Supreet P Marathe1, Jacob Y Cao2, David Celermajer2, Julian Ayer3, Gary F Sholler3, Yves d'Udekem4, David S Winlaw5. 1. Queensland Children's Hospital, Brisbane, Australia; Faculty of Medicine, University of Queensland, Brisbane, Australia. 2. Faculty of Health and Medicine, Sydney Medical School, University of Sydney, Sydney, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia. 3. Faculty of Health and Medicine, Sydney Medical School, University of Sydney, Sydney, Australia; Heart Centre for Children, The Children's Hospital at Westmead, Sydney, Australia. 4. Murdoch Children's Research Institute, Melbourne, Australia; Department of Pediatrics, Faculty of Medicine, University of Melbourne, Melbourne, Australia; Royal Children's Hospital, Melbourne, Australia. 5. Faculty of Health and Medicine, Sydney Medical School, University of Sydney, Sydney, Australia; Heart Centre for Children, The Children's Hospital at Westmead, Sydney, Australia. Electronic address: david.winlaw@sydney.edu.au.
Abstract
BACKGROUND: Heterotaxy is considered a risk factor for adverse events at all stages in the pre-Fontan pathway, and Fontan outcomes are expected to be worse in patients with heterotaxy. The aim of this study was to review existing literature reporting outcomes of the Fontan operation systematically in patients with heterotaxy. METHODS: A systematic review and meta-analysis was performed to identify and synthesize early mortality and medium- and long-term survival in heterotaxy patients after the Fontan procedure. Subsequent outcome analyses were stratified by study period era, cohort size, and proportion of right versus left atrial isomerism to explore predictors of outcome. RESULTS: A total of 21 studies were included for analysis, which were composed of 848 post-Fontan heterotaxy patients. Early mortality varied between 1% and 30% with a weighted event rate of 14% (95% confidence interval [CI], 10%-19%). Survival at 1, 5, and 10 years was 86% (95% CI, 79%-91%), 80% (95% CI, 71%-87%), and 74% (95% CI, 59%-85%), respectively. Stratification by study period highlighted that studies with a median study period year of 1995 or later had similar early mortality and 1- and 5-year survival, but superior 10-year survival (P = .02) compared with earlier studies. Stratification by cohort size and right versus left atrial isomerism did not reveal subgroup differences. CONCLUSIONS: Compared with existing literature, in patients with heterotaxy, early mortality after Fontan is higher than for the overall Fontan population. Long-term survival is comparable to the overall Fontan cohort. When heterotaxy patients are successfully transitioned to Fontan, subsequent survival is acceptable and predictable. Long-term follow-up is lacking.
BACKGROUND: Heterotaxy is considered a risk factor for adverse events at all stages in the pre-Fontan pathway, and Fontan outcomes are expected to be worse in patients with heterotaxy. The aim of this study was to review existing literature reporting outcomes of the Fontan operation systematically in patients with heterotaxy. METHODS: A systematic review and meta-analysis was performed to identify and synthesize early mortality and medium- and long-term survival in heterotaxy patients after the Fontan procedure. Subsequent outcome analyses were stratified by study period era, cohort size, and proportion of right versus left atrial isomerism to explore predictors of outcome. RESULTS: A total of 21 studies were included for analysis, which were composed of 848 post-Fontan heterotaxy patients. Early mortality varied between 1% and 30% with a weighted event rate of 14% (95% confidence interval [CI], 10%-19%). Survival at 1, 5, and 10 years was 86% (95% CI, 79%-91%), 80% (95% CI, 71%-87%), and 74% (95% CI, 59%-85%), respectively. Stratification by study period highlighted that studies with a median study period year of 1995 or later had similar early mortality and 1- and 5-year survival, but superior 10-year survival (P = .02) compared with earlier studies. Stratification by cohort size and right versus left atrial isomerism did not reveal subgroup differences. CONCLUSIONS: Compared with existing literature, in patients with heterotaxy, early mortality after Fontan is higher than for the overall Fontan population. Long-term survival is comparable to the overall Fontan cohort. When heterotaxy patients are successfully transitioned to Fontan, subsequent survival is acceptable and predictable. Long-term follow-up is lacking.
Authors: Elena Hadjicosta; Rodney Franklin; Anna Seale; Oliver Stumper; Victor Tsang; David R Anderson; Christina Pagel; Sonya Crowe; Ferran Espuny Pujol; Deborah Ridout; Kate L Brown Journal: Heart Date: 2022-06-10 Impact factor: 7.365
Authors: Elisabeth Seidl-Mlczoch; Gregor Kasprian; Erwin Kitzmueller; Daniel Zimpfer; Irene Steiner; Victoria Jowett; Marlene Stuempflen; Alice Wielandner; Barbara Ulm; Ina Michel-Behnke Journal: Front Pediatr Date: 2022-07-14 Impact factor: 3.569