Heart failure in patient with congenitally corrected transposition of the great arteries
Patients with congenitally corrected transposition of the great arteries (ccTGA) are born with a rare heart defect. This condition is characterized by abnormal connections between the ventricles and their outflow tracts: such that the aorta originates from the right ventricle (instead of the left ventricle in a structurally normal heart). Many patients face deterioration of heart function over the course of a lifetime, leading to severe heart failure and premature death. But not all patients develop these problems. Our aim was to identify which patients are more at risk to develop severe heart failure and why.
Through international collaboration with 29 centres worldwide, we gathered information from medical records on a large group of 558 patients with ccTGA. These patients were under regular follow-up by their cardiologist at an Adult Congenital Heart Disease centre. Most of them were in their early thirties to forties during the study period.
We found that individuals with ccTGA have variable disease course towards deterioration of their heart function. About 1 in every 7 patients was hospitalized for heart failure. And, 1 in every 24 patients underwent heart transplantation or mechanical device assistance to support the blood circulation / heart function. Nearly 1 in every 10 patients died, with heart failure as the leading cause of death. Although one might expect differently, we found that those with more complex cardiac anatomy (having substantial coexisting heart defects at birth) did not manifest heart failure sooner and/or more severe than those with a simpler cardiac anatomy. However, need for cardiac surgery during childhood was indeed associated with more problems during adulthood. Predictors for severe heart failure were older age, previous hospitalization for heart failure and having both a severely reduced heart function and severe tricuspid valve regurgitation (‘leakage’). Yet, repair or replacement of the damaged tricuspid valve through surgery did not necessarily lead to better outcomes afterwards. Though not all adults with ccTGA develop heart failure symptoms, if severe heart failure manifests, it usually does when people are in their forties to early fifties.
Van Dissel AC, Opotowsky AR, Burchill LJ, Aboulhosn J, Grewal J, Lubert AM, Antonova P, Shah S, Cotts T, John AS, Kay WA, DeZorzi C, Magalski A, Han F, Baker D, Kay J, Yeung E, Vonder Muhll I, Pylypchuk S, Kuo MC, Nicolarsen J, Sarubbi B, Fusco F, Jameson SM, Cramer J, Gupta T, Gallego P, O’Donnell C, Hannah J, Dellborg M, Kauling RM, Ginde S, Krieger EV, Rodriguez F, Dehghani P, Kutty S, Wong J, Wilson WM, Rodriguez-Monserrate CP, Roos-Hesselink J, Celermajer DS, Khairy P, Broberg CS. End-stage heart failure in congenitally corrected transposition of the great arteries: a multicentre study. Eur Heart J. 2023 Sep 7;44(34):3278-3291. doi: 10.1093/eurheartj/ehad511. PMID: 37592821; PMCID: PMC1048256
Prepared by Alexandra van Dissel and Simone Louise