, Children's Hospital, Harvard Medical School, Boston, Massachusetts
^b Department of Anesthesiology, Children's Hospital, Harvard Medical School, Boston, Massachusetts
^c Department of Biostatistics, Children's Hospital, Harvard Medical School, Boston, Massachusetts
^d Department of Cardiovascular Surgery, Saiseikai Kyotofu Hospital, Tokyo, Japan

Accepted for publication February 2, 2006.

^_* Address correspondence to Dr Takeuchi, Department of Cardiovascular Surgery, National Children's Hospital, Japan, 2-10-1 Ohkura, Setagaya, Tokyo, 157-8535, Japan (Email: koutakeuchi-circ{at}umin.ac.jp).

BACKGROUND: Double-outlet right ventricle encompasses a broad spectrum of anomalies. Heterotaxy syndrome, which is often associated with total anomalous pulmonary venous connection and complete atrioventricular canal defect, has been considered a risk factor for surgical repair of double-outlet right ventricle.

METHODS: From January 1992 to May 1999, medical records of 96 patients (50 males, 46 females) who had complex double-outlet right ventricle with heterotaxy and/or complete atrioventricular canal defect were reviewed (median age at initial surgery 3 months). Seventeen patients were neonates requiring surgery. Follow-up ranged from 1 day to 7.4 years (median, 16 months).

RESULTS: Sixty-eight patients had heterotaxy syndrome (27 with total anomalous pulmonary venous connection). Eighty-three had complete atrioventricular canal defect, 22 with moderate to severe atrioventricular valve regurgitation at the time of surgical repair. Eight patients had two-ventricle repair, and 88 patients were considered for single-ventricle management (bidirectional Glenn, 37; Fontan, 44). One patient had heart transplantation after bidirectional Glenn. There were 16 deaths including 10 early (<30 days postoperatively). Overall survival (95% confidence interval) estimated by the Kaplan–Meier method was 89% (83% to 96%) at 1 month, 84% (76% to 91%) at 1 year, and 81% (73% to 89%) at 5 years. Multivariate analysis revealed that neonatal presentation requiring surgery (p < 0.0001), moderate to severe atrioventricular valve regurgitation (p = 0.03), and pulmonary venous obstruction (p = 0.02) were risk factors for death.

CONCLUSIONS: Atrioventricular valve regurgitation, pulmonary venous obstruction, and neonatal presentation are risk factors for mortality in patients with complex double-outlet right ventricle. Early surgical intervention in symptomatic neonates and infants, including those with pulmonary venous obstruction, may reduce mortality and improve outcome after staged operation.

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