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J Thorac Cardiovasc Surg 2004;127:193-202
© 2004 The American Association for Thoracic Surgery

Surgery for congenital heart disease

Primary repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: A useful approach

^a Division of Cardiovascular Surgery, Hospital San Donato, San Donato Milanese, Italy
^b Division of Cardiovascular Surgery, Hospital Civico of Palermo, Palermo, Italy

Received for publication August 29, 2002; revisions received November 19, 2002; accepted for publication December 2, 2002.

^* Address for reprints: Dr Abella, Divisione di Cardiochirurgia I, Istituto Policlinico San Donato, Via Morandi 30, 20097 San Donato Milanese, Italy
r_abella{at}hotmail.com

BACKGROUND: The ultimate goal of surgical therapy for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is to create unobstructed and separate in series pulmonary and systemic circuits. Our preference has been a 1-stage complete unifocalization technique, avoiding collateral anastomosis with either the native pulmonary arteries or other aortopulmonary collateral vessels.

METHODS AND RESULTS: Since 1998, 5 patients (median age 29.6 months) with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have undergone surgical correction, consisting of (1) exclusion of a descending thoracic aortic segment from which all major aortopulmonary collateral arteries originate, and (2) connection of this aortic segment to the native pulmonary artery using an interposition polytetrafluoroethylene conduit. The ventricular septal defect was closed in all patients, and the right ventricle was connected to the unifocalized pulmonary artery with a valved conduit. All patients survived the operation. Two patients required reexploration for postoperative bleeding. One patient remained on mechanical ventilation for 17 days due to a pulmonary infection. During follow-up (12-21 months), no patient required additional interventions. The postoperative right ventricular/left ventricular pressure ratio was 0.55 median. No significant stenosis within the reconstructed pulmonary circuit was identified. All patients remain free of symptoms, requiring no medications.

CONCLUSION: Intracardiac repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries can be accomplished by a midline 1-stage repair including complete unifocalization of all pulmonary blood supply without individual collateral anastomosis in selected patients. This approach offers a convenient and satisfactory surgical option.

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