Systemic ventricular assist device support in Fontan patients: A report by ACTION.

BACKGROUND: The size of the Fontan population with end-stage heart failure is growing. In this population, heart transplantation has been the only option. This study sought to investigate the efficacy of ventricular assist device (VAD) support in Fontan patients. METHODS: We conducted a retrospective study of Fontan patients in the Advanced Cardiac Therapies Improving Outcomes Network. We evaluated patient characteristics, and the clinical and physiologic outcomes after VAD implantation. RESULTS: We identified 45 Fontan patients implanted with VAD. The average age of patients was 10 years (interquartile range: 4.5-18) and 30% were female. The majority had a morphologic right ventricle (69%), moderate or greater ventricular dysfunction (83%), and moderate or greater atrioventricular valve regurgitation (65%). The majority of implants were as a bridge to transplantation (76%), and the majority of patients were Interagency Registry for Mechanically Assisted Circulatory Support Profile 2 (56%). The most commonly employed device was the Medtronic HeartWare HVAD (56%). A total of 13 patients were discharged on device support, and 67% of patients experienced adverse events, the most common of which were neurologic (25%). At 1 year after device implantation, the rate of transplantation was 69.5%, 9.2% of patients continued to be VAD supported, and 21.3% of patients had died. Hemodynamically, VAD was effective in decreasing both Fontan and ventricular end-diastolic pressures in some individuals. CONCLUSIONS: VAD is effective in supporting patients with end-stage Fontan failure awaiting heart transplantation. Future research should focus on identifying clinical and physiologic characteristics predictive of a favorable response to VAD support.

Left main bronchus obstruction after patent ductus arteriosus ligation: an unusual complication.

This report discusses a premature complex newborn with persistent pulmonary difficulties following the clipping of the PDA. Bronchoscopy was critical in revealing a metallic clip obstructing the bronchus. Thoracothomy revealed that the clip had been placed across the bronchus. This case illustrates the importance of bronchoscopy in the face of persistent unexplained pulmonary symptoms as well as having clear intraoperative exposure of the anatomy in this region to assure accurate placement of PDA metallic clips.

Pediatric mechanical circulatory support.

Over the past two decades clinicians and researchers have sought to bring mechanical circulatory support (MCS) to pediatric patients with heart failure. ECMO, IABPs, and VADs have all been used in infants and children as a bridge to myocardial recovery or as a bridge to transplant. However, until recently, a commitment by industry, government, and researchers towards the development of pediatric MCS has not been present, especially in the United States. Advancements in adult VAD design to smaller, quieter, and fully implantable pumps capable of complete outpatient support have sparked curiosity in the application of this technology to children. Also, the increasing success of palliating congenital heart disease is creating an ever-growing cohort of children and adolescents with heart failure. These changing demographics and technological advances have caused a refocus of attention. This is most clearly demonstrated by the international use of several established MCS pediatric and neonatal systems, by the FDAOs increasing proclivity to allow the use of international pediatric VADs in the USA, and by the recent National Heart, Lung, and Blood Institute funding of several institutions to develop pediatric VADs. This review describes the different aspects of pediatric MCS including indications, the features of the various VADs, and their current application in children worldwide.