Partial type of common atrioventricular canal defect associated with mitral stenosis.

We report a 63-year-old woman, with a partial type of common atrioventricular canal and mitral stenosis, who was hospitalized because of dyspnea on exertion. Two-dimensional echocardiogram showed an ostium primum atrial septal defect with two well-formed AV valves located at the same level. However, both anterior and posterior mitral leaflets were markedly thickened with a thickened subvalvular apparatus, and the commisures were fused. Echocardiographic measurements demonstrated a mitral valve area of 1.48 cm2 with mild mitral regurgitation. Cardiac catheterization demonstrated mild pulmonary artery hypertension with a large left to right shunt (72%) at the atrial level. The combination of the partial type of common atrioventricular canal and mitral stenosis is rare; only one similar case has been reported previously in the literature.

Dilated cardiomyopathy associated with hyperthyroidism.

We report a case of dilated cardiomyopathy with hyperthyroidism. A 28-year-old man was admitted because of congestive heart failure and atrial fibrillation, and was newly diagnosed as having hyperthyroidism. Despite administration of antithyroid medication, he developed recurrent congestive heart failure. An echocardiogram revealed a moderately dilated left ventricle with diffuse hypokinesis. Though his thyroid function normalized, the patient's cardiac dysfunction did not improve. Beta-blocker therapy was begun with subsequent improvement in clinical symptoms. This suggests that beta-blocker treatment may be effective in patients with atrial fibrillation associated with cardiomyopathy and hyperthyroidism.

[A Japanese family with congenital abnormal plasminogen].

A Japanese family with congenital abnormal plasminogen is reported. The patient was a 44-year-old male with no past history of thrombosis. Since only the plasminogen (PLG) activity was reduced on laboratory tests before surgery for lumbar disc herniation, coagulation and fibrinolysis studies were performed in the patient and his family. The patient underwent resection of the nucleus pulposus and posteriorlateral fixation of the lumbar spine. The PLG activity was 8% in the patient and his sister, 55% in his father, and 53% and 48% in his nephew brothers. The PLG antigen level was normal in all members of his family examined. IEF of PLG antigen showed abnormal patterns in which all bands were shifted slightly to the cathode side in the patient and his sister, but his father and nephew brothers exhibited duplicated bands showing combinations of normal and abnormal patterns. From these results, the proband and his sister were considered to be homozygotes, and his father and nephew brothers to be heterozygotes for congenital abnormal plasminogen. Acute reactant substances (fibrinogen, CRP, CPK, C1IN, alpha 1AT, etc.) and PIC (plasmin, alpha 2-plasmin inhibitor complex) increased after the operation due to the surgical insult, but the surgery did not trigger thrombosis. This patient is considered not to have developed thrombosis although he was a homozygote for congenital abnormal plasminogen, because the anticoagulation process until thrombogenesis was normal.

[Effects of repetitive myocardial ischemia on collateral circulation, ST deviation and epicardial wall motion].

The effects of repetitive myocardial ischemia on collateral circulation, ST deviation and epicardial wall motion were examined in 12 patients undergoing percutaneous transluminal coronary angioplasty (PTCA) for single left anterior descending artery disease. Rentrop collateral filling grade was assessed using contrast injection to the contralateral artery during the first and final episodes of coronary occlusion. ST deviation was measured by intracoronary electrocardiography. Epicardial wall motion (%pre PTCA) was measured by guide wire motion analysis according to the centerline method. Collateral filling grade was assessed 30 sec after balloon inflation. The ST segment and the epicardial wall motion were measured 60 sec after balloon inflation and deflation, respectively. There was no change in the collateral filling grade between the first and final episode of coronary occlusion. Patients with collateral filling grade I (R-I group, n = 4), II (R-II group, n = 3) and III (R-III group, n = 5) during coronary occlusion showed mean ST segment shifts of 13.2, 9.4, and 0.9 mm, respectively, and mean epicardial wall motion of 41.4%, 67.2%, and 78.5%, respectively. The collateral filling grade correlated with ST deviation and epicardial wall motion, and there was a significant correlation between epicardial wall motion and ST deviation (r = -0.67). Comparison of the R-I group or severe ischemia (n = 4) and the R-III group or slight ischemia (n = 4) during coronary occlusion for the fourth time showed the effect of preconditioning was obtained in R-I group. More R-III group patients than R-I group had hyperkinetic epicardial wall motion during coronary reperfusion. Stunned myocardium was demonstrated in both R-I group and R-III group patients. Epicardial wall motion was poorer in the R-I group than R-III group. We concluded the following: There is no change in the grade of collaterals during repetitive coronary occlusion, and there is a relationship between the grade of collateral and degree of myocardial protection; there is good correlation between ST segment and epicardial wall motion; ischemic preconditioning is obtained during repetitive severe myocardial ischemia; recovery from brief episodes of slight myocardial ischemia is associated with hyperkinesia of epicardial wall motion; the reduction of stunned myocardium is related to the degree of premyocardial ischemia; preconditioning is sufficient to cause myocardial stunning, but myocardial stunning is insufficient to cause preconditioning.

Preventive effects of an antiallergic drug, pemirolast potassium, on restenosis after percutaneous transluminal coronary angioplasty.

BACKGROUND: We recently confirmed that pemirolast potassium, an antiallergic agent, markedly inhibits migration and proliferation of vascular smooth muscle cells. It has also been reported that pemirolast inhibits intimal hyperplasia in animal experiments. METHODS AND RESULTS: To elucidate the preventive effects of pemirolast on restenosis after percutaneous transluminal coronary angioplasty (PTCA), 227 patients were enrolled in this prospective, randomized trial. A total of 205 patients who were compatible with the protocol were analyzed (pemirolast group, 104 patients with 140 lesions; control group, 101 patients with 133 lesions). Patients in the pemirolast group received 20 mg/d of pemirolast from 1 week before PTCA until the time of follow-up angiography (4 months after PTCA). Angiographic restenosis was defined as diameter stenosis >/=50% at follow-up. Restenosis rates were significantly lower in the pemirolast group than in the control group (24.0% vs 46.5% of patients, 18.6% vs 35.3% of lesions, P <.01, respectively). During 8 months of follow-up, there were no coronary events (death, myocardial infarction, coronary artery bypass surgery, or repeated PTCA) in 81.7% of the pemirolast group and in 63.4% of the control group (P =.013). CONCLUSIONS: This study suggested that pemirolast would be useful in the clinical setting to prevent restenosis after PTCA.

Angioscopic evaluation of stabilizing effects of bezafibrate on coronary plaques in patients with coronary artery disease.

Background Since long-term administrations of anti-hyperlipidemic agents result in reduction in % stenosis or increase in minimum lumen diameter (MLD) of stenotic coronary segments, it is generally believed that anti-hyperlipidemic agents stabilize vulnerable coronary plaques. However, recent pathologic and angioscopic studies revealed that vulnerability of coronary plaques is not related to severity of stenosis and the rims rather than top of the plaques disrupt, and therefore, angiography is not adequate for evaluation of vulnerability.Angioscopy enables macroscopic pathological evaluation of the coronary plaques. Therefore, we carried out a prospective angioscopic open trial for evaluation of the stabilizing effects of bezafibrate on coronary plaques.Methods From April, 1997 to December, 1998, 24 patients underwent coronary angioscopy of the plaques in the non-targeted vessels during coronary interventions and 6 months later. The patients were divided into control (10 patients, 14 plaques) and bezafibrat (14 patients, 21 plaques) groups. Oral administration of bezafibrate (Bezatol SR, 400mg/day) was started immediately after the interventions and was continued for 6 months. The vulnerability score was determined based on angioscopic characteristics of plaques and it was compared before and 6 months later.Results Six months later, vulnerability score was reduced (from 1.6 to 0.8;p < 0.05) in bezafibrate group and unchanged (from 1.4 to 1.3; NS) in control group. In bezafibrate group, the changes in vulnerability score was not correlated with those in % stenosis or MLD. Conclusion The results indicate that bezafibrate can stabilize coronary plaques.