Results of Primary Repair Versus Shunt Palliation in Ductal Dependent Infants With Pulmonary Atresia and Ventricular Septal Defect

The Impact of 22q11.2 Deletion Syndrome on Surgical Repair Outcomes of Conotruncal Cardiac Anomalies

“…A prior study that examined patients who had undergone interrupted aortic arch repair at our institution showed that patients with 22q11DS and other genetic syndromes were more likely to have narrower left ventricular outflow tracts and, hence, were more likely to require left ventricular outflow tract enlargement or bypass procedure [14]. Another prior study that examined patients with pulmonary atresia and ventricular septal defect from our institution showed that patients with 22q11DS and other genetic syndromes were more likely to require unplanned reoperation, mainly to the pulmonary artery branches and right ventricular outflow tract [15,16].…”

Surgical Options for Pulmonary Atresia with Ventricular Septal Defect in Neonates and Young Infants

“…In patients with PA/VSD, initial post-natal palliation with staged repair is more frequently practiced than performing an early primary repair. However, high operative [2] and inter-stage mortality [3][4][5] rates following a palliative procedure may be a rationales for attempting an initial primary repair. Inter-stage mortality after systemic-to-pulmonary shunt is usually attributed to thrombotic occlusion of the graft, which could be more detrimental in patients with pulmonary atresia than in patients with pulmonary stenosis or additional collateral blood flow [12].…”

“…Initial palliation with staged repair enables neonates to defer the definitive operation until they can undergo repair with relatively lower risks of CPB-associated complications, and larger RV-PA conduit with prolonged durability can be used. However, high in-hospital [2] and inter-stage mortality [3][4][5] have been considered as caveats of the staged repair strategy. In this study, we sought to determine the impact of surgical strategies on overall survival and the incidence of post-repair re-interventions.…”

Staged Palliation of Cyanotic Obstructive Lesions With a Modified Right Ventricular Outflow Procedure

“…6 However, all shunts require postoperative antiaggregant therapy and have associated problems including ongoing hypoxemia and risk of occlusion, which can extend postoperative recovery periods, require reoperation, and be life threatening. 3,4,[7][8][9] In addition to the aforementioned complications, shunt physiology may be suboptimal. If oversized, shunts can contribute to diastolic runoff with compromised coronary and visceral blood flow, leading to extremes of myocardial ischemia and circulatory collapse and less dramatic but serious complications such as necrotizing enterocolitis and acute prerenal failure, 8 though these complications are more common and threatening in univentricular physiology.…”

“…3,4,[7][8][9] In addition to the aforementioned complications, shunt physiology may be suboptimal. If oversized, shunts can contribute to diastolic runoff with compromised coronary and visceral blood flow, leading to extremes of myocardial ischemia and circulatory collapse and less dramatic but serious complications such as necrotizing enterocolitis and acute prerenal failure, 8 though these complications are more common and threatening in univentricular physiology. 3,4 Furthermore, some evidence suggests that infants with very hypoplastic branch PAs who undergo primary complete repair in infancy may have suboptimal outcomes.…”