title: Meta-Analysis of the Effectiveness of Heart Transplantation in Patients With a Failing Fontan.
abstract: The Fontan procedure is increasingly being used to palliate univentricular physiology. It is a complex anatomic and physiologic repair that can fail at any age, often leaving heart transplantation as the only remaining solution. A meta-analysis was performed to achieve the aim of systematically evaluating the existing evidence for survival after heart transplantation in patients who have undergone a Fontan palliation. MEDLINE, Embase, PubMed, and Web of Science were searched for original research studies. The primary outcome was mortality at 1 and 5 years after transplantation. Five hundred eighty-two records were screened, after the removal of duplicates, 12 retrospective observational studies were selected for inclusion in our meta-analysis. This encompassed a total of 351 Fontan patients undergoing heart transplantation. Mean age was 14 years (range 7 to 24 years) and 65% were men. One- and 5-year survival rates after heart transplantation were found to be 80.3% (95% CI 75.9% to 84.2%) and 71.2% (95% CI 66.3% to 75.7%), respectively. No significant association was found between age, gender, and pulmonary pressures and 1-year mortality. In conclusion, in the largest analysis to date, we found that heart transplantation in younger patients after Fontan procedure has an acceptable early and mid-term mortality. It is comparable to published mortality data of heart transplantation for other forms of congenital heart disease. Heart transplantation in the younger failing Fontan population appears to be a reasonable option when all other avenues have been exhausted and appropriate screening has taken place.
authors.affiliation: Division of Cardiology, St. Paul's Hospital, University of British Columbia, Vancouver, British Columbia, Canada.
authors.forename: Nazlee
authors.initials: N
authors.lastname: Tabarsi
authors.affiliation: Division of Cardiology, St. Paul's Hospital, University of British Columbia, Vancouver, British Columbia, Canada.
authors.forename: Meijiao
authors.initials: M
authors.lastname: Guan
authors.affiliation: Division of Cardiology, Great Ormond Street Hospital for Children, London, United Kingdom.
authors.forename: Jacob
authors.initials: J
authors.lastname: Simmonds
authors.affiliation: Division of Cardiology, St. Paul's Hospital, University of British Columbia, Vancouver, British Columbia, Canada.
authors.forename: Mustafa
authors.initials: M
authors.lastname: Toma
authors.affiliation: Division of Cardiology, St. Paul's Hospital, University of British Columbia, Vancouver, British Columbia, Canada.
authors.forename: Marla
authors.initials: M
authors.lastname: Kiess
authors.affiliation: Division of Cardiology, Great Ormond Street Hospital for Children, London, United Kingdom.
authors.forename: Victor
authors.initials: V
authors.lastname: Tsang
authors.affiliation: Division of Cardiology, Auckland City Hospital, Auckland, New Zealand.
authors.forename: Peter
authors.initials: P
authors.lastname: Ruygrok
authors.affiliation: Division of Cardiothoracic Surgery, Royal Children's Hospital, Melbourne, Australia.
authors.forename: Igor
authors.initials: I
authors.lastname: Konstantinov
authors.affiliation: Division of Cardiothoracic Surgery, Royal Children's Hospital, Melbourne, Australia.
authors.forename: William
authors.initials: W
authors.lastname: Shi
authors.affiliation: Division of Cardiology, St. Paul's Hospital, University of British Columbia, Vancouver, British Columbia, Canada. Electronic address: jasmine.grewal@vch.ca.
authors.forename: Jasmine
authors.initials: J
authors.lastname: Grewal
mesh_terms: D018729:Fontan Procedure / Q000009:adverse effects*; D006330:Heart Defects, Congenital / Q000601:surgery*; D016027:Heart Transplantation / Q000401:mortality; D006801:Humans; D010166:Palliative Care; D015996:Survival Rate