Early Complete Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals

Surgical Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals With Absent Intrapericardial Pulmonary Arteries

“…Our current preference is to perform elective surgical repair for these patients between 3 and 6 months of age. The indications for early repair (which include unremitting heart failure and a ductus or hemitruncus to one lung with MAPCAs to the other) have been outlined in a previous publication [29].…”

Accuracy of Fetal Echocardiography in the Differential Diagnosis between Truncus Arteriosus and Pulmonary Atresia with Ventricular Septal Defect

“…Clinical manifestations in these children can vary widely depending on the presence of a patent DA and/or native pulmonary arteries as well as on the distribution of the pulmonary arteries and the MAPCAs to the pulmonary segments. Recent advances in the fields of interventional cardiology and cardiovascular surgery have increasingly allowed more patients to achieve a biventricular circulation [22], by different approaches [23,24,25] that are basically intended to complete the unifocalization of the supplying MAPCAs, with or without incorporation of the central pulmonary arteries, if present, and concomitant or delayed closure of the VSD. On the other hand, previous studies [2,26,27] have demonstrated that fetuses with a 22q11.2 microdeletion have more severely hypoplastic central pulmonary arteries and more branching anomalies, which determines a poorer postnatal outcome.…”

Tetralogy of Fallot: Everything you wanted to know but were afraid to ask

“…However, our approach is via median sternotomy . The objectives for the management of these patients at our center include: Unifocalization of blood supply to all pulmonary segments, including native pulmonary arteries and MAPCAs, to maximize incorporation of native vascular tissue and cross‐sectional area while preserving growth potential and minimizing total pulmonary resistance To achieve complete biventricular repair with closure of the VSD and placement of a valved RV to PA conduit with a right ventricular systolic pressure less than half systemic.…”

“…The preference is to perform complete unifocalization in infants greater than 3 months of age. However, surgery in the neonatal period (15%) is indicated in the following situations: Unilateral lung supply by a ductus arteriosus or hemi‐truncus (ascending aortic origin of a branch pulmonary artery) with the contralateral lung supplied by MAPCAs. Hypoplastic native pulmonary arteries with normal arborization (ie, all MAPCAs are dual‐supply), in which case a neonatal aortopulmonary window is performed, followed later in infancy, by complete repair. Profound overcirculation creating failure to thrive. Profound hypoxia due to low pulmonary blood flow. …”