ABSTRACT
The mitochondrial cytopathies or oxidative-phosphorylation diseases are a diverse group of disorders that result from the structural, biochemical, or genetic derangement of mitochondria. Because mitochondrial dysfunction can affect the most highly energy-dependent organs, cardiac involvement is frequent in these diseases. To identify the clinical features of Kearns-Sayre syndrome, an entity associated with this group of diseases, we evaluated cardiac structure and function in 5 patients with Kearns-Sayre syndrome and followed the clinical course of these patients for 5 years.
Subject(s)
Heart Diseases/etiology , Kearns-Sayre Syndrome/complications , Adult , Female , Humans , Kearns-Sayre Syndrome/diagnosis , MaleSubject(s)
Angiotensin-Converting Enzyme Inhibitors/therapeutic use , Aortic Valve Stenosis/drug therapy , Vasodilator Agents/therapeutic use , Angiotensin-Converting Enzyme Inhibitors/adverse effects , Angiotensin-Converting Enzyme Inhibitors/pharmacology , Aortic Valve Stenosis/complications , Humans , Hypotension/complications , Vasodilator Agents/adverse effectsABSTRACT
Las citopatías mitocondriales o enfermedades de las fosforilación oxidativa constituyen un diverso grupo de enfermedades cuya etiología subyace en trastornos estructurales, bioquímicos o genéticos de la mitocondria. Dado que una disfunción mitocondrial afecta a los órganos con mayores requerimientos energéticos, la afección cardíaca es frecuente en estos trastornos. Con el fin de determinar las características clínicas de la involucración cardíaca en el síndrome de Kearns-Sayre, entidad asociada a este grupo de enfermedades, hemos estudiado la estructura y la función cardíacas en una serie de 5 casos que, además, han sido seguidos durante 5 años
The mitochondrial cytopathies or oxidative-phosphorylation diseases are a diverse group of disorders that result from the structural, biochemical, or genetic derangement of mitochondria. Because mitochondrial dysfunction can affect the most highly energy-dependent organs, cardiac involvement is frequent in these diseases. To identify the clinical features of Kearns-Sayre syndrome, an entity associated with this group of diseases, we evaluated cardiac structure and function in 5 patients with Kearns-Sayre syndrome and followed the clinical course of these patients for 5 years
Subject(s)
Electrocardiography, Ambulatory , Electrophysiologic Techniques, Cardiac , Follow-Up Studies , Kearns-Sayre Syndrome , EchocardiographyABSTRACT
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