Cryo-EM Structure of AAV2 Rep68 bound to integration site AAVS1: Insights into the mechanism of DNA melting.

The Rep68 protein from Adeno-Associated Virus (AAV) is a multifunctional SF3 helicase that performs most of the DNA transactions required for the viral life cycle. During AAV DNA replication, Rep68 assembles at the origin and catalyzes the DNA melting and nicking reactions during the hairpin rolling replication process to complete the second-strand synthesis of the AAV genome. Here, we report the Cryo-EM structures of Rep68 bound to double-stranded DNA (dsDNA) containing the sequence of the AAVS1 integration site in different nucleotide-bound states.

Perimount bovine pericardial valve to restore pulmonary valve competence late after right ventricular outflow tract repair.

Objective: No ideal option exists for restoring pulmonary valve competence late after repair of the congenitally abnormal right ventricular outflow tract (RVOT). This has driven a continued search for new alternatives. Texas Children's Hospital has recently used the Carpentier-Edwards Perimount RSR Pericardial Aortic Prosthesis (Edwards Lifesciences, Irvine, Calif, USA) for this indication and reports the initial experience.

Use of mechanical circulatory support in pediatric patients with acute cardiac graft rejection.

Patients suffering from acute cardiac graft rejection can die because of hemodynamic collapse while being treated with vigorous immunosuppressive therapies. There is little pediatric data on the use of mechanical circulatory support (MCS) in patients with acute cardiac graft rejection accompanied by hemodynamic instability. This report reviews our experience using MCS in patients with severe acute allograft rejection and cardiogenic shock.

Heterotaxy patients with total anomalous pulmonary venous return: improving surgical results.

Background: Survival after repair of total anomalous pulmonary venous return in patients with heterotaxy syndrome has consistently been reported in the literature to be far inferior to the results obtained in nonheterotaxy patients. The outcomes of heterotaxy patients at Texas Children's Hospital undergoing repair of total anomalous pulmonary venous return were examined and compared with those of nonheterotaxy patients.

Methods: Between July 7, 1995, and November 22, 2005, 122 consecutive patients underwent primary repair of total anomalous pulmonary venous return.

Interrupted aortic arch repair: aortic arch advancement without a patch minimizes arch reinterventions.

Background: Surgical repair of interrupted aortic arch (IAA) remains challenging and is associated with significant mortality and incidence of late arch obstruction, as recently reported by the Congenital Heart Surgeons' Society (CHSS). In particular, the CHSS reported that any technique other than direct anastomosis with patch augmentation is a risk factor for arch reintervention. The experience at Texas Children's Hospital with IAA repair using an aortic arch advancement technique without a patch was examined.