Luke J Burchill1, Leah B Edwards2, Anne I Dipchand3, Joseph Stehlik4, Heather J Ross5. 1. Knight Cardiovascular Institute, Oregon Health and Science University, Portland, Oregon. 2. United Network for Organ Sharing, Richmond, Virginia. 3. Hospital for Sick Children, Toronto, Ontario, Canada. 4. School of Medicine, University of Utah, Salt Lake City, Utah. 5. Heart Failure and Transplantation Program, Toronto General Hospital, University Health Network, Toronto, Ontario, Canada. Electronic address: heather.ross@uhn.ca.
Abstract
BACKGROUND: Reduced early survival has been reported in adult congenital heart disease (ACHD) heart transplant (HTx) recipients, but little is known about late outcomes after HTx. The aim of this study was to examine survival; causes of death; and predictors of early (<1 year), mid-term (1 to 5 years) and late (>5 years) mortality in ACHD HTx recipients. METHODS: ACHD patients undergoing HTx between 1985 and 2010 were identified in the transplant registry of the International Society for Heart and Lung Transplantation (ISHLT). Survival was compared between ACHD and other adult HTx recipients ("controls") using the Kaplan-Meier method. Factors associated with survival beyond 1 year were assessed using multivariable proportional hazards regression analysis. RESULTS: Of 85,647 adults who underwent HTx, 1,851 (2.2%) were transplanted for ACHD. Early death secondary due to technical reasons was high among ACHD HTx recipients: 10% vs. 4% in controls (p < 0.0001). However, long-term survival of ACHD recipients who survived the early hazard phase was superior compared with controls (p < 0.0001). This was in part due to a lower infection (p < 0.0001) and malignancy-related (p < 0.01) mortality. Cardiac re-transplantation in ACHD HTx recipients was associated with a 2.75-fold increase in mortality. CONCLUSION: A "survival paradox" exists among ACHD recipients, whose high early mortality is balanced by better long-term survival in those who survive the early hazard phase after HTx. A high mortality risk after cardiac re-transplantation in this group of patients suggests that this treatment option should only be considered in carefully selected ACHD HTx recipients.
BACKGROUND: Reduced early survival has been reported in adult congenital heart disease (ACHD) heart transplant (HTx) recipients, but little is known about late outcomes after HTx. The aim of this study was to examine survival; causes of death; and predictors of early (<1 year), mid-term (1 to 5 years) and late (>5 years) mortality in ACHD HTx recipients. METHODS: ACHD patients undergoing HTx between 1985 and 2010 were identified in the transplant registry of the International Society for Heart and Lung Transplantation (ISHLT). Survival was compared between ACHD and other adult HTx recipients ("controls") using the Kaplan-Meier method. Factors associated with survival beyond 1 year were assessed using multivariable proportional hazards regression analysis. RESULTS: Of 85,647 adults who underwent HTx, 1,851 (2.2%) were transplanted for ACHD. Early death secondary due to technical reasons was high among ACHD HTx recipients: 10% vs. 4% in controls (p < 0.0001). However, long-term survival of ACHD recipients who survived the early hazard phase was superior compared with controls (p < 0.0001). This was in part due to a lower infection (p < 0.0001) and malignancy-related (p < 0.01) mortality. Cardiac re-transplantation in ACHD HTx recipients was associated with a 2.75-fold increase in mortality. CONCLUSION: A "survival paradox" exists among ACHD recipients, whose high early mortality is balanced by better long-term survival in those who survive the early hazard phase after HTx. A high mortality risk after cardiac re-transplantation in this group of patients suggests that this treatment option should only be considered in carefully selected ACHD HTx recipients.
Authors: Michelle Gurvitz; Kristin M Burns; Ralph Brindis; Craig S Broberg; Curt J Daniels; Stephanie M P N Fuller; Margaret A Honein; Paul Khairy; Karen S Kuehl; Michael J Landzberg; William T Mahle; Douglas L Mann; Ariane Marelli; Jane W Newburger; Gail D Pearson; Randall C Starling; Glenn R Tringali; Anne Marie Valente; Joseph C Wu; Robert M Califf Journal: J Am Coll Cardiol Date: 2016-04-26 Impact factor: 24.094
Authors: Werner Budts; Jolien Roos-Hesselink; Tanja Rädle-Hurst; Andreas Eicken; Theresa A McDonagh; Ekaterini Lambrinou; Maria G Crespo-Leiro; Fiona Walker; Alexandra A Frogoudaki Journal: Eur Heart J Date: 2016-01-18 Impact factor: 29.983
Authors: Christoph Sinning; Elvin Zengin; Gerhard-Paul Diller; Francesco Onorati; María-Angeles Castel; Thibault Petit; Yih-Sharng Chen; Mauro Lo Rito; Carmelina Chiarello; Romain Guillemain; Karine Nubret-Le Coniat; Christina Magnussen; Dorit Knappe; Peter Moritz Becher; Benedikt Schrage; Jacqueline M Smits; Andreas Metzner; Christoph Knosalla; Felix Schoenrath; Oliver Miera; Mi-Young Cho; Alexander Bernhardt; Jessica Weimann; Alina Goßling; Amedeo Terzi; Antonio Amodeo; Sara Alfieri; Emanuela Angeli; Luca Ragni; Carlo Pace Napoleone; Gino Gerosa; Nicola Pradegan; Inez Rodrigus; Julia Dumfarth; Michel de Pauw; Katrien François; Olivier Van Caenegem; Arnaut Ancion; Johan Van Cleemput; Davor Miličić; Ajay Moza; Peter Schenker; Josef Thul; Michael Steinmetz; Gregor Warnecke; Fabio Ius; Susanne Freyt; Murat Avsar; Tim Sandhaus; Assad Haneya; Sandra Eifert; Diyar Saeed; Michael Borger; Henryk Welp; László Ablonczy; Bastian Schmack; Arjang Ruhparwar; Shiho Naito; Xiaoqin Hua; Nina Fluschnik; Moritz Nies; Laura Keil; Juliana Senftinger; Djemail Ismaili; Shinwan Kany; Dora Csengeri; Massimo Cardillo; Alessandra Oliveti; Giuseppe Faggian; Richard Dorent; Carine Jasseron; Alicia Pérez Blanco; José Manuel Sobrino Márquez; Raquel López-Vilella; Ana García-Álvarez; María Luz Polo López; Alvaro Gonzalez Rocafort; Óscar González Fernández; Raquel Prieto-Arevalo; Eduardo Zatarain-Nicolás; Katrien Blanchart; Aude Boignard; Pascal Battistella; Soulef Guendouz; Lucile Houyel; Marylou Para; Erwan Flecher; Arnaud Gay; Éric Épailly; Camille Dambrin; Kaitlyn Lam; Cally Ho Ka-Lai; Yang Hyun Cho; Jin-Oh Choi; Jae-Joong Kim; Louise Coats; David Steven Crossland; Lisa Mumford; Samer Hakmi; Cumaraswamy Sivathasan; Larissa Fabritz; Stephan Schubert; Jan Gummert; Michael Hübler; Peter Jacksch; Andreas Zuckermann; Günther Laufer; Helmut Baumgartner; Alessandro Giamberti; Hermann Reichenspurner; Paulus Kirchhof Journal: ESC Heart Fail Date: 2021-09-12