Structural remodeling of the intramyocardial coronary arterioles and the accumulation of fibrillar collagen are decisive factors for a reduced coronary dilatory capacity in patients with arterial hypertension and angina pectoris in the absence of relevant coronary artery stenoses.

The prevalence of renal artery stenosis in diabetic patients is unknown, since no noninvasive and valid screening procedures are available. We have therefore evaluated 5194 consecutive autopsy protocols from patients who died between 1980 and 1988. In addition, all available clinical records for patients with renal artery stenosis (RAS) and a random sample were evaluated. It was found that 73% of patients with RAS were hypertensive, and 53% had diabetes, all but one being Type 2 (non-insulin-dependent). Renal artery stenosis was present in 225 (4.3%) of all patients [95% confidence interval (95% CI), 3.8-4.9], and was not reported in the patients' clinical records in 93% of cases. RAS was present in 8.3% of all diabetic patients (95% CI, 6.8-9.8%), the odds ratio being 3.5 (95% CI, 2.6-4.6). The frequency of renal artery stenosis in diabetic patients with hypertension was 10.1%. Bilateral renal artery stenosis was found in 43% of patients with RAS and diabetes, and in 30% of non-diabetic patients with RAS (P = 0.059). Our results indicate that renal artery stenosis often goes undetected before autopsy. The presence of non-insulin-dependent diabetes mellitus increases the risk of renal artery stenosis. The risk of bilateral renal artery stenosis is greater in diabetic patients. These results may be of significance with regard to the diagnostic evaluation and choice of antihypertensive treatment in hypertensive non-insulin-dependent diabetic patients.

We studied the size of infarcts in 25 dogs 48 hrs after proximal occlusion of the left anterior descending coronary artery. In one group of animals infarct size was measured by histologic criteria, in another group the infarct was measured macrohistochemically using p-NBT and malate to incubate unfixed slices of myocardium. In both groups infarct size was expressed as percentage of the area of perfusion of the occluded artery. Infarct size was 72% of the area-at-risk in the group studied by histology and 74.5% in the macrohistochemical group. The satisfactory agreement of both methods favors the p-NBT technique because of its ease and speed. It is suggested that the expression of infarct size as percentage of the perfusion area is a good definition and should be used in experiments designed to manipulate infarct size. In this way differences in the size of occluded arteries and their respective perfusion areas have no or only a negligible influence on infarct size.

Myocytic hypertrophy and increased perimyocytic fibrosis accompany intraventricular pressure overload (hypertension and aortic stenosis) in human hearts. Myocardial structure as a result of arterial hypertension, but not aortic stenosis, is also characterized by intramyocardial arteriole wall-thickening and increased perivascular fibrosis. Thus, distinct structural reaction patterns are noted in the cardiac hypertrophy associated with hypertension and aortic stenosis.

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.

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Kearns-Sayre syndrome is clinically defined by progressive external ophthalmoplegia, atypical retinitis pigmentosa and the potential occurrence of complete atrioventricular (AV) block. Right septal endomyocardial biopsy specimens from nine patients (four men and five women with a mean [+/- SD] [corrected] age of 36.3 +/- 14.4 years) with chronic progressive external ophthalmoplegia and mitochondrial skeletal myopathy were studied. Three patients had atypical retinal pigmentation. An atrioventricular or intraventricular conduction defect was observed in five patients. A pacemaker was prophylactically implanted in one patient because of abnormal conduction distal to the His bundle. Ultrastructural investigations revealed mitochondriosis in many heart muscle cells and an increased variability of mitochondrial form and size in all patients. In seven patients, 0.4 to 2.1% of all examined myocytes contained exclusively abnormal mitochondria. Three main types were observed: huge, mainly round mitochondria with concentric cristae; large, round or oval mitochondria with transverse or curved cristae; and small, vacuolated mitochondria. The volume density of myofibrils was reduced (41.9 +/- 11.1 compared with the normal value of 56.5 +/- 2.5 volume density [in percent], p less than 0.01) in these myocytes. Increasing numbers of vacuolated mitochondria correlated significantly with a reduction of myofibrils (r = -0.64, p less than 0.01). The data suggest that the ventricular myocardium of most patients with complete and even incomplete Kearns-Sayre syndrome is affected by disseminated mitochondrial cytopathy.