Unmet needs in the cardiologic and neurologic work-up of left ventricular hypertrabeculation/noncompaction.

INTRODUCTION: Left-ventricular hypertrabeculation/noncompaction (LVHT) is a rare myocardial abnormality of the distal left ventricular myocardium, which may be complicated by functional abnormalities such as heart failure, arrhythmias, or thrombus formation.

AREA COVERED: This review discusses unsolved issues concerning LVHT, such as the lack of uniformly accepted diagnostic criteria, the unclear pathogenesis of LVHT, the relevance of clinical manifestations of LVHT as prognostic factors, the unclear association between LVHT and neuromuscular disorders, and the question which therapy LVHT patients should receive. Expert commentary: Despite controversy about these unsolved issues, there is consensus, however, that investigations need to be encouraged and undertaken which address the many open questions. This may help to prevent misdiagnosing or overdiagnosing LVHT, clarify etiology and pathomechanisms of LVHT, estimate the prognosis and outcome of these patients, identify the relevance of the association with certain neuromuscular disorders, chromosomal defects, and mutations, and to provide the optimal treatment and care.