Standard ECG for differential diagnosis between Anderson-Fabry disease and hypertrophic cardiomyopathy
Jazyk angličtina Země Anglie, Velká Británie Médium print-electronic
Typ dokumentu časopisecké články, multicentrická studie
PubMed
33563631
DOI
10.1136/heartjnl-2020-318271
PII: heartjnl-2020-318271
Knihovny.cz E-zdroje
- Klíčová slova
- cardiomyopathy, electrocardiography, genetic diseases, hypertrophic, inborn, metabolic diseases,
- MeSH
- blokáda Tawarova raménka diagnóza MeSH
- diferenciální diagnóza MeSH
- elektrokardiografie MeSH
- Fabryho nemoc * diagnóza MeSH
- hypertrofická kardiomyopatie * diagnóza MeSH
- hypertrofie levé komory srdeční diagnóza etiologie MeSH
- lidé MeSH
- retrospektivní studie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
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.
Cardiomyopathy Unit Careggi University Hospital Firenze Toscana Italy
Department of Biomedical and Neuromotor Sciences University of Bologna Bologna Emilia Romagna Italy
Maria Cecilia Hospital GVM Care and Research Cotignola Ravenna Emilia Romagna Italy
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