There was a need to identify risks for end-stage heart failure outcomes in individuals with d-loop transposition of the great arteries (d-TGA) who have a systemic right ventricle following an atrial switch procedure. For a study, researchers sought to identify variables linked to survival in a sizable sample of these people.

The multicenter, retrospective cohort research included adults with d-TGA and a history of atrial switch surgery who were examined at a congenital cardiac clinic. Clinical information was gathered from the first and most recent visits. Death, organ transplantation, or mechanical circulatory support (MCS) comprised the composite main outcome.

A total of 91 (8.8% per 10 person-years) of the 1,168 patients (38% female, age at first visit 29±7.2 years) with a median follow-up of 9.2 years reached the objective (66 deaths, 19 transplantations, 6 MCS). Patients who died suddenly or as a result of arrhythmia were younger than those who died from other causes (32.6±6.4 vs. 42.4±6.8 years; P< 0.001). The time between sentinel clinical events and end-stage heart failure was considerable. The primary result was correlated with age, atrial arrhythmia, pacemaker, biventricular enlargement, systolic dysfunction, and tricuspid regurgitation. Prior ventricular arrhythmia, heart failure hospitalization, complicated architecture, QRS length >120 ms, and significant right ventricle dysfunction as determined by echocardiography were independent 5-year predictors of the primary outcome.

Most persons with d-TGA following atrial switch had a gradual decline towards end-stage heart failure or death. To assist in determining who was most at risk, a simple prediction score for a negative result over a five-year period was developed.

Reference: jacc.org/doi/10.1016/j.jacc.2022.06.020