PT  - JOURNAL ARTICLE
AU  - Vitale, Giovanni
AU  - Ditaranto, Raffaello
AU  - Graziani, Francesca
AU  - Tanini, Ilaria
AU  - Camporeale, Antonia
AU  - Lillo, Rosa
AU  - Rubino, Marta
AU  - Panaioli, Elena
AU  - Di Nicola, Federico
AU  - Ferrara, Valentina
AU  - Zanoni, Rossana
AU  - Caponetti, Angelo Giuseppe
AU  - Pasquale, Ferdinando
AU  - Graziosi, Maddalena
AU  - Berardini, Alessandra
AU  - Ziacchi, Matteo
AU  - Biffi, Mauro
AU  - Santostefano, Marisa
AU  - Liguori, Rocco
AU  - Taglieri, Nevio
AU  - Nardi, Elena
AU  - Linhart, Ales
AU  - Olivotto, Iacopo
AU  - Rapezzi, Claudio
AU  - Biagini, Elena
TI  - Standard ECG for differential diagnosis between Anderson-Fabry disease and hypertrophic cardiomyopathy
AID  - 10.1136/heartjnl-2020-318271
DP  - 2022 Jan 01
TA  - Heart
PG  - 54--60
VI  - 108
IP  - 1
4099  - http://heart.bmj.com/content/108/1/54.short
4100  - http://heart.bmj.com/content/108/1/54.full
SO  - Heart2022 Jan 01; 108
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.