Abstract

GALIZIO, Néstor O et al. Non-Compaction Cardiomyopathy: Risk Stratification of Sudden Death for Automatic Cardioverter Defibrillator Implantation. Rev. argent. cardiol. [online]. 2011, vol.79, n.1, pp.14-20. ISSN 1850-3748.

Background Non-compaction cardiomyopathy is a rare disease. The natural history of this condition includes heart failure, thromboembolic events, arrhythmias and sudden death (SD). In the absence of information from randomized studies or registries, the 2008 ACC/AHA/HRS guidelines recommended cardioverter defibrillator implantation in all patients with non-compaction cardiomyopathy to reduce the risk of sudden death. Objective To describe the outcomes of patients with non-compaction cardiomyopathy according to the selection criteria used for risk stratification of sudden death for automatic implantable cardioverter defibrillator therapy. Material and Methods A total of 80 patients were analyzed. The diagnosis was made using criteria from echocardiography and cardiac magnetic resonance imaging. The criteria used for implantation of an ICD were sudden death and sustained ventricular tachycardia (SVT) for secondary prevention, and left ventricular ejection fraction (LVEF) <30% or ≥2 risk factors (family history of sudden death [FHSD], syncope and non-sustained VT). Results Group ICD (n=26) for secondary prevention (n=3): 3 patients presented sudden death (2 SVT). Group ICD for primary prevention (n=23): 10 patients had LVEF <30%, 1 LVEF <30% + FHSD, 1 LVEF <30% + syncope, 5 LVEF <30% + nonsustained VT, 3 non-sustained VT + syncope, 2 non-sustained + FMSD and 1 VT in the electrophysiologic study. Median follow-up was 16.61 months. Two patients underwent heart transplantation, 3 received appropriate ICD shocks, while ICD shocks were inappropriate in 4 patients. Group without ICD (n=54): 4 patients presented syncope and 4 non-sustained VT. Median follow-up was 12.15 months. Two patients died due to heart failure and 3 underwent cardiac transplantation. Conclusions ICD was implanted in 32.5% of patients with non-compaction cardiomyopathy, 88.5% for primary prevention; appropriate shocks were received in 11.5% of patients. None of the patients who did not receive an ICD presented sudden death; these patients died due to heart failure progression. This registry suggests that patients with non-compaction cardiomyopathy might be stratified to select those at a higher risk of sudden death who might beneficiate from ICD therapy.

Keywords : Cardiomyopathies; Cardiac Ventricles; Risk; Cardiac Sudden Death; Prognosis.

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