We have examined the outcome of a large series of patients treated surgically for HOCM since 1963. The majority of patients were in NYHA class III and came to surgery after long-term medical, but finally insufficient, management. The perioperative risk could be reduced considerably during recent years, despite the advanced cardiomyopathy status. The long-term postoperative observation of the patients demonstrated an unexpectedly continuing good outcome. Therefore these results may serve as a standard for assessing the results after the less invasive alcohol-induced transcoronary ablation of septal hypertrophy.

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SummaryIn order to find evidence of prognosis and of presymptomatic manifestation of congestive cardiomyopathy (COCM) in fifty-eight patients, the extent of morphological changes of endomyocardial catheter biopsy (EMCB), clinical and haemodynamic data were correlated to the clinical course. In addition, clinical, haemodynamic, angiographic, morphological and His-bundle electrographic studies were performed in patients with left bundle branch block (LBBB), normal left ventricular end-diastolic volume, and normal coronary arteries (n = 43).Related to a 10-year mortality rate of 70%o from the onset of symptoms, COCM is one of the most severe heart diseases. Endomyocardial catheter biopsy (EMCB) allowed clear prognostic separation in patients with COCM and seems to be of diagnostic value in patients with only slightly enlarged hearts and in patients with a short history of symptoms. The studies also revealed much evidence that at least some patients with LBBB, normal left ventricular enddiastolic volume (LVEDV) and normal coronary arteries exhibit an early stage of COCM. In these patients especially EMCB with severe changes of heart muscle cells and/or impaired left ventricular function may indicate subsequent COCM. So that there is now a new indication for performing EMCB. IntroductionThe clinical course of patients with congestive cardiomyopathy can vary considerably, i.e. it can be fast or slow or may even show transient remission (Breithardt, Kuhn and Knieriem, 1978;

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In four patients with unexplained, abnormal thickening of the interventricular septum as demonstrated by echocardiography, right ventricular endomyocardial biopsy revealed unexpected cardiac amyloid deposits that resulted in increased myocardial thickness and rapidly progressive heart failure. Light microscopically, amyloid was observed in the subendocardial layer, interstitium, and walls of the intramural arterioles. Electron-microscopically, the amyloid fibrils were adjacent to the basement membranes of the heart muscle cells and the vascular smooth muscle cells. Immunohistochemical typing with specific antibodies against different amyloid fibril proteins on glutaraldehyde-fixed paraffin sections revealed different amyloid types. In two patients with generalized idiopathic amyloidosis and in two others with amyloidosis in multiple myeloma, the A-lambda form was diagnosed. In a fifth patient, AA-amyloidosis was found in familial Mediterranean fever with cardiac manifestation without thickening of the interventricular septum. The amyloid deposits were located almost exclusively within the walls of the myocardial arterioles. The amount of amyloid as observed in the myocardial biopsies correlates with the rapidly progressive cardiac failure. It is suggested that in patients with abnormal thickening of the interventricular septum of unknown origin the diagnosis should be clarified by endomyocardial biopsy.