Coronary ectasia in a homozygous patient with familial hypercholesterolemia.

A 23-year-old man with homozygous familial hypercholesterolemia was found to have coronary ectasia by coronary angiography. This case showed generalized xanthomatosis and severe hyper low density lipoproteinemia, and his cultured skin fibroblasts showed LDL receptor activities compatible with the receptor-defective homozygous type of familial hypercholesterolemia. Coronary angiography showed fusiform aneurysmal involvements in the right coronary artery and left circumflex artery, and 50% stenosis in the right coronary artery and left anterior descending artery. Thus, homozygous familial hypercholesterolemia produces coronary ectasia as well as premature coronary stenosis.

Coronary angiographic characteristics in Japanese patients with heterozygous familial hypercholesterolemia.

Coronary angiographic findings were analyzed in 51 consecutive patients (36 males and 15 females) with heterozygous familial hypercholesterolemia (FH) and 279 consecutive patients (216 males and 63 females) without FH (non-FH). The coronary stenosis index and over 75% stenosis vessel subset were almost three times as high in the FH group. The incidence of myocardial infarction was almost twice as high in the FH group. Levels of total cholesterol and its lipoprotein fractions, except HDL-cholesterol, were almost twice as high in the FH group. In the FH group aged under 50 years, the two parameters of coronary angiogram and the incidence of myocardial infarction were significantly higher in males than in females. However, in the group aged over 50 years, all three parameters were not significantly different between those in males and females. The level of HDL-cholesterol was significantly lower in males than in females. A significantly higher incidence (18%) of coronary ectasia was observed in the FH group compared with the incidence (2%) in non-FH. All patients with coronary ectasia were males, except one female with FH. On comparison of the males among the FH patients with those among the non-FH patients matched for total cholesterol, age and other risk factors, the FH patients were associated with a significantly higher degree of coronary atherosclerosis and lower level of HDL-cholesterol. Seven FH patients with a normal coronary angiogram were observed. However, any factors as regards age, total cholesterol, HDL-cholesterol and Achilles tendon thickness failed to distinguish between the FH patients with a normal coronary angiogram and those without.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute increases in plasma oxidized low-density lipoprotein immediately after percutaneous transluminal coronary angioplasty.

To investigate the acute changes in plasma oxidized low-density lipoprotein before and immediately after coronary angioplasty, we studied 132 consecutive patients who successfully underwent this procedure. Plasma oxidized low-density lipoprotein levels were significantly increased immediately after coronary angioplasty in patients with stable angina pectoris as well as in those with acute coronary syndromes.

Germanium intoxication with sensory ataxia.

Sensory ataxia in inorganic germanium intoxication is rare. A 63-year-old housewife had taken inorganic germanium preparations at a dosage of 36 mg a day for about 6 years (total dose about 80 g). She subsequently developed difficulty in writing and gait disturbance with peripheral neuropathy and renal involvement. Germanium, which is not usually detected in the non-germanium user, was accumulated in her hair and nails, permitting a diagnosis of inorganic germanium intoxication. The peripheral neuropathy and renal injury were not reversible after discontinuing the preparation. Pneumonia and sepsis then supervened and the patient died. Autopsy findings showed degeneration and loss of the dorsal root ganglion cells and degeneration of the dorsal column of the spinal cord. Two previously reported cases presented with ataxia. These patients took germanium for long periods and/or large quantities like our case. It was supposed that sensory ataxia was induced by chronic and dose dependent toxicity of inorganic germanium.

Quantitation of myocardial fibrosis and its relation to function in essential hypertension and hypertrophic cardiomyopathy.

Myocardial interstitial fibrosis is an important microscopic feature of hypertrophic cardiomyopathy. To determine whether interstitial fibrosis of the myocardium in hypertrophic cardiomyopathy and essential hypertension differ in quality or quantity, and to determine whether fibrosis affects cardiac function directly, we measured the percentage of fibrosis in patients of both categories and compared the severity of fibrosis with several cardiac functions. Left and right ventricular endomyocardial biopsies were performed in 25 patients with essential hypertension and in 19 patients with hypertrophic cardiomyopathy. Interstitial fibrosis was classified into four different microscopic types, and the percentage of total and of each type was calculated using the point-counting method. Although the percentage of total fibrosis was similar between the two groups, the type of fibrosis was different. There was no correlation between the percentage of total fibrosis and the mean size of myocytes in either group. Although there was a significant correlation between the percentage of total fibrosis and the thickness of the interventricular septum in hypertrophic cardiomyopathy, such correlation was lacking in hypertension. There was no correlation between the percentage of total fibrosis and the ejection fraction, cardiac index, or left ventricular end-diastolic pressure in either group. We concluded that the amount of myocardial interstitial fibrosis in hypertrophic cardiomyopathy is no greater than that in essential hypertension, but the type of fibrosis is different. Furthermore, in subjects in whom the ejection fraction is normal or only slightly decreased, fibrosis does not influence global cardiac functions.

Clinical studies on diabetic myocardial disease using exercise testing with myocardial scintigraphy and endomyocardial biopsy.

Nine diabetics without significant coronary stenosis participated in an exercise testing protocol with thallium-201 myocardial scintigraphy. Endomyocardial biopsy of right ventricle was also obtained. There were 4 patients with abnormal perfusion (positive group) and 5 patients with normal perfusion (negative group). All cases of the positive group were familial diabetics and there was only one case of dietary treatment, whereas in the negative group, there were only 2 cases of familial diabetics and 3 cases receiving dietary treatment. No statistical differences between the positive and negative groups were observed for the data of exercise performance and hemodynamic parameters in cardiac catheterization at rest. However, the mean ejection fraction in the positive group (62 +/- 13%) was significantly lower than in the negative group (77 +/- 4%). In both groups, the mean diameter of myocardial cells and the mean percent fibrosis of biopsy specimens showed significant increases compared with the control group. The mean percent fibrosis in the positive group (24.1 +/- 8.5%) compared with that in the negative group (16.5 +/- 5.9%) showed a tendency to increase. It is suggested that the abnormal perfusion of thallium-201 in the positive group indicates subclinically a pathological change of microcirculation caused by diabetes mellitus.

Goodpasture's-like syndrome and effect of extracorporeal membrane oxygenator support.

A 49-year-old man was admitted because of general fatigue, cough and hematuria. During the hospital course, acute renal failure, hemoptysis and dyspnea developed. A percutaneous renal biopsy revealed a diffuse crescentic glomerulonephritis, and direct immunofluorescence showed a linear pattern of IgG along the glomerular basement membrane. Although serum anti-glomerular basement membrane (anti-GBM) antibody was not detected. Goodpasture's-like syndrome was suspected, and methylprednisolone pulse therapy and plasmapheresis were administered. Concomitantly, extracorporeal membrane oxygenation (ECMO) was instituted because of deterioration in respiratory status due to a severe pulmonary hemorrhage despite maximal ventilatory support. Temporarily, the patient improved and ECMO was discontinued. ECMO may be a useful therapeutic support for hypoxia resulting from pulmonary hemorrhage in Goodpasture's syndrome (GPS) and Goodpasture's-like syndrome.

[Postoperative percutaneous coronary angioplasty for stenosed aortocoronary vein bypass].

This paper was presented to demonstrate the efficacy of postoperative PTCA for varying degree of stenosis aortocoronary bypass with saphenous vein grafts. The following conclusions were drown. 1. The success rate of postoperative PTCA for 15 grafts showed 88.8%. 2. The rate of success for PTCA was much more effective in patients within one year after bypass operations. The authors concluded that postoperative PTCA can be done with excellent rate of success. Therefore, postoperative cine-coronary angiography should be done to evaluate the state of the graft and to decide to perform possible postoperative PTCA in order to keep long time patency rate of saphenous vein grafts.

[A case of successful emergency coronary artery bypass grafting surgery for failed coronary angioplasty using percutaneous cardiopulmonary support].

This is the first Japanese case of the successful emergency coronary artery bypass grafting (CABG) surgery for failed percutaneous transluminal coronary angioplasty (PTCA) using percutaneous cardiopulmonary support (PCPS). A 81-year-old woman with old myocardial infarction and angina pectoris is presented. Her coronary angiogram showed the 90% and 50% stenosis of the right coronary artery (RCA) and the total occlusion of the left anterior descending artery (LAD). PTCA for the 90% stenosis of RCA was performed. But, during the balloon dilation, her heart rate and blood pressure decreased. PTCA was stopped. As her chest pain was worse, re-PTCA was tried, using PCPS. Under PCPS (3 l/min), the balloon dilation was performed safely and smoothly. But, the unexpected dissection of RCA occurred, and became larger rapidly. After 85 minutes, the emergency CABG was performed. By using PCPS, the stable hemodynamics was given till the operation. CABG to RCA and LAD was performed safely. After the surgery, the patient progressed well. PCPS was a very useful cardiopulmonary assist device.