Standard ECG for differential diagnosis between Anderson-Fabry disease and hypertrophic cardiomyopathy
- 9 February 2021
- journal article
- research article
- Published by BMJ
- Vol. 108 (1), 54-60
- https://doi.org/10.1136/heartjnl-2020-318271
Abstract
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.Keywords
This publication has 30 references indexed in Scilit:
- Usefulness of Electrocardiographic Patterns at Presentation to Predict Long-term Risk of Cardiac Death in Patients With Hypertrophic CardiomyopathyThe American Journal of Cardiology, 2016
- New Perspectives on the Prevalence of Hypertrophic CardiomyopathyJournal of the American College of Cardiology, 2015
- 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathyEuropean Heart Journal, 2014
- Electrocardiographic changes in early recognition of Fabry diseasePublished by BMJ ,2011
- Fabry disease: Guidelines for the evaluation and management of multi-organ system involvementGenetics in Medicine, 2006
- Presentation of multivariate data for clinical use: The Framingham Study risk score functionsStatistics in Medicine, 2004
- Internal validation of predictive models: Efficiency of some procedures for logistic regression analysisJournal of Clinical Epidemiology, 2001
- Usefulness of total 12-lead qrs voltage compared with other criteria for determining left ventricular hypertrophy in hypertrophic cardiomyopathy: analysis of 57 patients studied at necropsyThe American Journal of Medicine, 1989
- Transformation of the Independent VariablesTechnometrics, 1962
- Index for rating diagnostic testsCancer, 1950