TY - JOUR
T1 - End-stage heart failure in congenitally corrected transposition of the great arteries
T2 - a multicentre study
AU - Van Dissel, Alexandra C.
AU - Opotowsky, Alexander R.
AU - Burchill, Luke J.
AU - Aboulhosn, Jamil
AU - Grewal, Jasmine
AU - Lubert, Adam M.
AU - Antonova, Petra
AU - Shah, Sangeeta
AU - Cotts, Timothy
AU - John, Anitha S.
AU - Kay, William Aaron
AU - Dezorzi, Christopher
AU - Magalski, Anthony
AU - Han, Frank
AU - Baker, David
AU - Kay, Joseph
AU - Yeung, Elizabeth
AU - Vonder Muhll, Isabelle
AU - Pylypchuk, Stephen
AU - Kuo, Marissa C.
AU - Nicolarsen, Jeremy
AU - Sarubbi, Berardo
AU - Fusco, Flavia
AU - Jameson, Susan M.
AU - Cramer, Jonathan
AU - Gupta, Tripti
AU - Gallego, Pastora
AU - O'donnell, Clare
AU - Hannah, Jane
AU - Dellborg, Mikael
AU - Kauling, Robert M.
AU - Ginde, Salil
AU - Krieger, Eric V.
AU - Rodriguez, Fred
AU - Dehghani, Payam
AU - Kutty, Shelby
AU - Wong, Joshua
AU - Wilson, William M.
AU - Rodriguez-Monserrate, Carla P.
AU - Roos-Hesselink, Jolien
AU - Celermajer, David S.
AU - Khairy, Paul
AU - Broberg, Craig S.
N1 - Publisher Copyright:
© 2023 The Author(s). Published by Oxford University Press on behalf of the European Society of Cardiology.
PY - 2023/9/7
Y1 - 2023/9/7
N2 - 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.
AB - 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.
KW - Adult congenital heart disease
KW - Congestive heart failure
KW - Heart transplantation
KW - Mechanical circulatory support
KW - Systemic right ventricle
KW - Transposition of the great arteries
UR - http://www.scopus.com/inward/record.url?scp=85170110511&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85170110511&partnerID=8YFLogxK
U2 - 10.1093/eurheartj/ehad511
DO - 10.1093/eurheartj/ehad511
M3 - Article
C2 - 37592821
AN - SCOPUS:85170110511
SN - 0195-668X
VL - 44
SP - 3278
EP - 3291
JO - European heart journal
JF - European heart journal
IS - 34
ER -