Standard ECG for differential diagnosis between Anderson-Fabry disease and hypertrophic cardiomyopathy

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.

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Abstract P369: Left Ventricular Hypertrophy and Coronary Heart Disease Risk Reclassification in Blacks and Whites: The Atherosclerosis Risk in Communities (ARIC) Study

Circulation ◽

10.1161/circ.127.suppl_12.ap369 ◽

2013 ◽

Vol 127 (suppl_12) ◽

Author(s):

Tochi Okwuosa ◽

Elsayed Z Soliman ◽

Alvaro Alonso ◽

Kim A Williams ◽

Faye Lopez ◽

...

Keyword(s):

Blood Pressure ◽

Coronary Heart Disease ◽

Heart Disease ◽

Left Ventricular Hypertrophy ◽

Ventricular Hypertrophy ◽

Risk Model ◽

Left Ventricular ◽

C Statistic ◽

Framingham Risk ◽

Ecg Score

Introduction: Left ventricular hypertrophy (LVH) is more prevalent in blacks than whites, and is a major independent predictor of coronary heart disease (CHD)/CVD survival in blacks. We evaluated the ability of LVH to predict CHD outcomes beyond traditional cardiovascular risk factors in blacks, compared with whites from a large community-based cohort. Methods: Data were analyzed on 14,489 participants (mean age 54 +/- 5.7 years, 43.5% men, and 26% black) within the ARIC cohort, with baseline (1987-1989) electrocardiograms (ECG), followed through 2009. Risk estimates for incident CHD were assessed using the 10-year Framingham Risk Score (FRS). Model 1 was the Framingham base model, while model 2 included the base model plus LVH by any of 11 traditional ECG-LVH criteria (Table). Net reclassification improvement (NRI) was calculated, and the distribution of risk was compared using model 2 vs. model 1. Results: There were 690 (4.8%) 10-year and 1515 (10.5%) 20-year CHD events. LVH defined by any criteria was associated with CHD events in the entire cohort [HR (95% CI): 1.42 (1.20-1.7)]. LVH defined by the Framingham ECG score and LV strain criteria were the criteria most associated with CHD overall. LVH by Framingham ECG score was most associated with CHD in blacks [HR (95%CI): 2.53 (1.65-3.89)], while LV strain showed the strongest association with CHD in whites [1.73 (1.18-2.56)]. No statistically significant improvement in NRI or C-statistic was observed in model 2 [C-statistic (95% CI): 0.779 (0.763-0.794), NRI = 0.006 (p = 0.41)], compared with the base model [0.777 (0.762-0.792)]; and no racial interactions were observed. Findings were unchanged when the base model was replaced with the 10 and 20-year ARIC risk model (includes diabetes) for CHD. Conclusions: In this cohort of black and white men and women, LVH (defined by ECG) was significantly associated with CHD after adjustment for FRS; but did not significantly improve CHD risk prediction beyond the FRS. No significant black-white differences in risk prediction were observed. Table. Reclassification of Coronary Heart Disease by the addition of each Criterion for Left Ventricular Hypertrophy, based on a 10-year Framingham Risk Model * Base model factors in age, gender, current smoking, diabetes, systolic blood pressure, diastolic blood pressure, HDL cholesterol and total cholesterol as separate variables. * NRI categorized as <10%, 10-20% and >20%. Abbreviations: NRI = Net Reclassification Index, IDI = Integrated Discrimination Index, HR = Hazard Ratio, CI = Confidence Interval

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