Cerebrovascular events in left ventricular hypertrabeculation/noncompaction with and without myopathy

Little is known about frequency, morphology, and causes of stroke in patients with left-ventricular hypertrabeculation/noncompaction (LVHT). Aim of this study was to find out how many LVHT-patients suffer from stroke, which abnormalities are found on cerebral imaging, and if stroke LVHT-patients differ from non-stroke LVHT-patients with regard to cardiac and neuromuscular abnormalities. Method and materials Records of all included patients were reviewed for stroke diagnosed upon history, clinical examination, or cerebral CT/MRI (CCT, cMRI). Results Among 104 LVHT-patients included, stroke was diagnosed in 16 (15%). Of these two had a transitory ischemic attack and one a prolonged ischemic deficit. Among 36 patients undergoing a CCT (n = 32), cMRI (n = 9), or both (n = 5) territorial stroke was found in 7, lacunar stroke in 2, and intracerebral bleeding in 1. Other imaging findings were atrophy (n = 7), microangiopathy (n = 4), leucencephalopathy (n = 3), basal-ganglia-calcification (n = 2), or non-specific hypodensities (n = 2). Imaging studies without vascular lesions were found in 2 patients. At least one classical risk factor for stroke was found in 15 patients. Stroke-patients did not differ from non-stroke-patients regarding cardiac or neuromuscular findings. Conclusions If systematically looked for, stroke is a frequent feature of LVHT. A causal relation between LVHT and stroke, however, is rather the exception than the rule.