Ventricular Cardiomyopathy not compact. About his association with other extracardiac entities, alternative therapies and prognostic factors

Abstract

Hypertrabeculation / non-compaction of the left ventricle (LVHT) is associated, in most cases, a disease of the heart or skeletal hereditary chromosomal abnormalities or muscle. Depending on the study, more than two thirds of patients with LVHT also have a neuromuscular disease (NMD). The NMD LVHT associated with most frequently are the Barth syndrome, mitochondrial diseases, zaspopatía, and myotonic dystrophy. The NMD that are only present with LVHT are occasionally distrobrevinopatía, laminopathies, dystrophinopathies, miodelinato of deaminase deficiency, hereditary inclusion body myositis, and CMT1A neuropathy. A causal relationship between NMD and LVHT is likely, but the exact relationship and partnership pathomechanics remain elusive. The nearby pathogenic relationship is supported by the fact that the phenomenon of acquired LVHT occurs predominantly in the NMD. A consistent referral of patients with LVHT the neurologist, the subsequent referral of patients with NMD cardiologist, and family research can help clarify matters still unresolved concerning the pathogenesis, course and prognosis of LVHT.