Metabolic heart failure

Stanley H. Korman and Andre Keren

in Oxford Textbook of Heart Failure

Published on behalf of Oxford University Press

ISBN: 9780199577729
Published online July 2011 | e-ISBN: 9780199697809 | DOI:

Series: Oxford Textbooks

Metabolic heart failure

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Inherited metabolic disorders are an important cause of heart failure(HF), particularly because many of them are amenable to treatment.It is therefore important that metabolic disorders arerecognized and accurately diagnosed. The heart is dependent onnormal metabolic function to provide a continuous source ofenergy at rest and even more so during exertion or stress. Manydisorders of metabolism therefore affect cardiac function and mayhave devastating consequences).Cardiac failure in metabolic disorders is most commonly a consequenceof cardiomyopathy. Hypertrophic cardiomyopathy istypical of the glycogen and lysosomal storage diseases as well asthe disorders of mitochondrial energy production. Dilated cardiomyopathyis a feature of some of the organic acidurias, disordersof carnitine metabolism, and some mitochondrial disorders. Oftenthe distinction is unclear, with a transition from hypertrophic todilated cardiomyopathy as the disease progresses.Additional mechanisms leading to cardiac failure in metabolicdisorders include valvular disease, arrhythmias and conductiondisturbances, coronary artery disease, and pulmonaryhypertension.

Chapter.  9460 words.  Illustrated.

Subjects: Cardiovascular Medicine

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