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Long-Term Outcomes of Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collateral
Samuel J Macalister1,2,3, Igor E Konstantinov1,2,3,4, Tyson A Fricke1,2,3
1Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia.
Background:
While complete repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is achieved with low surgical mortality, this condition poses ongoing mid- and late-term mortality. The long-term data for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries at a single institution are presented in this paper.
Methods:
We reviewed the outcomes for 100 patients with PA/VSD/MAPCAs who had surgery between January 1987 and July 2018. Two patients were excluded who had palliative surgery not intended to reach a complete repair.
Results:
Of the 98 patients who were suitable candidates for complete repair, 13% (13/98) had primary complete repair and 64% (63/98) had staged complete repair. Estimated survival at 1, 5, 10, and 20 years of age was 93%, 87%, 81%, and 76%, respectively. The median age of those patients alive at follow-up was 15 (interquartile range 9-21) years. Freedom from reintervention after staged complete repair was 58% at 5 years after complete repair. Ligating or embolizing MAPCAs was associated with a decreased hazard of death in the staged complete repair group (hazard ratio 0.6, p-value 0.04).
Conclusions:
A strategy of staged complete repair can be used to achieve complete repair and stable long-term survival for patients with PA/VSD/MAPCAs.
