Cardiac Resynchronization for Corrected Transposition of the Great Arteries with Systemic Right Ventricle Failure after Tricuspid Valve Replacement and Ventricle Septal Defect Closure

A 32-year-old man developed systemic right ventricular (RV) heart failure after ventricular septal defect (VSD) closure and tricuspid valve replacement for corrected transposition of the great arteries with VSD and Ebstein anomaly. He subsequently experienced RV failure with wide QRS and atrial fibrillation (AF). Because corrective surgery for this condition seemed over risky, we decided to perform cardiac resynchronization therapy with implantation of an implantable cardioverter defibrillator (CRT-D). After CRT-D device implantation, the patient showed improved performance status in terms of New York Heart Association functional class, B-type brain natriuretic peptide levels, RV ejection fraction and cardiac electrical rhythm. CRT-D implantation is a useful approach for systemic RV failure with wide QRS duration showing right bundle branch block and AF. (J Arrhythmia 2010; 26: 267-271) [Introduction] Cardiac resynchronization therapy (CRT) is effective in selected patients with ischemic and dilated cardiomyopathy and symptomatic heart failure despite optimal medical therapy. However, indications for CRT have not been established in patients with adult congenital heart disease (ACHD), due to the heterogeneity of anatomy and physiology.