THE DIAGNOSTIC YIELD OF BRUGADA SYNDROME IN VICTIMS OF ...
THE DIAGNOSTIC YIELD OF BRUGADA SYNDROME IN VICTIMS OF SUDDEN ARRHYTHMIC DEATH SYNDROME: AN UNDERESTIMATED ENTITY?
M. Papadakis1, N. Chandra1, H. Raju2, R. Bastiaenen2, A. O’Sullivan3, T. Fonseca4,
E. Sawyer4, N. van Niekerk4, S. Sharma1
1St George's University of London & University Hospital Lewisham, 2St George's University of London, 3King's College Hospital & University Hospital Lewisham, 4University Hospital Lewisham, London, UK
Background: In a significant proportion of sudden cardiac deaths (SCD) a cause cannot be identified and such cases are classified as sudden arrhythmic death syndrome (SADS). The recognition of such deaths is of utmost importance since studies relating to evaluation of 1st-degree relatives of victims of SADS indicate a high yield of hereditary ion-channel disorders. We report our UK experience from a large tertiary referral centre.
Methods: Between 2006 and 2009, we evaluated 1st-degree relatives of 135 victims of SCD who had undergone post-mortem evaluation. Ninety-five families, comprising of 298 relatives, where no definite cause of death was identified were included in the study. Relatives underwent cardiovascular evaluation with 12-lead ECG, echocardiogram, exercise testing, Holter monitor, Ajmaline provocation test and cardiac MRI, as appropriate. All ECGs were performed with leads V1 and V2 placed in both the 4th and the 2nd intercostal space.
Results: Following cardiovascular evaluation of 1st-degree relatives, 46 (48%) out of the 95 families received a diagnosis: 36, Brugada syndrome (BS); 7, long-QT syndrome; 2, arrhythmogenic right ventricural cardiomyopathy; 1, dilated cardiomyopathy. Sixty-nine (23%) relatives were diagnosed with a cardiovascular pathology, not previously identified.
Discussion: The diagnostic yield in this study is in keeping with existing studies in smaller number of families. In contrast to existing studies however, BS predominates accounting for nearly 80% of the conditions identified. Factors accounting for the predominance of BS include differences in the cohort characteristics, with 80% of our victims dying at rest, and the use of the higher intercostal leads.
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