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

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.

Keywords: Adult congenital heart disease; Congestive heart failure; Heart transplantation; Mechanical circulatory support; Systemic right ventricle; Transposition of the great arteries.

Structured Graphical Abstract

Figure 1

Patient sample by age decade at initial visit. Light blue blocks represent patients not reaching the primary outcome (mechanical circulatory support, transplantation, or death), and dark blue blocks represent those reaching the primary outcome in follow-up (n given for each block). Percentages of those reaching the primary outcome for each age decile are superimposed (horizontal line) with 95% confidence interval lines perpendicularly.

Figure 2

Breakdown of systemic right ventricular systolic function, semi-quantitatively assessed by echocardiography at initial visit and most recent echo at follow-up, comparing those who did (left) and did not (right) reach the primary outcome (mechanical circulatory support, transplantation, or death). Follow-up time (between echocardiograms) is given for both groups and was longer for those not reaching the primary outcome (P < .001). Clinical follow-up time was not different.

Figure 3

Occurrence of the primary outcome (mechanical circulatory support, transplantation, or death) by designation of right ventricular dysfunction and tricuspid valve regurgitation designations at the time of the initial echocardiogram. Excludes patients with prior tricuspid valve surgery.

Figure 4

Superimposed Kaplan–Meier curves with 95% confidence interval lines perpendicularly for subgroups of patients who have experienced various clinical events (pacemaker placement, tricuspid valve replacement, atrial arrhythmia, and ventricular arrhythmia) and progression to end-stage heart failure (% freedom from mechanical circulatory support, transplantation, or death) based on time of first occurrence of a specified clinical event as time zero. (A) Freedom from the primary outcome since first pacemaker implantation. (B) Freedom from the primary outcome since first tricuspid valve repair/replacement. (C) Freedom from the primary outcome since first ventricular arrhythmia. (D) Freedom from the primary outcome since first atrial arrhythmia.

Figure 5

Scatter plot of the entire cohort ordered by age at either last contact or primary endpoint (y-axis, oldest patient at bottom) plotted against age at time of event (x-axis) such that each row represents an individual patient. Red squares represent those who met the mechanical circulatory support, transplantation, or death primary endpoint, whereas blue circles represent those who did not. Additional circles show age at first atrial arrhythmia (yellow), ventricular arrhythmia (orange), pacemaker placement (green), or tricuspid valve repair/replacement (blue) as applicable for each patient. The scatter plot demonstrates a broad range of age at various events across the lifespan for both survivors and non-survivors.