Abstract
BACKGROUND: Dextrocardia is a congenital anomaly in which the apex of the heart is abnormally located on the right side of the chest. Situs solitus describes viscera that are in the normal position, with the stomach on the left side. In these patients, implantation of transvenous implantable cardioverter-defibrillator (ICD) can be limited by anatomical abnormalities commonly associated with this condition. CASE SUMMARY: We present the case of a young female patient with absent right atrioventricular connection, morphologically left systemic ventricle, muscular restrictive ventricular septal defect, and dextrocardia with situs solitus who was indicated for secondary prophylactic ICD implantation after resuscitation for polymorphic ventricular tachycardia. Due to a bilateral bidirectional Glenn anastomosis, transvenous access via the vena cava superior to the right ventricle could not be achieved. For this reason, we successfully implanted a subcutaneous ICD (S-ICD) with an individually optimized right parasternal electrode position. Potential complications of epimyocardial implantation via re-thoracotomy could thus be circumvented. DISCUSSION: In patients with complex congenital heart disease, the S-ICD is an effective method of preventing sudden cardiac death. Our case report demonstrates the feasibility of left S-ICD implantation even in the presence of dextrocardia with situs solitus.