Pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries is an extremely complex, heterogeneous, and rare anomaly. This group of patients may not be able to survive until adulthood without any interventions or treatment. Although surgical management of patients diagnosed in newborn, infant, or early childhood is clear, treatment of patients diagnosed in adulthood still remains a significant problem. The pre-operative clinical status, imaging methods, and operative findings might be helpful for planning the most appropriate management. Herein, we report a unique case of pulmonary atresia and ventricular septal defect with major aortopulmonary collateral arteries who remained asymptomatic until the age of 18 years.
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