RT Journal Article
SR Electronic
T1 Standard ECG for differential diagnosis between Anderson-Fabry disease and hypertrophic cardiomyopathy
JF Heart
JO Heart
FD BMJ Publishing Group Ltd and British Cardiovascular Society
SP heartjnl-2020-318271
DO 10.1136/heartjnl-2020-318271
A1 Giovanni Vitale
A1 Raffaello Ditaranto
A1 Francesca Graziani
A1 Ilaria Tanini
A1 Antonia Camporeale
A1 Rosa Lillo
A1 Marta Rubino
A1 Elena Panaioli
A1 Federico Di Nicola
A1 Valentina Ferrara
A1 Rossana Zanoni
A1 Angelo Giuseppe Caponetti
A1 Ferdinando Pasquale
A1 Maddalena Graziosi
A1 Alessandra Berardini
A1 Matteo Ziacchi
A1 Mauro Biffi
A1 Marisa Santostefano
A1 Rocco Liguori
A1 Nevio Taglieri
A1 Elena Nardi
A1 Ales Linhart
A1 Iacopo Olivotto
A1 Claudio Rapezzi
A1 Elena Biagini
YR 2021
UL http://heart.bmj.com/content/early/2021/02/09/heartjnl-2020-318271.abstract
AB Objectives To evaluate the role of the ECG in the differential diagnosis between Anderson-Fabry disease (AFD) and hypertrophic cardiomyopathy (HCM).Methods In this multicentre retrospective study, 111 AFD patients with left ventricular hypertrophy were compared with 111 patients with HCM, matched for sex, age and maximal wall thickness by propensity score. Independent ECG predictors of AFD were identified by multivariate analysis, and a multiparametric ECG score-based algorithm for differential diagnosis was developed.Results Short PR interval, prolonged QRS duration, right bundle branch block (RBBB), R in augmented vector left (aVL) ≥1.1 mV and inferior ST depression independently predicted AFD diagnosis. A point-by-point ECG score was then derived with the following diagnostic performances: c-statistic 0.80 (95% CI 0.74 to 0.86) for discrimination, the Hosmel-Lemeshow χ2 6.14 (p=0.189) for calibration, sensitivity 69%, specificity 84%, positive predictive value 82% and negative predictive value 72%. After bootstrap resampling, the mean optimism was 0.025, and the internal validated c-statistic for the score was 0.78.Conclusions Standard ECG can help to differentiate AFD from HCM while investigating unexplained left ventricular hypertrophy. Short PR interval, prolonged QRS duration, RBBB, R in aVL ≥1.1 mV and inferior ST depression independently predicted AFD. Their systematic evaluation and the integration in a multiparametric ECG score can support AFD diagnosis.