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Neuromuscular implications in left ventricular hypertrabeculation/noncompaction.

Abstract
This review focuses on recent advances in the association between left ventricular hypertrabeculation/noncompaction (LVHT), a form of unclassified cardiomyopathy, and neuromuscular disorders (NMD). So far, LVHT has been found in single patients with dystrophinopathy, dystrobrevinopathy, laminopathy, zaspopathy, myotonic dystrophy, infantile glycogenosis type II (Pompe's disease), myoadenylate-deaminase deficiency, mitochondriopathy, Barth syndrome, Friedreich ataxia, and Charcot-Marie-Tooth disease. Most frequently LVHT is found in patients with Barth syndrome and mitochondrial disorders. The prevalence of LVHT in NMD patients is not known. On the contrary, NMD can be detected in up to four fifths of the patients with LVHT. Because LVHT is associated with an increased risk of rhythm abnormalities and heart failure, it is essential to detect LVHT as soon as possible. Because of adequate therapeutic options, all patients with NMD should undergo a comprehensive cardiological examination as soon as their neurological diagnosis is established. In reverse, all patients with LVHT should undergo a comprehensive neurological investigation following the detection of LVHT.
AuthorsJosef Finsterer, Claudia Stöllberger, Gerhard Blazek
JournalInternational journal of cardiology (Int J Cardiol) Vol. 110 Issue 3 Pg. 288-300 (Jun 28 2006) ISSN: 0167-5273 [Print] Netherlands
PMID16364474 (Publication Type: Journal Article, Review)
Topics
  • Heart Defects, Congenital (pathology)
  • Humans
  • Hypertrophy, Left Ventricular (complications, metabolism, therapy)
  • Mitochondria (metabolism)
  • Neuromuscular Diseases (complications, diagnosis, metabolism, therapy)
  • Prognosis
  • Thromboembolism (pathology)

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