Abstract

GANAME, Javier; AYRES, Nancy A.  and  PIGNATELLI, Ricardo H.. Left Ventricular Noncompaction, a Recently Recognized Form of Cardiomyopathy. Insuf. card. [online]. 2006, vol.1, n.3, pp.119-124. ISSN 1852-3862.

Left ventricular noncompaction (LVNC) is a recently and increasingly recognized form of cardiomyopathy. It is characterized by the presence of an extensive trabeculated layer of myocardium and multiple deep intertrabecular recesses reinforcing the luminal aspect of the compacted part of the ventricular wall. It is probably secondary to an arrest of the normal process of compaction that occurs early in fetal life. LVNC seems to be a morphologic abnormality with genetic heterogeneity; both familial and isolated cases have been described. A few causal mutations have been discovered. Clinically, LVNC presents with: heart failure (usually severe), systemic embolism, arrhythmia or sudden death. Association of LVNC with other congenital heart and neuromuscular diseases has been reported. The diagnosis is generally made by the echocardiographic demonstration of a thick noncompacted layer with a maximal noncompacted to compacted ratio > 2; flow from the left ventricular (LV) cavity into the intertrabecular recesses; and areas of hypokinesia with depressed systolic and diastolic function. Also, magnetic resonance imaging appears promising to increase the diagnostic accuracy. Prognosis was initially thought to be very poor; but this belief has changed in the last years. There is no specific therapy for LVNC, so patients are treated with digoxin, inhibitors of angiotensin converting enzyme, diuretics and beta blockers. Anticoagulation is recommended after embolic episodes. Cardiac transplantation remains as the last option for cases refractory to medical therapy.

Keywords : Cardiomyopathy; Heart failure; Myocardium; Noncompaction; Echocardiography.

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