Left ventricular noncompaction cardiomyopathy: updated review

Abstract

Abstract:The first case of noncompaction was described in 1932 after an autopsy performed on a newborn infant with aortic atresia/coronary-ventricular fistula. Isolated noncompaction cardiomyopathy was first described in 1984. A review on selected/relevant medical literature was conducted using Pubmed from 1984 to 2013 and the pathogenesis, clinical features, and management are discussed. Left ventricular noncompaction (LVNC) is a relatively rare congenital condition that results from arrest of the normal compaction process of the myocardium during fetal development. LVNC shows variability in its genetic pattern, pathophysiologic findings, and clinical presentations. The genetic heterogeneity, phenotypical overlap, and variety in clinical presentation raised the suspicion that LVNC might just be a morphological variant of other cardiomyopathies, but the American Heart Association classifies LVNC as a primary genetic cardiomyopathy. The familiar type is common and follows a X-linked, autosomal-dominant, or mitochondrial-inheritance pattern (in children). LVNC can occur in isolation or coexist with other cardiac and/or systemic anomalies. The clinical presentations are variable ranging from asymptomatic patients to patients who develop ventricular arrhythmias, thromboembolism, heart failure, and sudden cardiac death. Increased awareness over the last 25 years and improvements in technology have increased the identification of this illness and improved the clinical outcome and prognosis. LVNC is commonly diagnosed by echocardiography. Other useful diagnostic techniques for LVNC include cardiac magnetic resonance imaging, computerized tomography, and left ventriculography. Management is symptom based and patients with symptoms have a poorer prognosis. LVNC is a genetically heterogeneous disorder which can be associated with other anomalies. Making the correct diagnosis is important because of the possible associations and the need for long-term management and screening of living relatives.

Left Ventricular Noncompaction Combined With Epinephrine-Secreted Pheochromocytoma Inducing Heart Failure

“…Also, a two-layered structure between the noncompacted layer and the compacted layer is consistent with LVNC. 16) In this case, the echocardiographic imaging showed ventricular trabeculations and deep intertrabecular recesses in the apex and lateral wall, and blood flow running through the recesses and cardiac chambers. Otherwise, the thickened left ventricle was a double-walled structure.…”

“…In 1984, the first isolated noncompaction cardiomyopathy was described. 6) Left ventricular noncompaction (LVNC), also known as "spongy myocardium", is a rare abnormality of the left ventricular myocardium with two layers: a compacted layer and a noncompacted layer. 7) LVNC was first reported in children and has recently become increasingly diagnosed in adults, which is associated with high morbidity and mortality.…”

Clinical Applications of Late Gadolinium in Children

“…Histologically the findings are non-specific and include myocytes hypertrophy and fibrosis usually in the base and mid-ventricular regions. When LGE is seen (30% in adults), the typical pattern is in form of linear stripes and patches in the basal to midventricular regions in a non-vascular distribution (non-ischemic pattern) (14,20). In children, LGE has been showed in only 16% possibly due to a different repair mechanism for fibrosis in children that enables favourable reverse remodelling [4,7,9].…”

“…The diagnosis is made by the presence of exaggerated ventricular trabeculations, with a ratio of non-compacted to compacted myocardium >2.3 in diastole at echocardiography or CMR [14,20,21]. LGE is present in 25-40% of the patients including different patterns (subendocardial (70%), midwall (11%) and transmural (15%) enhancement) [4,21], and its presence is associated with a worse left ventricular ejection fraction and the development of ventricular arrhythmias.…”

Left Ventricular Noncompaction Cardiomyopathy Presenting with Heart Failure in a 35-Year-Old Man

“…3,13 Prophylactic anticoagulation is generally indicated in cases of LV systolic dysfunction, previous embolic events, cardiac thrombus, and atrial fibrillation. 14,15 Proper therapy for heart failure, involving angiotensin-converting enzyme inhibitors, β-blockers, and appropriate diuretics or dioxin, is essential. Cardiac resynchronization therapy can be helpful in drug-refractory cases.…”