Progressive Systemic Ventricular Dysfunction in Congenitally Corrected TGA

Quick Takes

  • Patients with congenitally corrected transposition of the great arteries are at risk for progressive RV dysfunction and measured by RV longitudinal strain.
  • Risk factors for progressive RV dysfunction include LV systolic dysfunction, LV pacing, and systemic hypertension.

Study Questions:

What are the risk factors for and prognostic implications of progressive right ventricular (RV) systolic dysfunction in adults with congenitally corrected transposition of the great arteries (CCTGA)?


A retrospective review was performed at a single center. The primary mode of assessment of RV systolic function was RV longitudinal strain (RV-LS). The first echocardiogram was considered the baseline study and all subsequent annual echocardiograms performed within 5 years were analyzed.


A total of 186 patients (mean age 40 ± 12 years) were studied, with a median follow-up of 10.4 (4.2-16.1) years. During this period, 34 (18%) patients were hospitalized for heart failure, 3 (2%) underwent heart transplant, and 25 (13%) died of cardiovascular cause. The RV-LS at baseline was -17% ± 4%, and the annual decline in RV-LS was -4% (95% confidence interval, -6 to -2). Risk factors for progressive RV systolic dysfunction were left ventricular (LV) systolic dysfunction, LV pacing, and systemic hypertension. Progressive RV systolic dysfunction was associated with cardiovascular events independent of RV systolic function at baseline. In subgroup analysis assessing impact of therapies such as medical therapy, cardiac resynchronization, and tricuspid valve replacement, only tricuspid valve replacement performed prior to development of RV dysfunction was associated with improvement in RV systolic function.


The authors concluded that patients with CCTGA were at risk for progressive RV dysfunction, and that the risk factors for progressive RV dysfunction were LV pacing, systemic hypertension, and LV dysfunction.


CCTGA, characterized by atrioventricular discordance and ventriculoarterial discordance, is associated with progressive RV dysfunction and increasing risk of heart failure in adulthood. This study assessed systemic RV dysfunction using the echocardiographic parameter of RV-LS. Interestingly, while the baseline degree of tricuspid valve regurgitation was not associated with RV dysfunction, tricuspid valve replacement was associated with improved RV-LS. The finding that systemic hypertension is a risk for progressive RV dysfunction suggests a role for meticulous blood pressure managing in this patient population and a need for further study in this area.

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, 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, Hypertension

Keywords: Blood Pressure, Cardiac Resynchronization Therapy, Congenitally Corrected Transposition of the Great Arteries, Diagnostic Imaging, Echocardiography, Heart Defects, Congenital, Heart Failure, Heart Transplantation, Hypertension, Risk Factors, Transposition of Great Vessels, Tricuspid Valve Insufficiency

< Back to Listings