title: Outcomes in adult congenital heart disease patients undergoing heart transplantation: A systematic review and meta-analysis.
abstract: Studies assessing mortality and morbidity in adult transplant recipients with congenital heart disease (CHD) are limited. We conducted a systematic review and meta-analysis comparing post-transplant outcomes in these 2 populations.
  
  
  
  After conducting an electronic database search, we selected studies evaluating mortality, cause-specific mortality, and risk of reoperation and dialysis in adult CHD vs non-CHD patients. We used random-effects models for the meta-analysis.
  
  
  
  Thirty-day mortality was significantly higher in CHD vs non-CHD patients (risk ratio [RR], 2.18; 95% confidence interval [CI], 1.62-2.93; I2= 41%). This was influenced by increased mortality in Fontan/Glenn patients compared with non-CHD patients (RR, 3.3; 95% CI, 1.89-5.77; I2= 0%). Mortality at 1 and 5 years was higher in the CHD population, although neither achieved statistical significance. Ten-year mortality was significantly lower in CHD patients (RR, 0.75; 95% CI, 0.60-0.95, I2= 42%). Deaths caused by malignancy, infection, rejection, and cardiac allograft vasculopathy were decreased in CHD patients, although only death from malignancy achieved significance. Death secondary to primary graft failure, stroke, and hemorrhage was significantly higher in CHD patients. Risk of reoperation and dialysis were not statistically different between the 2 groups.
  
  
  
  Although adult CHD patients have higher early mortality, post-transplantation long-term survival is superior to non-CHD recipients. The challenge is to identify the CHD patients who will benefit from transplantation vs those who are higher risk.
authors.affiliation: Heart Failure and Transplant Program, Toronto General Hospital, University Health Network, University of Toronto, Toronto, Ontario, Canada. Electronic address: barbara.doumouras@mail.utoronto.ca.
authors.forename: Barbara Stella
authors.initials: BS
authors.lastname: Doumouras
authors.affiliation: Heart Failure and Transplant Program, Toronto General Hospital, University Health Network, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Ana Carolina
authors.initials: AC
authors.lastname: Alba
authors.affiliation: Heart Failure and Transplant Program, Toronto General Hospital, University Health Network, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Farid
authors.initials: F
authors.lastname: Foroutan
authors.affiliation: Adult Congenital Heart Disease Program, Knight Cardiovascular Institute, Oregon Health Science University, Portland, Oregon, USA.
authors.forename: Luke James
authors.initials: LJ
authors.lastname: Burchill
authors.affiliation: Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Anne I
authors.initials: AI
authors.lastname: Dipchand
authors.affiliation: Heart Failure and Transplant Program, Toronto General Hospital, University Health Network, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Heather Joan
authors.initials: HJ
authors.lastname: Ross
mesh_terms: D000328:Adult; D014943:Global Health; D006084:Graft Rejection / Q000401:mortality*; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery*; D016027:Heart Transplantation / Q000401:mortality*; D006801:Humans; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends
keywords: Fontan/Glenn; adults; congenital heart disease; morbidity; mortality; outcomes; transplantation