An atypical variant of Fabry's disease in men with left ventricular hypertrophy

Article Abstract:

Fabry's disease may be the cause of otherwise unexplained enlargement of the left ventricle in men. Fabry's disease is a sex-linked inherited enzymatic defect. It normally causes symptoms in many organ systems, but cases have been reported in which only heart symptoms were found. A group of 230 men at a Japanese hospital had enlargement of the left ventricle. Blood levels of the potentially affected enzyme were measured and compared with samples from healthy volunteers. Seven of the men had enzyme levels less than 14% that of controls and were diagnosed with Fabry's disease; six came from a group of 58 patients with otherwise unexplained disease. Five of these patients had a heart muscle biopsy. When examined under the microscope, the muscle cells showed damage consonant with Fabry's disease. Only two patients had an abnormal gene for the enzyme, but four other patients had abnormally low levels of messenger RNA for this enzyme, suggesting a genetic defect elsewhere.

Causes of, Physiological aspects, Fabry's disease

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Prediction of heart failure - an art aided by technology

Article Abstract:

Heart failure is a complex disease that requires acute clinical skills to diagnose and it is unlikely that advances in technology will improve the diagnosis. In a 1997 study, researchers found that an increase in heart size measured by echocardiography could predict which patients would develop heart failure. An increase in heart size is believed to be a compensatory mechanism to overcome the death of heart cells. However, there are many other signs of heart failure and a large heart is not always one of them. In addition, echocardiography may not be a suitable device for screening large groups of people.

Editorial, Measurement, Echocardiography

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Left ventricular assist device and drug therapy for the reversal of heart failure

Article Abstract:

Fifteen patients with severe heart failure underwent implantation of left ventricular assist devices followed by a specific pharmacologic regimen. It was found that sustained reversal of severe heart failure secondary to nonischemic cardiomyopathy could be achieved in selected patients with the use of a left ventricular assist device and a specific pharmacologic regimen.

Care and treatment, Clinical report

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