Unclassified Cardiomyopathies

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  • Unclassified cardiomyopathies (CMPs) include left ventricular hypertrabeculation or noncompaction (LVHT). Left ventricular non-compaction cardiomyopathy (LVNC) is characterised by abnormal trabeculation, most commonly at the apex, and is often associated with ventricular hypertrophy, dilatation or impairment of function. The molecular mechanisms of LVNC are not yet fully understood but it is inherited in 30–50 % of patients.

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    Unclassified cardiomyopathies (CMPs) include left ventricular hypertrabeculation or noncompaction (LVHT). Left ventricular non-compaction cardiomyopathy (LVNC) is characterised by abnormal trabeculation, most commonly at the apex, and is often associated with ventricular hypertrophy, dilatation or impairment of function. The molecular mechanisms of LVNC are not yet fully understood but it is inherited in 30–50 % of patients.

    LVHT has been described in association with dystrophinopathies, myotonic dystrophies, zaspopathies, laminopathies, dystrobrevinopathies, oculopharyngeal muscular dystrophy, tropomyosin-1 mutations, multiminicore disease, Danon disease, mitochondrial disorders, myoadenylate deaminase deficiency, Pompe's disease, glycogen storage disease-IV, fatty acid oxidation disorder, Barth syndrome, ryanodine receptor mutation, inclusion body myopathy, dystrophic epidermolysis bullosa, Charcot-Marie-Tooth neuropathy, hereditary cobolamine deficiency, beta-thalassemia, poliomyelitis, and Friedreich ataxia.

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