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J Thorac Cardiovasc Surg 1998;116:924-931
© 1998 Mosby, Inc.
SURGERY FOR CONGENITAL HEART DISEASE |
From the Divisions of Cardiology and Cardiothoracic Surgery, Children's Hospital of Philadelphia, and the Departments of Pediatrics and Surgery, University of Pennsylvania School of Medicine, Philadelphia, Pa.
Read at the Seventy-eighth Annual Meeting of The American Association for Thoracic Surgery, Boston, Mass, May 3-6, 1998.
Received for publication May 8, 1998. Revisions requested June 18, 1998.Revisions received Aug 18, 1998. Accepted for publication Aug 18, 1998. Address for reprints: Jack Rychik, MD, Division of Cardiology, Children's Hospital of Philadelphia, 324 S 34th St, Philadelphia, PA 19104.
Objective:Pulmonary atresia with intact ventricular septum is an anatomically heterogeneous anomaly with a variety of surgical strategies possible. We sought to compare the outcome of patients with a single ventricle approach to those with a biventricular repair and to compare outcome of patients with coronary abnormalities to those with normal coronary arteries.
Methods: A retrospective review of our surgical database revealed 67 patients with pulmonary atresia with intact ventricular septum operated on between 1981 and 1998. Patients were categorized on the basis of initial surgical strategy: strategy A, aortopulmonary shunt alone (n = 31); strategy B, right ventricular recruitment (n = 32); strategy C, heart transplantation (n = 4). Tricuspid valve size (Z-score) and coronary anatomy were determined. Right ventricular–coronary artery dependency was noted in 8 patients.
Results: Overall actuarial survivals at 1, 5, and 8 years were 82%, 76%, and 76%. Mortality was highest in infancy (10 of 16 deaths). Outcome was equivalent for all 3 strategies. There was no difference in tricuspid valve size between survivors and nonsurvivors (mean Z-score –2.0 (2.5) vs –2.0 (1.9), P = .83). There was no difference in survival based on severity of coronary abnormality. Only one third of patients had a successful biventricular repair, and the tricuspid valve was significantly larger in these than in patients who had Fontan operation (mean Z-score –0.53 [1.6], range –3.5 to 1, versus mean Z-score –3.03 [2.7], range –5.5 to 0, P = .002).
Conclusions: Surgical outcome for patients born with pulmonary atresia with intact ventricular septum is satisfactory. The strategies of biventricular repair, single ventricle palliation, and heart transplantation allow for equal outcome among all anatomic subtypes.
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