Clinical features of Noncompaction Cardiomyopathy

Noncompaction cardiomyopathy (NCCM) is recognized as a separate disease entity since the first report in 1984 of a rare case with persistent myocardial sinusoids and a series of 8 pediatric and adolescent patients in 1990 with increased trabeculation of the left ventricular endocardium. Given the cumulative evidence since then, NCCM is considered as a primary, predominantly genetic disorder of the myocardium. The classic clinical presentation includes severe heart failure, malignant ventricular arrhythmias, thrombo-embolic events and sudden cardiac death. But the complete spectrum comprises also asymptomatic to minor cardiac complaints like atypical chest pain and nonspecific palpitations. The noncompacted myocardium is characterized by an excessively prominent trabecular meshwork and deep intertrabecular recesses, as seen early in human embryogenesis. Therefore, the primary patho-physiological hypothesis is that incomplete compaction of the loose myocardial meshwork during gestation results in a noncompacted, heart, prone to heart failure, arrhythmias and intracardiac thrombus formation. The diagnostic criteria proposed by Jenni et al. include abnormally thickened ventricular walls with a two-layered structure, consisting of thickened, noncompacted (NC) endocardial myocardium and a thin compacted (C) epicardial myocardium (maximal end-systolic ratio NC/C 2 at parasternal short axis view). The diagnosis is established by imaging of the ventricular walls and cavities, classically by two-dimensional transthoracic echocardiography with color Doppler flow. The abnormal structures of NCCM could be identified as deep intertrabecular recesses with color Doppler flow as well as regional hypokinesia. Other imaging modalities like left ventricular (LV) angiography, computed tomography, contrast echocardiography and maghese recesses. Increased awareness of this disease entity made us recognize more and more cases of NCCM, especially with help of modern maging modalities like contrast echocardiography and MRI allowing better visualization the left ventricular cavity. However, in daily clinical practice, many questions about several clinical aspects remained open, because the available literature consisted of several case reports and small case series only.

• View at Worldcat

Free Full Text ( Final Version , 16mb )