Congenital heart disease
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The Fontan procedure has undergone many modifications since first being performed on a patient with tricuspid valve atresia in 1968. It is now the procedure of choice for individuals born with single-ventricle physiology or for those in whom a biventricular repair is not feasible. Forty years of experience with the Fontan procedure have gradually revealed the shortfalls of such a circulatory arrangement. ⋯ The clinical significance of these findings, however, has remained poorly understood. As Fontan survivors have increased in age and number, we have begun to better recognize subclinical hepatic dysfunction and the contribution of liver disease to adverse outcomes in this population. The purpose of this review is to summarize the existing data pertaining to liver disease in the Fontan population and to identify some questions that have yet to be answered.
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Congenital heart disease · May 2011
Interhospital transport of children requiring extracorporeal membrane oxygenation support for cardiac dysfunction.
Many centers are able to emergently deploy extracorporeal membrane oxygenation (ECMO) as support in children with refractory hemodynamic instability, but may be limited in their ability to provide prolonged circulatory support or cardiac transplantation. Such patients may require interhospital transport while on ECMO (cardiac mobile [CM]-ECMO) for additional hemodynamic support or therapy. There are only three centers in the United States that routinely perform CM-ECMO. Our center has a 20-year experience in carrying out such transports. The purpose of this study was twofold: (1) to review our experience with pediatric cardiac patients undergoing CM-ECMO and (2) identify risk factors for a composite outcome (defined as either cardiac transplantation or death) among children undergoing CM-ECMO. ⋯ Air and ground CM-ECMO transport of pediatric patients with refractory myocardial dysfunction is safe and effective. In our study cohort, the need for post-CM-ECMO renal support was associated with the composite outcome of death/need for cardiac transplant.