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Background-Although studies have suggested that "late-onset" hypertrophic cardiomyopathy (HCM) may be caused by sarcomeric protein gene mutations, the cause of HCM in the majority of patients is unknown. This study determined the prevalence of a potentially treatable cause of hypertrophy, Anderson-Fabry disease, in a HCM referral population. Methods and Results-Plasma ␣-galactosidase A (␣-Gal) was measured in 79 men with HCM who were diagnosed at Ն40 years of age (52.9Ϯ7.7 years; range, 40 -71 years) and in 74 men who were diagnosed at Ͻ40 years (25.9Ϯ9.2 years; range, 8 -39 years). Five patients (6.3%) with late-onset disease and 1 patient (1.4%) diagnosed at Ͻ40 years had low ␣-Gal activity. Of these 6 patients, 3 had angina, 4 were in New York Heart Association class 2, 5 had palpitations, and 2 had a history of syncope. Hypertrophy was concentric in 5 patients and asymmetric in 1 patient. One patient had left ventricular outflow tract obstruction. All patients with low ␣-Gal activity had ␣-Gal gene mutations. Conclusion-Anderson-Fabry disease should be considered in all cases of unexplained hypertrophy. Its recognition is important given the advent of specific replacement enzyme therapy.

The clinical spectrum of Fabry disease includes a "renal variant" phenotype in patients without classic symptoms who develop ESRD. Affected males undergoing hemodialysis or renal transplantation can be readily diagnosed by plasma alpha-Gal A assays. These patients and their family members may benefit from enzyme replacement therapy for the later, life-threatening cardiovascular and cerebrovascular complications of Fabry disease.

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The Doppler-derived index of combined systolic and diastolic myocardial performance correlates with global cardiac dysfunction and is a useful predictor of clinical outcome in patients with cardiac amyloidosis.

Repeated sauna treatment improves vascular endothelial function, resulting in an improvement in cardiac function and clinical symptoms.