Biventricular Pacing as Alternative Therapy for Dilated Cardiomyopathy Associated with Congenital Heart Disease

Abstract

Biventricular, alternative, and multisite pacing are currently being explored to improve cardiac function among patients with medically refractory, end-stage dilated cardiomyopathies. Although, due to inherent myocardial abnormalities, patients with repaired congenital heart defects may be at a greater risk than others to develop heart failure, often requiring cardiac transplantation. The efficacy of biventricular pacing among these patients is unknown. This report presents a patient with successfully repaired congenital heart disease in infancy who developed a symptomatic dilated cardiomyopathy at 22 years of age. Following biventricular pacing, systemic ventricular function showed a 14% improvement in ventricular dP/dt. One month later, subjective symptoms improved and cardiac ultrasound illustrated a 125% increase in fractional area of change. Exercise stress testing showed a 17% improvement in aerobic work capacity.

Cardiac resynchronization therapy: A novel adjunct to the treatment and prevention of systemic right ventricular failure

“…After a previous case report (16), this is the first study documenting a positive effect of CRT for either spontaneous or pacing-induced RV desynchronization in this population. The results correspond with those reported for patients with idiopathic or ischemic dilated cardiomyopathy and normal cardiac anatomy (17).…”

Cardiac resynchronization therapy in congenital heart disease

“…CRT for systemic right ventricular dysfunction was first reported in a 24 year-old man with L-TGA, ventricular septal defect, and pulmonary atresia [34]. At 4 years of age, he had a modified Rastelli operation with an intraventricular baffle directing right ventricular outflow to the aorta and a valved conduit connecting left ventricle to pulmonary artery.…”

Defibrillators and cardiac resynchronization therapy in congenital heart disease: evolving indications

“…Cardiac resynchronization therapy for systemic right ventricular dysfunction was first reported in a 24-year-old man with L-TGA and complete heart block [24]. Technical feasibility and hemodynamic benefits were more formally assessed in eight patients with systemic right ventricles [25].…”