CARDIAC RESYNCHRONIZATION THERAPY IN CONGENITAL HEART DISEASE
Case presented by:
A 51-year-old woman with asymptomatic congenital heart disease (left ventricular ejection fraction [LVEF] 64%) underwent chemotherapy with doxorubicin (Adriamycin) for breast cancer. Four weeks after completing chemotherapy, she presented with atrial fibrillation (AF), a rapid ventricular response, and heart failure. She was treated with amiodarone, digoxin, beta-blockers, and warfarin. One week later, she developed complete heart block with a junctional escape rhythm at 45 bpm, and worsening heart failure. Systemic LVEF was now 25%. Cardiac computed tomography (CT) was obtained (Figure 66.1).
Question No. 1: The best course of action is:
A.Insert temporary pacemaker and stop amiodarone; await recovery of atrioventricular (AV) conduction.
B.Implant dual-chamber implantable cardioverter-defibrillator (ICD).
C.Implant transvenous cardiac resynchronization therapy-defibrillator (CRT-D).
D.Implant CRT-D with epicardial lead via thoracotomy.
Panel A
Figure 66.1. Cardiac CT images showing location and course of the coronary sinus (CS), and coronary arteries (CA). A: The right (RV) and left ventricles (LV) were transposed as were the aorta (Ao) and pulmonary arteries (PA). The systemic LV was dilated (LVEF 30%). B: The CS was small and followed the course of the right coronary artery (RCA). LA, left atrium; LAD, left anterior descending artery; LCx, left circumflex artery; RA, right atrium.
Discussion
Congenitally corrected transposition of the great vessels (CCTGV) accounts for less than 1% of congenital heart disease. It is usually associated with a ventricular septal defect or pulmonic stenosis, and survival depends upon the severity of the associated abnormalities. CCTGV without associated abnormalities is a very rare condition1 and patients can survive up to middle or even old age.2 Death usually occurs due to heart failure or complete heart block; the latter condition occurs in 10% of patients even without Adriamycin-induced cardiomyopathy.3 Thus, implantation of a temporary pacemaker and discontinuing amiodarone is unlikely to resolve the heart block, and recurrence of AF is likely in the absence of antiarrhythmic therapy.
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