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Long-term outcomes after intervention for pulmonary atresia with intact ventricular septum

Heart Feb 07, 2019

Wright LK, et al. - Researchers performed a cohort study from the Pediatric Cardiac Care Consortium to ascertain the long-term transplant-free survival of patients with pulmonary atresia with intact ventricular septum (PA/IVS) according to treatment strategy. Between 1982 and 2003, neonatal surgery or catheter-based intervention was performed on eligible patients for PA/IVS (median follow-up of 16.7 years). Six hundred sixteen patients with PA/IVS underwent either aortopulmonary shunt, right ventricular decompression or both as initial intervention. Significant infantile and interstage mortality results in poor transplant-free survival in PA/IVS. Earlier birth era (1982–1992), chromosomal abnormality, and atresia of one or both coronary ostia were the risk factors for death at initial intervention. Patients who complete their intended path displayed excellent survival into early adulthood independent of the type of repair.

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