Purpose: To review findings in close relatives of victims of sudden unexplained death (SUD) with “undetermined cause of death” or newly identified cardiomyopathy.MethodMedical examiners advised families of victims of SUD to contact our cardiology clinic for evaluation.ResultsBetween March 2010 and October 2013, medical examiners identified 27 SUD victims probably resulting from cardiac arrhythmia (17 male, age 2 - 64, mean 38 years, 5 under 18 years) with unrevealing autopsy results (16), RV cardiomyopathy (8) and LV cardiomyopathy (3).Families of 15 deceased (10 male, age 2 - 52, mean 31 years, 5 under 18 years) sought clinical evaluation. Four deceased collapsed with exertion (3 unrevealing autopsy, 1 RV cardiomyopathy) and 11 died in sleep or at rest (6 unrevealing autopsy, 3 RV cardiomyopathy, 2 LV cardiomyopathy). Subsequently, 33 family members (8 males, age 0.2 - 67, mean 27 years, 11 under 18 years, 31 first degree relatives) were evaluated in the clinic with diagnostic tests appropriate for suspected familial disorder. One 64 year old woman was diagnosed with hypertrophic cardiomyopathy; autopsy in a 39 year old son showed hypertrophic cardiomyopathy. Two patients had borderline QT intervals of less than 500 ms and 2 had frequent premature ventricular beats (“undetermined cause of death” in the related deceased). One patient had mild segmental left ventricular dysfunction by MRI (RV cardiomyopathy in the deceased brother).ConclusionClinical evaluation of SUD victims’ relatives referred by medical examiners is effective in diagnosing arrhythmogenic conditions in other family members. Purpose: To review findings in close relatives of victims of sudden unexplained death (SUD) with “undetermined cause of death” or newly identified cardiomyopathy. MethodMedical examiners advised families of victims of SUD to contact our cardiology clinic for evaluation. Medical examiners advised families of victims of SUD to contact our cardiology clinic for evaluation. ResultsBetween March 2010 and October 2013, medical examiners identified 27 SUD victims probably resulting from cardiac arrhythmia (17 male, age 2 - 64, mean 38 years, 5 under 18 years) with unrevealing autopsy results (16), RV cardiomyopathy (8) and LV cardiomyopathy (3).Families of 15 deceased (10 male, age 2 - 52, mean 31 years, 5 under 18 years) sought clinical evaluation. Four deceased collapsed with exertion (3 unrevealing autopsy, 1 RV cardiomyopathy) and 11 died in sleep or at rest (6 unrevealing autopsy, 3 RV cardiomyopathy, 2 LV cardiomyopathy). Subsequently, 33 family members (8 males, age 0.2 - 67, mean 27 years, 11 under 18 years, 31 first degree relatives) were evaluated in the clinic with diagnostic tests appropriate for suspected familial disorder. One 64 year old woman was diagnosed with hypertrophic cardiomyopathy; autopsy in a 39 year old son showed hypertrophic cardiomyopathy. Two patients had borderline QT intervals of less than 500 ms and 2 had frequent premature ventricular beats (“undetermined cause of death” in the related deceased). One patient had mild segmental left ventricular dysfunction by MRI (RV cardiomyopathy in the deceased brother). Between March 2010 and October 2013, medical examiners identified 27 SUD victims probably resulting from cardiac arrhythmia (17 male, age 2 - 64, mean 38 years, 5 under 18 years) with unrevealing autopsy results (16), RV cardiomyopathy (8) and LV cardiomyopathy (3). Families of 15 deceased (10 male, age 2 - 52, mean 31 years, 5 under 18 years) sought clinical evaluation. Four deceased collapsed with exertion (3 unrevealing autopsy, 1 RV cardiomyopathy) and 11 died in sleep or at rest (6 unrevealing autopsy, 3 RV cardiomyopathy, 2 LV cardiomyopathy). Subsequently, 33 family members (8 males, age 0.2 - 67, mean 27 years, 11 under 18 years, 31 first degree relatives) were evaluated in the clinic with diagnostic tests appropriate for suspected familial disorder. One 64 year old woman was diagnosed with hypertrophic cardiomyopathy; autopsy in a 39 year old son showed hypertrophic cardiomyopathy. Two patients had borderline QT intervals of less than 500 ms and 2 had frequent premature ventricular beats (“undetermined cause of death” in the related deceased). One patient had mild segmental left ventricular dysfunction by MRI (RV cardiomyopathy in the deceased brother). ConclusionClinical evaluation of SUD victims’ relatives referred by medical examiners is effective in diagnosing arrhythmogenic conditions in other family members. Clinical evaluation of SUD victims’ relatives referred by medical examiners is effective in diagnosing arrhythmogenic conditions in other family members.
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Jan 4, 2022
Gustavo Jardim Volpe + 8
Open Access