RT-242
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Right bundle branch block, right
precordial ST segment elevation, and sudden death in the young
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Domenico Corrado, Cristina Basso,
Gianfranco Buja, Andrea Nava, Gaetano Thiene.
Department of Cardiology and Pathology, University of Padua, Italy
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This study was supported by the Juvenile
Sudden Death Research Project of the Veneto Region, Venice, and by the National Council
for Research, Target Project FATMA, Rome, Italy.
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Abstract
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Sudden death (SD) due to ventricular fibrillation may
occur in patients with the ECG pattern of right bundle branch block (RBBB), and persistent
right precordial ST segment elevation. The underlying structural heart disease is still
unknown. Among 262 consecutive cases of SD in young people (< 35 years) which
have been prospectively collected from 1979, 202 had pathological evidence of an
"arrhythmic" cardiac arrest. Twelve lead ECG recordings, available in 82
patients, were analysed and correlated to the underlying heart disease. RBBB, right
precordial ST segment elevation, or both were recorded in 21 SD victims (17 males, 4
females, aged 10 to 35 years, mean 24.6 years). Thirteen patients had complained syncope
and 10 had experienced ventricular arrhythmias. Persistent right precordial ST segment
elevation was documented in 10 patients, all with pathological evidence of arrhythmogenic
right ventricular cardiomyopathy (ARVC). Isolated RBBB was documented in 7 patients: 3
with postoperative congenital heart disease, 2 with paroxysmal AV block due to
sclerocalcific interruption of His-Purkinje system, and in 2 with ARVC. Both RBBB and
persistent right precordial ST segment elevation were recorded in 3 patients with ARVC and
fibrosis of right bundle branch, and in one with familial "RV conduction and
myocardial disease", namely fibrotic interruption of right bundle branch and adipous
myocardial dystrophy of RV wall, accounting for both "septal" and
"parietal" RV conduction defects. In conclusion, in our series of young SD
victims, RBBB and persistent right precordial ST segment elevation reflected an underlying
RV myocardial and/or specialized conduction system disease at risk of
"arrhythmic" SD.
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Key Words
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Brugada, Martini-Nava syndromes
sudden death, young patients, right bundle branch block, right precordial ST segment
elevation, ventricular fibrillation, syncope, arrhythmogenic right ventricular
cardiomyopathy, conduction system disease, OA
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