[Hypertrophic cardiomyopathy: ECG-VCG abnormalities in absence of the echocardiographic markers in a family (author's transl)].
We report a family, in which two members, the propositus and his father had an left ventriculography highly indicative of non-obstructive or labile mild obstructive hypertrophic cardiomyopathy. In these two members the M-mode and the two dimensional echocardiography did not reveal the features of the hypertrophic cardiomyopathy, i.e. the increased thickness of the interventricular septum and the asymmetric septal hypertrophy, whereas the electrocardiogram and the Frank vectorcardiogram showed an increase of the QRS anterior forces voltage in the transverse plane. In a third relative, the younger brother of the propositus, a similar discrepancy between the VCGraphic and the echocardiographic data was present. The presence of significant ECG-VCGraphic abnormalities in subjects with documented hypertrophic cardiomyopathy and slightly increased septalto-free wall ratio has been previously reported by other Authors. However, the interest of our observation is in the presence of this discrepancy in many members of the same family. Although the ventriculographic pattern was not consistent in our subjects with that found by Yagamuchi et al. in patients with apical non-obstructive hypertrophic cardiomyopathy, a prevailing obliteration of the apical portion of the left ventricular chamber at end-systole was evident in both our cases. This finding however was not revealed by two-dimensional echocardiogram. Although it was not possible exclude on firm grounds that cases described were a normal variant, our observation seems to confirm that the echocardiography does not offer the unique "gold standard" for the non-invasive identification of the hypertrophic cardiomyopathy.
D000293:Adolescent; D002312:Cardiomyopathy, Hypertrophic / Q000175:diagnosis* / Q000235:genetics; D004452:Echocardiography; D004562:Electrocardiography; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D014672:Vectorcardiography
Cardiomiopatia ipertrofica. Anormalità ECG-VCG in assenza di markers ecocardiografici in una famiglia.