The failing Fontan: what’s NEXT…?

• Published in: Perfusion Vol. 29; no. 1; pp. 89 - 93
• Main Authors: Sanders, DB, Sowell, SR, Park, SS, Derby, C, Willis, BC, Lane, JE, Pierce, CN, Arabia, FA, Pophal, SG, Nigro, JJ
• Format: Journal Article
• Language: English
• Published: London, England SAGE Publications 01.01.2014; Sage Publications Ltd
• Subjects: Adolescent; Cardiopulmonary Resuscitation - methods; Cardiopulmonary Resuscitation - standards; Extracorporeal Membrane Oxygenation - standards; Female; Heart Failure - therapy; Heart Transplantation; Heart-Assist Devices - standards; Humans; Treatment Outcome; ventricular assist device; extracorporeal life support; cardiac transplant; late failed Fontan circulation; single ventricle; bridge to transplant
• ISSN: 0267-6591; 1477-111X
• DOI: 10.1177/0267659113495913

The 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.