Gene/Protein
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Enzyme
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Pivot Concepts:
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Target Concepts:
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Query: UMLS:C0018801 (
heart failure
)
72,216
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Simultaneous or temporarily staggered affection of both the skeletal as well as the cardiac muscle (cardiac involvement, CI) is a frequent finding in primary myopathies (MPs). CI leads to impulse generation defects, impulse conduction defects, thickened myocardium, left ventriculalr hypertrabeculation, dilatation of the cardiac cavities, secondary valve insufficiency, reduction of coronary vasodilative reserve, intracardial thrombus formation, and
heart failure
with systolic and diastolic dysfunction. CI has been found in Duchenne muscular dystrophy (MD), Becker MD, Emery-Dreifuss MD, facioscapulohumeral MD, sarcoglycanopathies, myotubular congenital MD, myotonic dystrophies type 1 and 2, proximal myotonic myopathy, myoadenylate deaminase deficiency, glycogenosis type II, III, IV, VII and IX, carnitine deficiency, mitochondriopathy, desmin MP, nemaline MP, central core disease, multicore MP, congenital fiber-type disproportion MP, Barth syndrome,
McLeod syndrome
and Bethlem MP. Patients with any of the above-mentioned myopathies should be cardiologically investigated as soon as their diagnosis is established, since sufficient cardiac therapy improves CI in MPs and since management of these patients is influenced by the degree of CI.
...
PMID:Cardiac involvement in primary myopathies. 1111 Nov 38
Myopathies are frequently not confined to the skeletal muscles but also involve other organs or tissues. One of the most frequently affected organ in addition to the skeletal muscle is the heart (cardiac involvement, CI). CI manifests as impulse generation or conduction defects, focal or diffuse myocardial thickening, dilation of the cardiac cavities, relaxation abnormality, hypertrophic, dilated, restrictive cardiomyopathy, apical form of hypertrophic cardiomyopathy, noncompaction, Takotsubo phenomenon, secondary valve insufficiency, intra-cardiac thrombus formation, or
heart failure
with systolic or diastolic dysfunction. CI occurs in dystrophinopathies, Emery-Dreifuss muscular dystrophy, facioscapulohumeral muscular dystrophy, limb girdle muscular dystrophies, laminopathies, congenital muscular dystrophies, myotonic dystrophies, congenital myopathies, metabolic myopathies, desminopathies, myofibrillar myopathy, Barth syndrome,
McLeod syndrome
, Senger's syndrome, and Bethlem myopathy. Patients with myopathy should be cardiologically investigated as soon as their neurological diagnosis is established, since supportive cardiac therapy is available, which markedly influences prognosis and outcome of CI in these patients.
...
PMID:Primary myopathies and the heart. 1827 31