LAUSR

Background Modern advances in surgery and percutaneous interventions have resulted in new therapeutic possibilities for end-stage heart failure in congenital heart disease. We present a case of a patient with D-transposition of the great arteries undergoing multiple surgeries and percutaneous interventions for advanced heart failure. Description A 44-year-old man with D-transposition of the great arteries underwent a Blalock-Hanlon septectomy at age 9 days and a Mustard atrial switch procedure at age 3 years. He received an epicardial ventricular pacemaker at age 12 years for high grade atrioventricular (AV) block. He developed supraventricular tachycardia ten years ago, received electrophysiology ablation, and was found to have an inferior vena cava (IVC) baffle leak for which he underwent Amplatzer occluder device placement. Four years ago, he developed superior vena cava (SVC) and IVC baffle stenosis and underwent dilation and covered stent placements in the SVC and IVC baffles. His pacemaker was upgraded to a biventricular implantable cardioverter defibrillator (ICD) utilizing the epicardial ventricular lead and an endocardial lead through the covered stent in the SVC baffle for primary prevention of sudden cardiac death. Due to worsening heart failure despite inotropic support, the patient underwent systemic HeartWare ventricular assist device (VAD) implantation as a bridge to transplantation. Discussion The chest X-ray shows a HeartWare ventricular assist device attached to the systemic right ventricle. The biventricular ICD includes endocardial right-atrial lead, ICD lead in the non-systemic left-ventricle, and two epicardial ventricular leads. Multiple sternotomy wires, endotracheal tube and right internal jugular catheter are seen. The stented superior and inferior vena cava baffles and Amplatzer closure device for baffle leak project over the mediastinum. Conclusion Significant advances in surgery and percutaneous interventions allow for complex therapeutic options for patients with congenital heart disease and advanced heart failure.