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Prevention of sudden death in cardiomyopathies

Session presentations
Myocardial Disease

This excellent session addressed the crucial issue of risk stratification and indication for ICD in Cardiomyopathies.

Hypertrophic cardiomyopathy (HCM)
Dr Perry Elliott (UK) underlines the limitations of commonly used risk factors for sudden cardiac death (SCD)(syncope, degree of hypertrophy, non sustained VT, exercise abnormal blood pressure, family history of SCD) as well as the high rate of complications of ICD in the patients. He reports on a new statistical model for risk stratification, based on the evaluation of 1643 HCM UK patients and a validation population of 456 Italian patients. The prediction of SCD was improved as compared to conventional evaluation. This could represent an important advance in the primary prevention of SCD in HCM.

Dilated cardiomyopathy (DCM)
Dr Ygal Pinto (Netherlands) indicates that the risk of SCD in DCM should be evaluated according to the precise underlying etiology. Familial forms of DCM represent more than 30% of cases and mutations of lamin A/C gene (LMNA) are associated with a high risk of SCD and probably an early indication for ICD. Recently a European registry coordinated by Dr Pinto reported follow-up data on 269 LMNA mutation carriers and identified 4 risk factors for malignant ventricular arrhythmia (2 of which suggesting ICD implantation).

Arrythmogenic right ventricular cardiomyopathy (ARVC)
Dr Thomas Wichter (Germany) reviewed risk factors for SCD in ARVC patients. Highest risk is related to patients with aborted SCD, hemodynamically unstable VT or syncope (rhythmic cause) and ICD is recommended in such patients. Intermediate risk is related to hemodynamically stable VT, nsVT, right or left ventricle systolic dysfunction and drugs may be sufficient as first line therapeutics. Low risk is characterized by the absence of the above criteria and patients can be treated by betablockers or with surveillance only. 

Left ventricular non compaction (LVNC)
Dr Gilbert Habib (France) said that the prognosis of LVNC was initially considered as poor but was probably overestimated as subsequent studies show various outcomes. The largest cohort of LVNC patients with prospective follow-up was recently reported from the French registry. Out of 105 patients with LVNC and 2.3 years FU, 7% developed severe ventricular arrhythmia, SCD rate was 1% and total death was 11%. Future prospective studies are required to determine potential specific indications for ICD and current indications for ICD should follow those related to other non-ischemic cardiomyopathies.





Prevention of sudden death in cardiomyopathies

The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.