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Standard ECG for differential diagnosis between Anderson-Fabry disease and hypertrophic cardiomyopathy
- Publication Year :
- 2022
-
Abstract
- ObjectivesTo evaluate the role of the ECG in the differential diagnosis between Anderson-Fabry disease (AFD) and hypertrophic cardiomyopathy (HCM).MethodsIn 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.ResultsShort 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.ConclusionsStandard 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.
- Subjects :
- medicine.medical_specialty
electrocardiography
Bundle-Branch Block
Cardiomyopathy
hypertrophic
030204 cardiovascular system & hematology
Left ventricular hypertrophy
metabolic diseases
NO
Diagnosis, Differential
03 medical and health sciences
0302 clinical medicine
genetic diseases
genetic disease
Internal medicine
Diagnosis
medicine
Humans
030212 general & internal medicine
cardiovascular diseases
PR interval
Retrospective Studies
ST depression
medicine.diagnostic_test
business.industry
Hypertrophic cardiomyopathy
Hypertrophy
Cardiomyopathy, Hypertrophic
Right bundle branch block
medicine.disease
Left Ventricular
Differential
Settore MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE
Cardiology
inborn
Fabry Disease
cardiomyopathy
Hypertrophy, Left Ventricular
medicine.symptom
Differential diagnosis
Cardiology and Cardiovascular Medicine
business
Electrocardiography
Subjects
Details
- Language :
- English
- Database :
- OpenAIRE
- Accession number :
- edsair.doi.dedup.....9d2299095b9fdb4b04e1a70527ff1068