A Case of ST-elevated myocardial infarction resulting from obstructive intramural coronary amyloidosis.

A 49-year-old man presenting with ST-elevated myocardial infarction was brought to our emergency department with AL amyloidosis. Baseline coronary angiography showed no significant stenosis of the epicardial coronary arteries, however, coronary artery angiography in response to acetylcholine and coronary flow reserve in response to papaverine were abnormal, which suggested impairment of vascular endothelial function. Myocardial biopsy revealed amyloid deposition exclusively in intramural coronary arteries. Early amyloidosis without myocardial involvement can produce acute coronary syndrome through the combination of spastic epicardial coronary arteries and obstruction of the intramural coronary arteries. In the management of certain patients with acute coronary syndrome, the possibility of cardiac amyliodosis should be taken into consideration.

A case of early phase dialysis associated effusive constrictive pericarditis with distinct surgical findings.

A 55-year-old male patient with a 10 year history of hemodialysis was admitted for examination of pericardial effusion. Specific echocardiography, MRI, and cardiac catheterization findings strongly suggested a diagnosis of effusive constrictive pericarditis. Pericardiectomy showed the following distinct findings. Two layers of fibrous tissue below the thickened pericardium emerged. The cardiac constriction had not been relieved until the inner layer was partially resected. We conclude that patients in dialysis associated constrictive pericarditis should undergo pericardiectomy before the adhesion between fibrous tissues becomes hard.

Beneficial effects of pitavastatin, a 3-hydroxy-3-methylglutaryl coenzyme a reductase inhibitor, on cardiac function in ischemic and nonischemic heart failure.

HMG-CoA reductase inhibitors (statins) have recently been reported to improve cardiac function, and decrease the incidence of heart failure (HF) in hyperlipidemic patients. However, evidence for statin treatment in patients with HF remains a subject of debate. Thus, a study was initiated to examine the effects of pitavastatin on cardiac function evaluated by echocardiographic findings and plasma brain natriuretic peptide (BNP) levels in patients with HF. Twenty-three patients with HF were treated with pitavastatin 1-2 mg/day in addition to standard therapy for 7.5 +/- 3.8 months. Left ventricular end-diastolic dimension (LVDd) and left ventricular end-systolic dimension (LVDs) were determined by echocardiography. Left ventricular ejection fraction (LVEF) was calculated using Teichholz's formula. Serum lipid and plasma BNP levels were also measured. During the follow-up period, LVEF was increased from 42 +/- 11 to 48 +/- 13% (P = 0.002). LVDs was reduced from 43 +/- 10 to 40 +/- 10 mm (P < 0.001), while there was no change in LVDd. E/A (n = 10) and deceleration time (n = 7), obtained in some patients, did not change significantly (0.89 +/- 0.33 to 0.77 +/- 0.17%, and 215 +/- 46 to 227 +/- 72 msec, respectively). In addition, the plasma BNP level was moderately, but significantly decreased from 94 +/- 78 to 70 +/- 56 pg/mL (P = 0.005). In subgroup analysis, LVEF was improved in both patients with ischemic and nonischemic HF. There was no significant correlation between the percent change in serum total cholesterol and the percent change in LVEF by pitavastatin treatment. Serum total cholesterol, LDL-cholesterol, and triglycerides decreased by 21%, 30%, and 15%, respectively, and HDL-cholesterol increased by 12%. Pitavastatin improved cardiac function in patients with HF, which generally worsens with time. The results suggest that pitavastatin may be beneficial for treatment of HF.

A one-year study of the antiatherosclerotic effect of the angiotensin-II receptor blocker losartan in hypertensive patients. A comparison with angiotension-converting enzyme inhibitors.

Angiotensin receptor blockers (ARB) have been emerging as drugs to treat atherosclerosis. The effectiveness of the ARB losartan at reducing atherosclerosis was compared with that of ACE inhibitors in hypertensive patients. A total of 50 patients with hypertension were divided into 3 groups: a control group receiving neither an ARB nor an ACE inhibitor (n = 14), a losartan group (n = 22) receiving 50 mg/day of losartan, and an ACE inhibitor group (n = 14) receiving either 5 mg/day of enalapril or 5 mg/day of imidapril. Atherosclerosis was evaluated based on the intima-media thickness (IMT) of the common carotid artery measured by B-mode ultrasound at baseline and after approximately 12 months of treatment. After the treatment, IMT significantly decreased with losartan (from 0.87 +/- 0.14 to 0.79 +/- 0.16 mm, P < 0.05) and with ACE inhibitor (from 0.81 +/- 0.14 to 0.74 +/- 0.11 mm, P < 0.05). The reduction was comparable between the two groups, -0.078 +/- 0.136 with losartan and -0.073 +/- 0.109 mm with ACE inhibitor, and the rate of the reduction was similar between the two drugs; -0.098 +/- 0.142 mm/year with losartan and (-0.076 +/- 0.118 mm/year) with ACE inhibitor. On the contrary, IMT did not change in the control group (from 0.90 +/- 0.20 to 0.95 +/- 0.26 mm) during the treatment period. Concomitant medication and coronary risk factors such as hyperlipidemia, diabetes mellitus, and smoking did not differ significantly among the groups. The antiatherosclerotic effect of losartan on the carotid artery was comparable to that of ACE-inhibitors, and less adverse effects, such as coughing that occurs with ACE inhibitors, were observed. Losartan appears to be a better alternative to ACE inhibitors for treating atherosclerosis in Japanese hypertensive patients.

A case of almost-exclusively atria-dominant cardiac amyloidosis related with hemodialysis.

We report a case of a 68-year-old man who had dialysis-related amyloidosis. The patient underwent neck surgery due to spondyloarthritis. Unfortunately he died after the surgery in sepsis. The preoperative routine echocardiography didn't suggest cardiac involvement of amyloidosis. However, the pathological findings in autopsy revealed severe amyloid depositions of both atria in contrast to the quite mild involvement of ventricles. We have experienced that the cardiac involvement of dialysis-related amyloidosis may be confined to atria almost sparing ventricles. Therefore, in patients with hemodialysis-related amyloidosis, even if echocardiogram doesn't suggest any ventricular amyloidosis, physicians should consider the possibility of atrial amyloidosis and should check the detail of atrial findings. If the A wave in the mitral filling pattern seems small or absent, physicians have to proceed transesophageal echocardiogram to detect intra atrial thrombus and consider anti-coagulation therapy to prevent thromboembolic complications of atrial amyloidosis.

A case of transient left ventricular ballooning with pheochromocytoma, supporting pathogenetic role of catecholamines in stress-induced cardiomyopathy or takotsubo cardiomyopathy.

We experienced a case with severe acute heart failure showing "takotsubo"-like left ventricular asynergy. Subsequently, he was found to have pheochromocytoma, and underwent emergent adrenalectomy, which almost completely reversed his cardiac function and symptoms. His clinical course, supports the pathogenetic role of catecholamines in "takotsubo cardiomyopathy".

Upregulation of heat shock transcription factor 1 plays a critical role in adaptive cardiac hypertrophy.

Exercise-induced cardiac hypertrophy has been reported to have better prognosis than pressure overload-induced cardiac hypertrophy. Cardiac hypertrophy induced by exercise was associated with less cardiac fibrosis and better systolic function, suggesting that the adaptive mechanisms may exist in exercise-induced hypertrophy. Here, we showed a critical role of heat shock transcription factor 1 (HSF1), an important transcription factor for heat shock proteins, in the adaptive mechanism of cardiac hypertrophy. We examined expression of 8800 genes in the heart of exercise-induced hypertrophy model using DNA chip technique and compared with pressure overload-induced hypertrophy. Expression of HSF1 and its target molecule heat shock proteins was significantly upregulated in the heart by exercise but not by chronic pressure overload. Constitutive activation of HSF1 in the heart significantly ameliorated death of cardiomyocytes and cardiac fibrosis and thereby prevented cardiac dysfunction as well as hypertrophy induced by chronic pressure overload. Conversely, decreased activity of HSF1 in the heart promoted cardiac dysfunction in response to exercise, a load that normally leads to adaptive hypertrophy with preserved systolic function. Likewise, cardiac function was significantly impaired from the early phase of pressure overload, when HSF1 activation was inhibited. These results suggest that HSF1 plays a critical role in the transition between adaptive and maladaptive hypertrophy.

A case of transient 2:1 atrioventricular block, resolved by thyroxine supplementation for subclinical hypothyroidism.

A 42-year-old man was admitted to our hospital with palpitation attacks. Holter ECG showed 2:1 atrioventricular block and bradycardia with the minimum heart rate of 44 beats/min. There was a possible indication of electrophysiological study and cardiac pacemaker implantation. Laboratory data on admission revealed elevated thyrotropin level, with normal thyroxine level. To rule out functional atrioventricular block, we tried 2 weeks of the thyroxine supplementation, and Holter ECG showed improved heart rate without any atrioventricular block or long pause. We experienced that subclinical hypothyroidism caused severe bradycardia and 2:1 atrioventricular block, and that thyroxine supplementation completely improved these conditions.

[Atrial wall hematoma after mitral valvuloplasty and maze procedure: a case report].

A 66-year-old male patient with mitral regurgitation and atrial fibrillation underwent mitral valvuloplasty with a modified maze procedure. Follow-up echocardiography performed on the 4th postoperative day revealed a high echoic mass of 6.7 x 3.0 cm which appeared continuous to the left atrial wall and protruded into the left atrial cavity. The irregular surface of the mass echo was not compatible with atrial wall hematoma, so computed tomography (CT) was performed for the differential diagnosis. CT showed extension of the mass from the left atrium to the interatrial septum and right atrium, which lead to a conclusive diagnosis of atrial wall hematoma. Echocardiography showed the hematoma was diminished and eventually disappeared after simply reducing the warfarinization level. This rare case of atrial wall hematoma, especially after the maze procedure, required differential diagnosis from intraatrial thrombus and atrial wall dissection.

A case of graft infection 10 years after ascending aorta replacement.

A 52-year-old man was admitted with anemia and slight fever, which he had for the last 2 months. He had undergone replacement of the ascending aorta for acute aortic dissection 10 years previously. Echocardiography demonstrated a flailing thin structure in the anterior wall of the ascending aorta corresponding to the proximal portion of the prosthetic graft. This abnormal echocardiographic finding led us to repeat blood cultivation. We finally detected Enterococcus facium and Staphylococcus epidermidis in his blood sample. We diagnosed this as a graft infection and prepared for surgical re-replacement of the infected graft. While he was waiting for the operation, an infectious aneurysm of a tibialis posterior artery ruptured and an emergency operation was done. Replacement of the infected ascending aorta graft was done thereafter. In surgery, 2-cm-long vegetation was found. It stuck to the graft wall near the former hole used for air removal in the first surgery. The patient recovered fully and left our hospital after 3 months of postoperative antibiotics therapy. This rare case of aortic graft infection long after the original replacement surgery suggests the importance of thorough echocardiographic investigation of prosthetic graft infection as a possible cause of fever of unknown origin.

A case of heparin-induced thrombocytopenia with sepsis and congestive heart failure--first autopsy report on Japan--.

An 84-year-old man was referred to the emergency department with severe dyspnea. Based on his physical findings, electrocardiogram, X-ray and echocardiographic findings, congestive heart failure was suspected and drip infusion of prophylactic heparin against intracardiac thrombosis was commenced together with dopamine, nitroglycerin and furosemide. Diuresis occurred and the pulmonary congestion ameliorated remarkably. Starting on the 20th hospital day, the platelet count was gradually reduced (from 256,000 to 55,000 /microl) and the fibrin degradation product concentration rose (27.6 microg/ml). However, prothrombin time was not prolonged (89%), the concentration of antithrombin III was low -normal (69%) and the fibrinogen concentration was high (650 mg/dl). Thus, heparin-induced thrombocytopenia (HIT), rather than disseminated intravascular coagulation (DIC), was suspected. Heparin was withdrawn on the 24th hospital day and replaced by nafamostat mesilate after which the platelet count was restored to 100,000 /microl. Enzyme-linked immunosorbent assay for HIT antibodies was positive. Unfortunately, the patient died from uncontrolled sepsis on the 29th hospital day. At autopsy, platelet-rich thrombi were found in the small pulmonary arteries and intestinal arteries. No evidence of DIC, such as fibrin-rich thrombosis, was observed. This is the first autopsy report of HIT in Japan.

[Differential diagnosis in GERD and cardiovascular disease].

In substantial portion of patients referred to the coronary care unit (CCU) with a tentative diagnosis of acute coronary syndrome (ACS), gastro-esophageal diseases are the origins of their symptoms such as chest pain. The differential diagnosis is difficult and the coronary angiography (CAG) and the gastro-esophageal endoscopy are often required. We recently evaluated our 100 consecutive cases that underwent CAG as ACS. Among the 100 cases, 72 had significant organic coronary artery lesions, 5 had significant coronary vasospasms proved by the acetylcholine provocation, and the other 23 showed no detectable major coronary artery abnormality. Among the last 23 cases, gastro-esophageal endoscopy revealed the gastro-esophageal diseases such as reflux esophagitis in 7 cases. Those 7 cases with gastro-esophageal diseases had less coronary risk factors than the 77 cases with the coronary artery diseases. Gastro-esophageal diseases are the major differential diagnoses of ACS, especially in those with few coronary risk factors.

["Chestnut-shaped" transient regional left ventricular hypokinesis with abnormal myocardial fatty acid metabolism, not corresponding to the coronary artery territories: a case report].

A 79-year-old female patient, who was initially suspected to have pneumonia, was admitted to the respiratory department of our hospital. She experienced chest pain on the second admission day. Electrocardiography showed ST-segment elevation in leads V3 through V6, and echocardiography revealed hypokinetic left ventricular wall motion. Therefore, myocardial infarction was suspected. She was transferred to the coronary care unit. Heart catheterization was immediately performed. Coronary angiography showed no significant stenotic lesion. Left ventriculography showed regional hypokinesis of the anterior and posterior walls near the base and normokinesis in the apex. Iodine-123-beta-methyl-p-iodophenyl-pentadecanoic acid (123-BMIPP)myocardial single photon emission computed tomography(SPECT) revealed inhomogeneous decrease in uptake, especially in the lateral wall, which did not correspond to any of the coronary artery territories. The echocardiographic asynergy was dramatically resolved after 1 week and the 123I-BMIPP SPECT finding was normal at 3 months. Although the clinical course of this patient was similar to that of tako-tsubo-like left ventricular dysfunction, the shape of her left ventricle was not typical. Left ventriculography showed hypokinesis of the anterior and posterior walls near the base and normokinesis in the apex, appearing like a chestnut rather than a tako-tsubo.

Common carotid intima-media thickness is correlated with myocardial flow reserve in patients with coronary artery disease: a useful non-invasive indicator of coronary atherosclerosis.

BACKGROUND: The common carotid intima-media thickness (IMT) is correlated with the angiographically determined coronary artery stenosis. However, their correlation is weak, which limits the clinical application of the IMT as a predictor of coronary artery stenosis. The IMT reflects diffuse early-phase atherosclerosis, whereas the angiographically determined coronary artery stenosis is a late-phase phenomenon. The latter is localized and rapidly progressive with plaque rupture and acute thrombosis. Instead of the angiographically determined coronary artery stenosis, we employed myocardial flow reserve (MFR) that reflects diffuse early-phase coronary atherosclerosis and impaired coronary vasodilatation function. We evaluated the relationship between the IMT and the MFR. METHODS: Twenty-three patients with angiographically diagnosed coronary artery disease (CAD) underwent B-mode ultrasound examination to measure their common carotid IMT and positron emission tomography (PET) with dipyridamole intervention to obtain their MFR. We also performed B-mode ultrasound examination in 21 patients with hypertension without CAD and in 15 control subjects. RESULTS: The common carotid IMT in patients with CAD was thickened (0.92+/-0.15 vs. 0.81+/-0.14 mm in patients with hypertension (P<0.05) and 0.69+/-0.13 mm in control subjects (P<0.01)). The IMT was inversely correlated with the MFR (r=0.51, P<0.01). The correlations between the MFR and most of the coronary risk factors (age, blood pressure, serum cholesterol level and triglyceride level, HbA1c level, smoking index) did not reach statistical significance. CONCLUSIONS: Thickened common carotid IMT is also an indicator of reduced MFR or early-phase coronary atherosclerosis.

[Coronary risk factor management in primary practice in Shibuya, Tokyo].

OBJECTIVES: Many large-scale clinical trials have confirmed that coronary risk factors such as hypertension, hyperlipidemia and diabetes mellitus predict a higher incidence of cardiovascular events and that control of these risk factors reduces the incidence. However, the actual management of such risk factors and the resultant improvement of the cardiovascular events in primary practice remains unclear. The Heart Care Network Shibuya, a voluntary study group of regional primary physicians, surveyed the management of coronary risk factors and the clinical outcomes. METHODS: Behavioral patterns of the coronary risk factor was investigated in the management and resultant changes of the risk factors in 209 outpatients (mean age 65.6 +/- 11.2 years) with more than one of hypertension, hyperlipidemia, diabetes mellitus or prior myocardial infarction for 1 year. RESULTS: Prescriptions of anti-hypertensive (from 1.3 +/- 0.8 to 1.4 +/- 0.8 drugs per patient) and antihyperlipidemia drugs (from 0.70 +/- 0.4 to 0.73 +/- 0.4 drugs per patient) did not significantly increase. Patient education for diet (93% to 97%, p = 0.077), exercise (69% to 81%, p < 0.05) and nonsmoking (66% to 86%, p < 0.05) significantly increased after 1 year. Blood pressure decreased from 142 +/- 16/81 +/- 10 to 138 +/- 78/78 +/- 9 mmHg (p < 0.05), serum total cholesterol level decreased from 215 +/- 39 to 203 +/- 39 mg/dl (p < 0.05). As a result, more patients attained the treatment goals recommended in the guidelines by the Japanese Society of Hypertension (34.6% to 46.8%) and Japan Atherosclerosis Society (40.2% to 49.5%), respectively. However, none of blood hemoglobin A1c level, body mass index or smokers significantly changed. CONCLUSIONS: Regional practitioners attempted to control all coronary risk factors. Hypertension and hyperlipidemia, which are relatively more dependent on the medical management, improved. In contrast, diabetes mellitus, obesity or smoking, which require life style changes, did not improve. The main issue in the risk factor management is how physicians act rather than specific actions.

Osteopenia and male-specific sudden cardiac death in mice lacking a zinc transporter gene, Znt5.

We isolated a mammalian gene whose expression transiently increased in response to intimal denudation of rabbit aorta. It was identical to a gene encoding a zinc transporter, ZNT5, reported very recently by others. Mice deficient for this gene showed poor growth and a decrease in bone density due to impairment of osteoblast maturation to osteocyte. More than 60% of male null mice died suddenly because of the bradyarrhythmias. Analysis of gene-expression profiles in murine hearts by means of an oligonucleotide microarray disclosed that a subset of genes encoding immediate-early response factors (IEGs) and heat shock proteins (HSPs) were down-regulated in Znt5-null mice. These results indicate that Znt5 protein plays an important role in maturation of osteoblasts and in maintenance of the cells involved in the cardiac conduction system, partly owing to dysregulated expression of IEGs and HSPs.