Case report of an S-ICD implantation for secondary prevention in a patient with complex congenital heart disease, dextrocardia, and situs solitus

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 (AV) 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.

Therapy of Patients with Cardiac Malposition

“…In such patients, subcutaneous ICDs (S-ICDs) (Emblem A219; Boston Scientific, Marlborough, MA, USA) may be implanted. The reported experience with this form of therapy is encouraging [ 113 , 114 , 115 , 116 , 117 ].…”

S-ICD Implantation "Tips and Tricks"

“…If screening is negative, alternative lead location (right parasternal) may be considered [ 11 , 12 , 13 , 14 , 15 ]. In specific situations (congenital heart disease, dextrocardia) different positions of the system (e.g., reversed right-to-left) have been described [ 16 , 17 , 18 , 19 ], but if such a case is planned, the ECG screening should incorporate fluoroscopy to ensure the correct relation of S-ICD system components to the cardiac silhouette and location of the heart within the chest in relation to anatomical landmarks [ 20 ]. On the other hand, the ECG screening may be dynamic, and repeated recordings are likely to give contradictory results [ 21 ].…”