title: [Hypertrophic non-obstructive cardiomyopathy (HNCM)--clinical, hemodynamic, electro-, echo- and angiocardiographic studies (author's transl)].
abstract: In order to characterize the clinical picture of hypertrophic non obstructive cardiomyopathy (HNCM) symptoms, ECG, echocardiogram, left ventricular angiogram, hemodynamics, the clinical course and the familial incidence were analyzed in 49 patients. The most striking findings in the ECG were slight or deeply negative T-waves with a maximum in leads V4 or V5. The diagnostic specifity for HNCM in 22 consecutive cases with this abnormal finding of unknown etiology was 91%. The 2-dimensional echocardiogram and the ventriculogram revealed a funnel-shaped obliteration of the apex of the left ventricle in 94% (wall thickness of the basis was 14.8 +/- 2.9 and at the apex 19.3 +/- 5.3 mm compared to 8.5 +/- 1 and 8.6 +/- 1.2 mm in normal subjects). The M-mode echocardiogram showed asymmetric septal hypertrophy in 72% and symmetric hypertrophy or a thicker posterior wall in 28%. By endomyocardial catheter biopsy (n = 9) and in two patients who died, no storage disease was detected. An abnormal increase of mean pulmonary artery pressure at exercise was measured in 59%. The clinical course (n = 37, follow-up 5.1 years in the mean) was stable in 68%, 5% died. There was a significant negative correlation between functional class and IVS/PW ratio, left ventricular enddiastolic diameter and enddiastolic volume and a significant positive correlation between functional class and left ventricular enddiastolic pressure. The familial incidence was 15%. The data demonstrate that HNCM is a well definable clinical entity which seems to be more frequent than it has been assumed before.
mesh_terms: D000293:Adolescent; D000328:Adult; D000790:Angiocardiography; D009202:Cardiomyopathies / Q000235:genetics / Q000473:pathology*; D002312:Cardiomyopathy, Hypertrophic / Q000473:pathology; D002648:Child; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006973:Hypertension / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011651:Pulmonary Artery
vernacular_title: Die hypertrophische nicht obstruktive Kardiomyopathie (HNCM)--klinische, hämodynamische, elektro-, echo- und angiokardiographische Untersuchungen.