Right-Sided Subcutaneous Implantable Cardioverter Defibrillator System Implantation in a Patient with Complex Congenital Heart Disease and Dextrocardia: A Case Report and Literature Review

Abstract

Patients with complex congenital heart disease (CHD) and low left ventricular ejection fraction are at an increased risk of sudden cardiac death (SCD). Prevention of SCD by subcutaneous implantable cardioverter defibrillator (S-ICD) implantation may represent a valuable option in certain CHD patients. Patients with CHD and dextrocardia pose a challenge in S-ICD system implantation, and nonstandard device placement may be required. Furthermore, electrocardiogram (ECG) screening prior to S-ICD implantation in CHD patients has significant limitations. This case represents the placement of a S-ICD system on the right side of the chest in a 26-year-old male with severe biventricular failure and nonsustained ventricular tachycardia following multiple corrective surgeries of situs inversus totalis, double-outlet right ventricle with a ventricular septal defect, and pulmonary atresia. The use of S-ICDs in a CHD population and in particular CHD patients with dextrocardia and right-sided S-ICD implantation is briefly discussed.

Figure 1

Echocardiogram: (a) parasternal long axis view showing right and left ventricular dilatation, (b) M-mode with left ventricular measurements, and (c) parasternal short axis view at mitral valve level with biventricular enlargement.

Figure 2

12-lead electrocardiogram showing atrial fibrillation with a ventricular rate of 104 beats per minute and right bundle branch block.

Figure 3

Nonsustained ventricular tachycardia on telemetry with two different morphologies.

Figure 4

(a) Manual ECG screening test showing only lead I (alternate vector) in supine and sitting positions as acceptable vector at 10 mm/mV. (b) Postimplantation S-ECG sensing with gain setting 1X showing adequate sensing in the primary, secondary, and alternate vectors. The primary vector was automatically selected. S: subcutaneous.

Figure 5

Posteroanterior (a) and lateral (b) chest x-rays showing situs inversus totalis, pectus excavatum, sternal wires from previous cardiac surgeries, and the S-ICD device and electrode. Note the presence of cardiomegaly, small left pleural effusion, and basilar atelectasis. S-ICD: subcutaneous implantable cardioverter defibrillator.