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Long-Term Outcomes After Atrial Switch Operation for d-TGA
Quick Takes
- For most adults with d-loop transposition of the great arteries (d-TGA) after atrial switch, progress to end-stage heart failure or death is slow.
- Independent predictors of terminal heart failure events within 5 years were limited to history of prior ventricular arrhythmia, prior heart failure admission, complex anatomy, QRS duration >120 ms, and severe RV dysfunction and were used to develop a risk prediction tool for these events.
- Additional prospective studies are indicated to assess the utility and validity of this proposed risk assessment tool to identify patients with d-TGA and a systemic RV at high risk of developing terminal heart failure.
Study Questions:
What are the factors associated with survival in a large cohort of individuals with d-loop transposition of the great arteries (d-TGA) with a systemic right ventricle (RV) after an atrial switch operation?
Methods:
The investigators conducted a multicenter, retrospective cohort study and included adults with d-TGA and prior atrial switch surgery seen at a congenital heart center. Clinical data from initial and most recent visits were obtained. The composite primary outcome was death, transplantation, or mechanical circulatory support (MCS). The authors constructed a Kaplan-Meier survival curve with 95% confidence intervals to visualize survival probability after the initial visit. Multivariable Cox regression models with the primary outcome were fit including predictors with <20% missingness that were significant in univariable analysis (p < 0.05) and that were considered clinically relevant.
Results:
From 1,168 patients (38% female, age at first visit 29 ± 7.2 years) over median 9.2 years of follow-up, 91 (8.8% per 10 person-years) met the outcome (66 deaths, 19 transplants, 6 MCS). Patients experiencing sudden/arrhythmic death were younger than those dying of other causes (32.6 ± 6.4 vs. 42.4 ± 6.8 years, p < 0.001). There was a long duration between sentinel clinical events and end-stage heart failure. Age, atrial arrhythmia, pacemaker, biventricular enlargement, systolic dysfunction, and tricuspid regurgitation were all associated with the primary outcome. Independent 5-year predictors of primary outcome were prior ventricular arrhythmia, heart failure admission, complex anatomy, QRS duration >120 ms, and severe RV dysfunction by echocardiography.
Conclusions:
The authors reported that for most adults with d-TGA after atrial switch, progress to end-stage heart failure or death is slow.
Perspective:
This study reports that end-stage heart failure events (death, heart transplant, or MCS) among individuals with d-TGA with a systemic RV after an atrial switch operation were associated with age, prior atrial arrhythmia, pacemaker, and systemic ventricular dysfunction, among others. Furthermore, independent predictors of these events within 5 years were limited to history of prior ventricular arrhythmia, prior heart failure admission, complex anatomy, QRS duration >120 ms, and severe RV dysfunction and were used to develop a risk prediction tool for end-stage heart failure events. Additional prospective studies are indicated to assess the utility and validity of this proposed risk assessment tool to identify patients with d-TGA and a systemic RV at risk of developing terminal heart failure.
Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Prevention, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement, Acute Heart Failure, Heart Transplant, Interventions and Imaging, Interventions and Structural Heart Disease, Echocardiography/Ultrasound
Keywords: Arrhythmias, Cardiac, Arterial Switch Operation, Cardiac Surgical Procedures, Death, Sudden, Cardiac, Echocardiography, Heart Defects, Congenital, Heart Failure, Heart Transplantation, Pacemaker, Artificial, Risk Assessment, Secondary Prevention, Transposition of Great Vessels, Tricuspid Valve Insufficiency, Ventricular Dysfunction, Right
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