title: The failing Fontan: what's NEXT...?
abstract: 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.
authors.affiliation: 1Scott and Laura Eller Congenital Heart Center, St. Joseph's Hospital and Medical Center, Phoenix, AZ, USA.
authors.forename: D B
authors.initials: DB
authors.lastname: Sanders
authors.forename: S R
authors.initials: SR
authors.lastname: Sowell
authors.forename: S S
authors.initials: SS
authors.lastname: Park
authors.forename: C
authors.initials: C
authors.lastname: Derby
authors.forename: B C
authors.initials: BC
authors.lastname: Willis
authors.forename: J E
authors.initials: JE
authors.lastname: Lane
authors.forename: C N
authors.initials: CN
authors.lastname: Pierce
authors.forename: F A
authors.initials: FA
authors.lastname: Arabia
authors.forename: S G
authors.initials: SG
authors.lastname: Pophal
authors.forename: J J
authors.initials: JJ
authors.lastname: Nigro
mesh_terms: D000293:Adolescent; D016887:Cardiopulmonary Resuscitation / Q000379:methods* / Q000592:standards; D015199:Extracorporeal Membrane Oxygenation / Q000592:standards; D005260:Female; D006333:Heart Failure / Q000628:therapy*; D016027:Heart Transplantation; D006353:Heart-Assist Devices / Q000592:standards; D006801:Humans; D016896:Treatment Outcome
keywords: bridge to transplant; cardiac transplant; extracorporeal life support; late failed Fontan circulation; single ventricle; ventricular assist device