Anomalous origin of the coronary artery can lead to angina pectoris, acute myocardial infarction or even sudden death in the absence of atherosclerosis. However, in Japan, this anomaly is usually treated medically rather than surgically. To clarify the clinical features of anomalous origin of the coronary artery in Japanese and the prognosis of such patients who are treated medically, we reviewed 56 patients with anomalous origin of the coronary arteries. The mean age of these patients was 55.9 +/- 11.5 years. Anomalous origin of the right coronary artery from the left sinus of Valsalva was seen most frequently (78.6%). In contrast, we found no cases of anomalous origin of the left coronary artery from the right sinus of Valsalva traversing between the aorta and the pulmonary trunk. A history of syncope (14.3%) and aorta regurgitation (21.4%) was frequent and serious complications during exercise stress testing occurred in 5 patients. These patients were treated medically, such as by limiting exercise or by the oral administration of medicine. During the follow up period (mean 5.6 +/- 4.2 years), death directly related to anomalous origin of the coronary artery was not found despite the lack of surgical treatment. Our results suggest that the prognosis of these middle-aged-to-elderly patients without atherosclerosis is relatively good, despite the lack of surgical treatment.

The relationship between the concentrations of intermediate-density lipoprotein (IDL) and other lipoproteins and the extent of coronary artery disease (CAD) was studied in 182 consecutive patients evaluated by selective coronary cineangiography. On univariate analysis, the extent of CAD correlated significantly and positively with very low density lipoprotein (VLDL) cholesterol, IDL cholesterol and low-density lipoprotein (LDL) cholesterol, and negatively with high-density lipoprotein (HDL) cholesterol. Analysis of four subgroups divided by IDL cholesterol and LDL cholesterol levels indicated that moderately increased levels of IDL cholesterol were closely associated with a high frequency of CAD. Moreover, multi-variate regression analysis demonstrated that IDL cholesterol for men, LDL cholesterol for men and women and HDL cholesterol for men were significant variables of use in the final weighting procedure. IDL cholesterol was closely associated with cholesterol-rich VLDL. This study shows that IDL and cholesterol-rich VLDL combine to contribute to the development of CAD.

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Background-The STA gene encodes emerin and is one of the genes that is affected in Emery-Dreifuss muscular dystrophy (EDMD). Although it has been reported that EDMD caused by the STA gene mutation is associated with X-linked recessive inheritance, the genotype-phenotype correlations, with special reference to cardiac manifestations, are not well defined. Methods and Results-We identified 16 carriers (7 male and 9 female) with a nonsense mutation in exon 6 of the STA gene in 2 EDMD families. Pacemakers were required for treatment of bradyarrhythmias in all 7 male carriers and in 2 of the 9 female carriers. In addition, 2 of the 9 female carriers displayed atrial fibrillation. In these 2 families, 3 males without pacemaker implantation, who were not tested genetically, had died suddenly. In these family members, the majority of carriers with the mutation had not been clinically diagnosed as having EDMD before genetic testing because of extremely mild or nonexistent skeletal myopathy. Conclusions-EDMD

Patients with LQTS (long QT syndrome) with a mutation in a cardiac ion channel gene, leading to mild-to-moderate channel dysfunction, may manifest marked QT prolongation or torsade de pointes only upon an additional stressor. A 59-year-old woman had marked QT prolongation and repeated torsade de pointes 3 months after initiation of probucol, a cholesterol-lowering drug. We identified a single base substitution in the HERG gene by genetic analysis. This novel missense mutation is predicted to cause an amino acid substitution of Met(124)-->Thr (M124T) in the N-terminus. Three other relatives with this mutation also had QT prolongation and one of them had a prolonged QT interval and torsade de pointes accompanied by syncope after taking probucol. We expressed wild-type HERG and HERG with M124T in Xenopus oocytes and characterized the electrophysiological properties of these HERG channels and the action of probucol on the channels. Injection of the M124T mutant cRNA into Xenopus oocytes resulted in expression of functional channels with markedly smaller amplitude. In both HERG channels, probucol decreased the amplitude of the HERG tail current, decelerated the rate of channel activation, accelerated the rate of channel deactivation and shifted the reversal potential to a more positive value. The electrophysiological study indicated that QT lengthening and cardiac arrhythmia in the two present patients were due to inhibition of I(Kr) (rapidly activating delayed rectifier K(+) current) by probucol, in addition to the significant suppression of HERG current in HERG channels with the M124T mutation.

Circ J 2009; 73: 589 -592 ransient left ventricular dysfunction with chest symptoms and electrocardiographic (ECG) changes mimicking those of acute myocardial infarction are known as takotsubo cardiomyopathy. [1][2][3] Although the pathogenesis of takotsubo cardiomyopathy remains unclear, physical or emotional stress is common and elevation of plasma catecholamines suggests that sympathetic stimulation plays a central role. When medical support is provided promptly, the long-term prognosis of takotsubo cardiomyopathy is generally good with full recovery of left ventricular contraction. Patients with takotsubo cardiomyopathy generally show T-wave inversion and QT-prolongation during the early phase, and these ECG changes typically normalize within several weeks with improvement of left ventricular wall motion. [1][2][3] The arrhythmias associated with takotusbo cardiomyopathy include atrioventricular (AV) block, sinus bradycardia, paroxysmal atrial fibrillation, ventricular tachycardia and ventricular fibrillation. [2][3][4][5][6][7][8][9] Here, we describe a case of takotsubo cardiomyopathy demonstrating persistent complete AV block after improvement of left ventricular wall motion that needed the implantation of a pacemaker. Case ReportAn 82-year-old woman with no history of cardiac disease or hypertension presented to the emergency department due to repeated episodes of syncope with incontinence. She had been stressed for about 2 weeks and could not sleep well because she was caring for her grandchildren who had been injured in a traffic accident. The patient had taken care of her grandchildren over the night before admission and complained of fatigue but denied chest pain. There was no family history of heart disease or sudden death. She had no history of arrhythmia or prior syncope. On admission her blood pressure was 110/72 mmHg, the pulse was 38 beats/min and body temperature was 35.6ºC. Her consciousness was clear, and a neurological examination revealed no neurological deficits. She had a repeated syncope lasting for about 10 s presenting torsades de pointes on monitoring ECG in the emergency department. The 12 leads ECG on admission showed complete AV block, diffuse T-wave inversions in leads I, aVL, V2-6 and prolonged QTc interval of 630 ms (Figure 1). Chest X-ray showed mild cardiomegaly and no pulmonary congestion. The laboratory data on admission are shown in Table 1. The serum level of creatine kinase was normal, but the rapid cardiac Troponin T assay was positive. Serum electrolytes, plasma norepinephrine and adrenaline levels were within the normal range. An elevated plasma level of brain natriuretic peptide suggested cardiac dysfunction. Echocardiography revealed akinesis in the left ventricular apical wall and no pericardial effusion. A temporary transvenous pacemaker was inserted due to complete AV block and to prevent the recurrence of torsade de pointes. Urgent coronary angiography was carried out and showed no significant coronary stenosis or vascular obstruction. Left ventriculography ...

A 56-year-old Japanese woman with an acute inferior myocardial infarction was admitted to hospital. Emergency coronary angiography revealed an anomalous origin of the right coronary artery from the left sinus of Valsalva, but there was no stenosis or thrombus in either the right or left coronary artery. Coronary spasm was provoked at the site of the proximal portion of the anomalous coronary artery, which was located between the aorta and pulmonary trunk. This was thought to be the cause of the myocardial infarction.

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