title: The evolving role of the total artificial heart in the management of end-stage congenital heart disease and adolescents.
abstract: Advances in medical therapies have yielded improvement in morbidity and a decrease in mortality for patients with congenital heart disease, both surgically palliated and uncorrected. An unintended consequence is a cohort of adolescent and adult patients with heart failure who require alternative therapies. One intriguing option is placement of a total artificial heart (TAH) either as a bridge to transplant or as a destination therapy. Of the 1091 Jarvik-7 type TAH (Symbion, CardioWest and SynCardia) placed between 1985 and 2012, only 24 have been performed in patients with congenital heart disease, and a total of 51 were placed in patients younger than 21. At our institution, the SynCardia TAH was implanted in a 19-year-old patient with cardiac allograft failure because of chronic rejection and related multisystem organ failure including need for hemodialysis. Over the next year, she was nutritionally and physically rehabilitated, as were her end organs, allowing her to come off dialysis, achieve normal renal function and eventually be successfully transplanted. Given the continued growth of adolescent and adult congenital heart disease populations with end-stage heart failure, the TAH may offer therapeutic options where previously there were few. In addition, smaller devices such as the SynCardia 50/50 will open the door for applications in smaller children. The Freedom Driver offers the chance for patients to leave the hospital with a TAH, as does the AbioCor, which is a fully implantable TAH option. In this report, we review the history of the TAH and potential applications in adolescent patients and congenital heart disease.
authors.affiliation: From The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
authors.forename: Thomas D
authors.initials: TD
authors.lastname: Ryan
authors.forename: John L
authors.initials: JL
authors.lastname: Jefferies
authors.forename: Farhan
authors.initials: F
authors.lastname: Zafar
authors.forename: Angela
authors.initials: A
authors.lastname: Lorts
authors.forename: David L S
authors.initials: DL
authors.lastname: Morales
mesh_terms: D000293:Adolescent; D000328:Adult; D001145:Arrhythmias, Cardiac / Q000601:surgery; D009202:Cardiomyopathies / Q000601:surgery; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects; D006331:Heart Diseases / Q000151:congenital* / Q000601:surgery*; D006333:Heart Failure / Q000601:surgery*; D006338:Heart Neoplasms / Q000601:surgery; D016027:Heart Transplantation / Q000009:adverse effects; D006354:Heart, Artificial / Q000639:trends; D006353:Heart-Assist Devices / Q000639:trends; D006801:Humans; D008297:Male; D009205:Myocarditis / Q000601:surgery; D055815:Young Adult