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J Thorac Cardiovasc Surg 2006;132:1099-1104
© 2006 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Superior outcomes for repair in infants and neonates with tetralogy of Fallot with absent pulmonary valve syndrome

^a Department of Cardiothoracic Surgery, Weill Medical School at Cornell University, New York, NY
^b Division of Cardiothoracic Surgery, Columbia University College of Physicians and Surgeons, New York, NY
^c Division of Pediatric Cardiology, Columbia University College of Physicians and Surgeons, New York, NY
^d Division of Pediatric Cardiology, Harvard Medical School, Cambridge, Mass.

Presented at the Seventy-fifth Annual Meeting of the American Academy of Pediatrics, Washington, DC, October 8, 2005.

Received for publication April 5, 2006; revisions received May 12, 2006; accepted for publication May 22, 2006.

^_* Address for reprints: Jonathan M. Chen, MD, Department of Cardiothoracic Surgery, Weill Medical School at Cornell University, 525 E. 68th Street, Box 110-Suite F695B, New York, NY 10021 (Email: jmc23{at}columbia.edu).

OBJECTIVE: Primary repair of tetralogy of Fallot with absent pulmonary valve syndrome has been associated with significant mortality, particularly for neonates in respiratory distress. Controversy persists regarding the method of establishing right ventricle–pulmonary artery continuity.

METHODS: Anatomic and demographic parameters were evaluated for patients undergoing repair of tetralogy of Fallot with absent pulmonary valve syndrome from 1990 to 2005, as were perioperative and late postoperative parameters (airway complications, reoperation or catheter-based intervention, and mortality).

RESULTS: Twenty-three patients underwent repair. Median age was 15 days (range 2-1154 days). Patients were followed up for 5.3 ± 3.9 years. Seventeen (85%) required preoperative ventilatory assistance. One patient died within 24 hours; 1 patient died 8 months postoperatively. Four patients received valved homografts, and the remainder had valveless connections. All patients underwent reduction pulmonary arterioplasty and mobilization, unifocalization (in 3), and ventricular septal defect closure. Valveless connection recipients had a transannular hood. No patient underwent a Lecompte maneuver. Four patients underwent reoperation for conversion to valveless connection (n = 1), reduction arterioplasty (n = 1), and repair of pulmonary stenosis (n = 2). Three patients required catheter-based intervention, with balloon angioplasty (n = 3) and stent placement (n = 1); 2 now demonstrate equal quantitative lung perfusion. No patient has had significant debility from airway compromise. All patients demonstrate free pulmonary insufficiency and good biventricular function.

CONCLUSIONS: We report excellent overall survival (89%) and low postoperative morbidity for neonates and infants undergoing primary repair of tetralogy of Fallot with absent pulmonary valve syndrome. Our recent experience supports the use of a valveless right ventricle–pulmonary artery connection, which, combined with catheter-based intervention, reduces the likelihood of reoperation necessitated by homograft placement.