Clinical features of isolated left ventricular noncompaction in children

Prognosis of left ventricular hypertrabeculation/noncompaction is dependent on cardiac and neuromuscular comorbidity

“…Additionally, LVHT is associated with neuromuscular disorders (NMD) in the majority of the cases [1,2]. The data about long-term prognosis of LVHT are controversial and derive from case reports or relatively small series [2][3][4][5][6][7][8][9][10]. Some authors documented an increased rate of mortality whereas other found LVHT to have a good prognosis.…”

Isolated left ventricular noncompaction in association with rheumatic mitral stenosis

“…The common presentation of IVNC is left ventricular systolic dysfunction, [5] but the index case had normal ejection fraction on both echocardiographic and angiographic examination. Since echocardiography is the diagnostic technique of choice, missed diagnoses may be due to non-optimal imaging of the lateral and apical myocardium, and/or insufficient disease awareness by echocardiographers [5,6]. The noncompaction area may be localized to left ventricular apex only [5,6] or involved both the ventricles; [7] however there was isolated noncompaction of whole of left ventricle in index case.…”

“…Since echocardiography is the diagnostic technique of choice, missed diagnoses may be due to non-optimal imaging of the lateral and apical myocardium, and/or insufficient disease awareness by echocardiographers [5,6]. The noncompaction area may be localized to left ventricular apex only [5,6] or involved both the ventricles; [7] however there was isolated noncompaction of whole of left ventricle in index case. The spongiform or honeycomb appearance of left ventricle inner wall on angiogram as present in the index case is rarely being described in the literature [7,8].…”

Isolated left ventricular non-compaction: A larger part of submerged iceberg with criteria for diagnosis to the limits of standard

“…Stolberger et al proposed the definition of hypertrabeculation for the patients that presented a morphological aspect, also without considering the spongious/compacted ratio [1][2][3][4][5][6][7][8][9][10].…”

“…All that is indirectly valuable through literature because Oechslin et al, in 2000, reported a mortality incidence of 35% [9]; Alehan, in 2004, reported a mortality incidence of about 22% while [10] Aras et al reported a mortality incidence of 15% [11]. This variability is probably linked to the different selection of evaluated casistic, in which the relationship between symptomatic and asymptomatic patients, equivalent to the relationship between compensated/non-compensated patients, is different in each study.…”