… 2 9 Survival curves (death or cardiac transplantation) of 126 patients with the dilated phenotype
of left ventricular hypertrabeculation/ noncompaction depending on presence (light blue) …

… (n=3), myotonic dystrophy (n=2) and Becker muscular dystrophy (n=1). Those patients in …
were brothers and two patients with myotonic dystrophy were father and daughter. In group …

… In rare cases, however, >3 trabeculations are found by autopsy or echocardiography in
the left ventricle. Left ventricular hypertrabeculation (LVHT) is easily overlooked, even by …

… Myotonic dystrophy type 1 follows an autosomal dominant trait. Depending on the size of
the CTG-repeat expansion and the dynamics of the expansion during mitosis, the clinical …

… , n=15; Leber's hereditary optic neuropathy, n=3; myotonic dystrophy, n=2 and Becker
muscular dystrophy, n=1). The remaining 27 had a NMD of unknown etiology. Neurologically …

… Left ventricular hypertrabeculation was diagnosed in 86 patients (21 female, 65 male, age: …
left ventricular hypertrabeculation in myotonic dystrophy type 1. Herz 2003; 28: 466– 470. …

… Among these are dystrophinopathies, dystrobrevinopathies, myotonic-dystrophy types 1
and 2, zaspopathy, myoadenylate-deaminase-deficiency, Charcot-Marie-Tooth disease, …

… left, showing left ventricular hypertrabeculation/noncompaction affecting the apical region of
the left ventricle… in four of these patients, myotonic dystrophy-1 in 2 patients, and myopathy of …

… This study aimed to determine whether white matter lesions, previously described as a
frequent feature in myotonic dystrophy type 1 (DM1), aggregate within DM1 families or are …

… Figure 1. Autopsy specimen of hypertrabeculated left ventricle (LV) shows numerous
trabeculations in LV apex (1). Only interventricular septum (2) and LV outflow tract are free of …