End-stage heart failure in congenitally corrected transposition of the great arteries: a multicentre study

• Published in: European heart journal Vol. 44; no. 34; pp. 3278 - 3291
• Main Authors: van Dissel, Alexandra C, Opotowsky, Alexander R, Burchill, Luke J, Aboulhosn, Jamil, Grewal, Jasmine, Lubert, Adam M, Antonova, Petra, Shah, Sangeeta, Cotts, Timothy, John, Anitha S, Kay, William Aaron, DeZorzi, Christopher, Magalski, Anthony, Han, Frank, Baker, David, Kay, Joseph, Yeung, Elizabeth, Vonder Muhll, Isabelle, Pylypchuk, Stephen, Kuo, Marissa C, Nicolarsen, Jeremy, Sarubbi, Berardo, Fusco, Flavia, Jameson, Susan M, Cramer, Jonathan, Gupta, Tripti, Gallego, Pastora, O’Donnell, Clare, Hannah, Jane, Dellborg, Mikael, Kauling, Robert M, Ginde, Salil, Krieger, Eric V, Rodriguez, Fred, Dehghani, Payam, Kutty, Shelby, Wong, Joshua, Wilson, William M, Rodriguez-Monserrate, Carla P, Roos-Hesselink, Jolien, Celermajer, David S, Khairy, Paul, Broberg, Craig S
• Format: Journal Article
• Language: English
• Published: US Oxford University Press 07-09-2023
• Subjects: Adult; Adult congenital heart disease; adults; atrioventricular valve-replacement; Cardiac and Cardiovascular Systems; Cardiovascular System & Cardiology; Child; circulatory support; Clinical Research; Congenitally Corrected Transposition of the Great Arteries; Congestive heart failure; disease; Female; Heart Failure - complications; Heart transplantation; Humans; impact; Kardiologi; Male; Mechanical; Middle Aged; outcomes; Retrospective Studies; Systemic right ventricle; Transposition of Great Vessels - complications; Transposition of Great Vessels - surgery; Transposition of the great arteries; Tricuspid Valve Insufficiency - complications; ventricular dysfunction; Ventricular Dysfunction, Right - complications; Young Adult; Systemic right ventricle; Transposition of the great arteries; Congestive heart failure; Adult congenital heart disease; Mechanical circulatory support; Heart transplantation
• ISSN: 0195-668X; 1522-9645; 1522-9645
• DOI: 10.1093/eurheartj/ehad511

Abstract
Background and Aims
For patients with congenitally corrected transposition of the great arteries (ccTGA), factors associated with progression to end-stage congestive heart failure (CHF) remain largely unclear.
Methods
This multicentre, retrospective cohort study included adults with ccTGA seen at a congenital heart disease centre. Clinical data from initial and most recent visits were obtained. The composite primary outcome was mechanical circulatory support, heart transplantation, or death.
Results
From 558 patients (48% female, age at first visit 36 ± 14.2 years, median follow-up 8.7 years), the event rate of the primary outcome was 15.4 per 1000 person-years (11 mechanical circulatory support implantations, 12 transplantations, and 52 deaths). Patients experiencing the primary outcome were older and more likely to have a history of atrial arrhythmia. The primary outcome was highest in those with both moderate/severe right ventricular (RV) dysfunction and tricuspid regurgitation (n = 110, 31 events) and uncommon in those with mild/less RV dysfunction and tricuspid regurgitation (n = 181, 13 events, P < .001). Outcomes were not different based on anatomic complexity and history of tricuspid valve surgery or of subpulmonic obstruction. New CHF admission or ventricular arrhythmia was associated with the primary outcome. Individuals who underwent childhood surgery had more adverse outcomes than age- and sex-matched controls. Multivariable Cox regression analysis identified older age, prior CHF admission, and severe RV dysfunction as independent predictors for the primary outcome.
Conclusions
Patients with ccTGA have variable deterioration to end-stage heart failure or death over time, commonly between their fifth and sixth decades. Predictors include arrhythmic and CHF events and severe RV dysfunction but not anatomy or need for tricuspid valve surgery.
Structured Graphical Abstract
Structured Graphical Abstract
The magnitude and broad range of events in the entire ccTGA cohort. The scatter plot demonstrates that clinical events such as arrhythmia, pacemaker implantation, tricuspid valve surgery, need for heart transplant or MCS and occurrence of death were common and occurred throughout the lifespan of patients. ccTGA, congenitally corrected transposition of the great arteries; MCS, mechanical circulatory support; TVR, tricuspid valve repair/replacement.