© Copyright 2026 American Medical Association. All rights reserved.
Repair of pulmonary atresia with ventricular septal defect (VSD) is a complex surgical procedure aimed at correcting a congenital heart defect where the pulmonary valve does not develop properly, leading to an obstruction of blood flow from the right ventricle to the lungs. In this condition, the heart's ability to deliver oxygenated blood to the body is compromised. The presence of a VSD, which is an abnormal opening in the septum separating the right and left ventricles, further complicates the situation by allowing blood to flow between the two ventricles. This procedure is specifically designed for patients who exhibit both pulmonary atresia and a VSD, and it requires careful planning and execution due to the variability in the anatomical presentation of each patient. The surgical approach is often tailored to the individual, and the repair may be performed in stages, depending on the severity of the defects and the overall health of the patient. The procedure involves accessing the heart through a median sternotomy, harvesting a patch of pericardium for grafting, and establishing cardiopulmonary bypass to facilitate the repair. The surgeon evaluates the anatomy of the pulmonary artery and its branches to determine the best site for constructing or replacing a conduit that will allow blood to flow from the ventricle to the pulmonary artery, thereby restoring proper circulation to the lungs.
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