Non-Compaction Cardiomyopathy. Risk Stratification of Sudden Death for Automatic Cardioverter Defibrillator Implantation

pp. 14-20

Abstract

Background

Non-compaction cardiomyopathy is a rare disease. Its natural history includes heart failure, thromboembolic events, arrhythmias and sudden death (SD). In the absence of data from randomized studies or records, the ACC / AHA / HRS 2008 guidelines recommend the automatic implantable cardioverter defibrillator (AICD) in all patients with non-compaction cardiomyopathy to reduce the risk of suden death.

Objective

Describe the outcomes of patients with non-compaction cardiomyopathy according to selected criteria for risk stratification of sudden death to decide the implantation of an AICD.

Material and Methods

A total of 80 patients were analyzed. The diagnosis was established by echocardiography and nuclear cardiac magnetic resonance imaging criteria. The criteria for implantation of an AICD as a secondary prevention included sudden death and sustained ventricular tachycardia (SVT); as primary prevention included left ventricular ejection fraction (LVEF) < 30% or ≥ 2 risk factors (family history of sudden death [FHSD], syncope and non-sustained VT).

Results

AICD group (n = 26) for secondary prevention (n = 3): 3 patients suffered sudden death (2 SVT). AICD group 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 VT + FHSD and 1 SVT in electrophysiological study. Follow up was a median of 16.61 months. Two patients underwent a heart transplant, 3 patients received appropriate shocks and 4 patients, inappropriate shocks. Group without AICD (n = 54): 4 patients had syncope and 4, nonsustained VT. Follow up was a median of 12.15 months. Two patients died due to heart failure and 3 underwent a heart transplant.

Conclusions

32.5% of patients with non-compaction cardiomyopathy received an AICD, 88.5% for primary prevention; 11.5% received appropriate shocks. There was no sudden death in patients without AICD, these patients died due to heart failure progression. This record suggests that patients with non-compaction cardiomyopathy could be stratified to select those at higher risk of sudden death and who could be benefited from an AICD implantation.