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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 10 000 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 morphological heterogeneity.3 4 Thus, a uniform management strategy cannot be applied to all patients.
The earliest approach to treat the condition was surgical,2 and surgery is still more frequently performed than catheter-based treatment.4 Several modifications of the operative management have been made to improve the outcome. After initial construction of a systemic-pulmonary shunt and/or opening the right ventricular outflow tract, the children receive—depending on the anatomy of the right ventricle—either a one or a one-and-a-half ventricle palliation, or a biventricular repair. To achieve the latter several modifications, like infundibular resection, sinus resection and tricuspid and pulmonary valvotomy are performed, as indicated.5 Current studies show that the overall 5-year-survival is between 63% and 85%,4 5 and about 45% of …
Competing interests None.
Provenance and peer review Commissioned; externally peer reviewed.