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Electrocardiographic signs of hypertrophy in Fabry disease‐associated hypertrophic cardiomyopathy
Author(s) -
Kampmann C,
Wiethoff CM,
Martin C,
Wenzel A,
Kampmann R,
Whybra C,
Miebach E,
Beck M
Publication year - 2002
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.2002.tb03105.x
Subject(s) - fabry disease , medicine , hypertrophic cardiomyopathy , cardiology , left ventricular hypertrophy , cardiomyopathy , qrs complex , muscle hypertrophy , electrocardiography , heart disease , disease , heart failure , blood pressure
Aims : Fabry disease results from deficient activity of the lysosomal enzyme α‐galactosidase A. Progressive accumulation of the major substrates leads, in both men and women, to progressive hypertrophic cardiomyopathy. We aimed to evaluate the utility of different electrocardiographic (ECG) parameters for assessing the degree and severity of hypertrophic cardiomyopathy in patients with Fabry disease. Methods : A total of 166 ECGs of 94 hemi‐ and heterozygous patients with Fabry disease were analysed and compared with echocardiographic‐estimated left ventricular mass (LVM). Results : There was a significant ( p > 0.0001) correlation between QRS duration (R 2 = 0.59), 12‐lead amplitude/duration product (R 2 = 0.61), Sokolow‐Lyon voltage/duration product (R 2 = 0.52) and LVM. Analysis of receiver operating characteristics revealed that the 12‐lead amplitude/duration product had the highest sensitivity‐specificity relationship ( p > 0.01 compared with the Cornell index). Conclusion : In general, ECG signs of left ventricular hypertrophy correlated well with LVM as revealed by echocardiography. Of the parameters studied, the 12‐lead amplitude/duration product was the most successful at describing the severity of cardiac involvement in Fabry disease. These data suggest that ECG parameters have potential for use as a simple and cost‐effective means of screening for hypertrophic cardiomyopathy in patients with Fabry disease.