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Eur J Cardiothorac Surg 2000;18:480-486
© 2000 Elsevier Science NL

The management of pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries by definitive single stage repair in early infancy

The Children's Mercy Hospital, University of Missouri Kansas City, 2401 Gillham Road, Kansas City, MO 64108, USA

Received 6 September 1999; received in revised form 26 May 2000; accepted 26 July 2000.

Tel.: +1-816-234-3580; fax: +1-816-460-1012

Objective: The management of infants and children with pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries (PA/VSD/MAPCA) has proven to be challenging. Therapeutic approaches have included staged unifocalization, shunting, coiling of collateral vessels, and heart/lung transplantation. Results have been variable and frustrating. Hoping to take advantage of growth potential in pulmonary segments supplied by MAPCA, a more radical approach was adopted in March of 1997. This consists of single stage complete unifocalization with closure of the ventricular septal defect and establishment of right ventricular to pulmonary arterial continuity with a cryopreserved pulmonary allograft (Rastelli type correction) through a midline sternal incision. Methods: During an 18-month period, eleven consecutive infants with PA/VSD/MAPCA underwent complete surgical correction. The ages ranged from 5 days to 5 months. Weights ranged from 2.2 to 5.6 kg. Through a standard median sternotomy incision, the pericardium and both pleural spaces were opened. Normothermic cardiopulmonary bypass was instituted. Section of all collaterals was accomplished without hypoxemia, and all collaterals were ligated at their origin from the aorta. They were then brought through posterior mediastinum to construct a pulmonary artery confluence. The ventricular septal defect was closed, and continuity was established between the right ventricle and the newly created pulmonary artery confluence with cryopreserved allografts. Results: Ten of 11 patients survived operation, with postoperative courses that were uncomplicated. Length of stay ranged from 7–16 days, with a median length of stay of 11 days. One perioperative death occurred in a patient with preoperative co-morbidities of necrotizing enterocolitis, with no functioning gastrointestinal tract, intraventricular hemorrhage, and ventilator dependency since birth. At angiography, this patient has no demonstrable central pulmonary arteries and multiple diminutive aorta pulmonary collaterals. Autopsy revealed no demonstrable pulmonary arteries within the pulmonary parenchyma. All patients have been followed closely, and have grown normally. Two patients undergone repeat cardiac catheterization because of the echocardiographic demonstration of right ventricle pressures that had exceeded 50% of systemic. Both patients were treated with balloon angioplasty and one of these patients has had stenting of stenotic pulmonary arterial segments. No other patients have required additional hospitalization. Right ventricular pressures have remained less than fifty percent of systemic by echocardiographic assessment in all other patients. Conclusions: We feel that a single stage correction of pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries can be accomplished in early infancy with acceptable morbidity and mortality. The initiation of normothermic cardio-pulmonary bypass greatly facilitates dissection of collaterals and prevents hypoxemia. Interventional cardiology with balloon angioplasty and stenting of abnormal pulmonary arterial segments is both an important essential adjunct in the management of these patients. We remain optimistic that a single stage approach to this complex lesion coupled with cardiac catheterization, balloon angioplasty, and stenting, will provide long-term results superior to staged approaches.

Key Words: Pulmonary atresia • Pulmonary atresia with ventricular septal defect • Major aorta pulmonary collaterals • Unifocalization

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