Key result
Fabry disease is identified in ~4% of men referred for hypertrophic cardiomyopathy.
Why the study?
Cardiac abnormalities may be the only clinical manifestation of Fabry disease, but their prevalence and characteristics in referred patients were not well defined.
What is the prevalence of Fabry disease among male patients referred for hypertrophic cardiomyopathy?
Cross-Sectional (n=153)
No
What is the prevalence of Fabry disease among male patients referred for hypertrophic cardiomyopathy?
Fabry disease is present in 4-6% of male patients referred for hypertrophic cardiomyopathy and should be considered in the differential diagnosis of unexplained cardiac disease.
Answers are grounded in this paper and cite their sources.
Supports Fabry screening in unexplained male HCM; leaves open need for prospective validation before practice change.
UNLABELLED: Cardiac abnormalities are common in patients with Fabry disease, and may be the only clinical manifestation of the disease in some patients. At St George's Hospital Medical School, a national referral centre for hypertrophic cardiomyopathy in the UK, a study of 153 consecutively referred male patients revealed that 4% had Fabry disease. This increased to 6% in patients over 40 years of age. All these patients had electrocardiographic (ECG) abnormalities, most of which were consistent with those found in patients with classic Fabry disease: left ventricular hypertrophy, repolarization abnormalities and a prolonged QRS complex. A study of heterozygote females with Fabry disease also showed ECG and echocardiographic abnormalities in most patients. CONCLUSIONS: Fabry disease should be considered in the differential diagnosis of otherwise unexplained cardiac disease.
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Sachdev et al. (2002) conducted a cross-sectional in Hypertrophic cardiomyopathy / Fabry disease (n=153). Fabry disease was evaluated on Prevalence of Fabry disease. Among 153 male patients referred for hypertrophic cardiomyopathy, 4% were found to have Fabry disease, all of whom exhibited electrocardiographic abnormalities.
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