In Eisenmenger syndrome, treatment with bosentan significantly improved walking distance, pulmonary blood flow, and PVR. Adding sildenafil to bosentan did not significantly improve walking distance but did increase saturation at rest. http://www.ClinicalTrial.gov: NCT00303004.

A previously healthy male infant was resuscitated after spontaneous ventricular fibrillation at 9 weeks of age. Echocardiography revealed three tumors in the left ventricle not amenable to complete resection. Despite treatment with antiarrhythmic agents the ventricular arrhythmias continued. When the child was 4 months old and weighed 7 kg an ICD system was implanted using epicardial sense-pacing leads and a superior vena caval lead as a subcutaneous defibrillator coil placed posterior on the left thorax. Shocks were delivered between the subcutaneous coil lead and the intraabdominally placed ICD can. This ICD array system has not been reported previously.

We report on a 10-year-old boy with medically refractory pulmonary arterial hypertension (PAH) and end-stage right heart failure after closure of a ventricular septal defect. The boy was a candidate for lung transplantation (LTX), but an alternative option was to create an Eisenmenger physiology with right-to-left shunting. The shunt could be created either as an intracardiac or as an extracardiac shunt. We decided to create a Potts shunt, a direct anastomosis between the left pulmonary artery and the descending aorta. The Potts shunt functioned as a right-to-left shunt, thus reducing the afterload on the right ventricle. The boy's clinical condition improved markedly, so he was discharged two weeks after the procedure. The ultimate therapeutic option for medically refractory PAH is LTX or heart-lung transplantation, but because of the short life span after LTX, time was bought by postponing the time of transplantation.

No abstract

Congenital aortocaval fistula is a very rare anomaly. Clinically, it resembles conditions that cause left-to-right shunt of blood. We report a case of such anomaly in combination with a secundum atrial septal defect in a 13-month-old girl who presented with failure to thrive and exertional respiratory symptoms. The aortocaval fistula was occluded percutaneously using an Amplatzer® Duct Occluder.

An infant with truncus arteriosus and severe dysfunction of the truncal valve including both stenosis and insufficiency successfully underwent primary repair. This included the insertion of two separate valved homograft conduits. Early outcome has been excellent and the patient is doing well after 6 months with only echocardiographic evidence of mild aortic valve regurgitation. Double-homograft repair is a realistic option in cases of truncus arteriosus with severe malformation of the truncal valve.