• Perfusion · Jan 2014

    Case Reports

    The failing Fontan: what's NEXT...?

    • D B Sanders, S R Sowell, S S Park, C Derby, B C Willis, J E Lane, C N Pierce, F A Arabia, S G Pophal, and J J Nigro.
    • 1Scott and Laura Eller Congenital Heart Center, St. Joseph's Hospital and Medical Center, Phoenix, AZ, USA.
    • Perfusion. 2014 Jan 1; 29 (1): 89-93.

    AbstractThe Fontan procedure represents the final stage of the transition to single ventricle physiology. Conversion of very complex congenital heart anatomy, such as hypoplastic left heart syndrome, double-outlet right ventricle or double-inlet left ventricle, to a single ventricle has grown in popularity as morbidity and mortality have improved. As these patients grow, survivors are at risk for impaired ventricular dysfunction, plastic bronchitis, protein-losing enteropathy and late failure. Late failing Fontan patients represent a particularly vexing scenario for clinicians, as the only durable treatment option is cardiac transplantation. However, in the short-term, some of these patients require support beyond medical management, with mechanical circulatory support via extracorporeal life support or a ventricular assist device. We report the successful bridge of an adolescent female post-Fontan conversion with late severe cardiac failure. The patient was initially resuscitated with extracorporeal life support, transitioned to a single Berlin Heart EXCOR® ventricular assist device and, subsequently, underwent successful cardiac transplantation.

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