Pulmonary atresia with or without a ventricular septal defect is a rare congenital heart disease. The incidence estimated from three large registries and more than four Million neonates is between 0.22 and 0.94 per 10000 life births.1 This condition in its natural course is lethal once the arterial duct closes after birth.2 Even though the condition is rare, it presents with a remarkable morphologic heterogeneity.3 4 Thus, a uniform management strategy cannot be applied to all patients.
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