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 54
OP 60
DO 10.1136/heartjnl-2020-318271
VO 108
IS 1
A1 Vitale, Giovanni
A1 Ditaranto, Raffaello
A1 Graziani, Francesca
A1 Tanini, Ilaria
A1 Camporeale, Antonia
A1 Lillo, Rosa
A1 Rubino, Marta
A1 Panaioli, Elena
A1 Di Nicola, Federico
A1 Ferrara, Valentina
A1 Zanoni, Rossana
A1 Caponetti, Angelo Giuseppe
A1 Pasquale, Ferdinando
A1 Graziosi, Maddalena
A1 Berardini, Alessandra
A1 Ziacchi, Matteo
A1 Biffi, Mauro
A1 Santostefano, Marisa
A1 Liguori, Rocco
A1 Taglieri, Nevio
A1 Nardi, Elena
A1 Linhart, Ales
A1 Olivotto, Iacopo
A1 Rapezzi, Claudio
A1 Biagini, Elena
YR 2022
UL http://heart.bmj.com/content/108/1/54.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.All data relevant to the study are included in the article or uploaded as supplementary material. The authors from each center guarantee the integrity of data from their institution. All provided data included in the article cannot be traced back to individuals that participated in the study.