著者
ICHIDA Fukiko
出版者
社団法人日本循環器学会
雑誌
Circulation journal : official journal of the Japanese Circulation Society (ISSN:13469843)
巻号頁・発行日
vol.73, no.1, pp.19-26, 2009-01-20
参考文献数
62
被引用文献数
7 98

Left ventricular noncompaction (LVNC) is a recently defined cardiomyopathy characterized by a pattern of prominent trabecular meshwork and deep intertrabecular recesses, and is thought to be caused by arrest of normal endomyocardial morphogenesis. Although LVNC has been classified as a primary cardiomyopathy of genetic origin, its definition and diagnostic criteria are still being debated. Isolated LVNC was thought to be rare; however, heightened awareness has resulted in an increased detection of the morphological features of LVNC in routine clinical practice, especially in the adult population. Clinical manifestations are highly variable, ranging from no symptoms to disabling congestive heart failure, arrhythmias, and systemic thromboemboli. LVNC, like other forms of inherited cardiomyopathy, is genetically heterogeneous and can be inherited as an autosomal-dominant or X-linked recessive disorder. It has been linked to mutations in several genes, including LIM domain binding protein 3 (<i>ZASP</i>), &alpha;-dystrobrevin (<i>DTNA</i>), tafazzin (<i>TAZ/G4.5</i>) and those encoding sarcomeric proteins. However, the relatively small contribution of known mutations to the disease, compared with the higher proportion of familial cases suggests that other elusive genes remain to be identified. (<i>Circ J</i> 2009; <b>73:</b> 19 - 26)<br>
著者
吉田 忠正
出版者
社団法人日本循環器学会
雑誌
JAPANESE CIRCULATION JOURNAL (ISSN:00471828)
巻号頁・発行日
vol.28, no.9, pp.704-711, 1964
被引用文献数
1

Recently, the hepatic circulation in hemorrhagic shock has been investigated by HEINEMANN, BRADLEY, SELKURT, FRANK and others. However, the opinions among the authors have not yet been in agreement but rather controversial, par icularly, as to the relationship between hepatic blood flow and cardiac output. HEINEMANN reported that hepatic blood flow fell sharply following hemorrhage and then returned spontaneously the control level in absence of the restoration of blood pressure. While, the others reported that hepatic blood flow did not recovered and remained in decreased level, having the same trend in blood pressure change. It seems likely that such a controversy will be partly due to the difference of the methods employed in measuring hepatic blood flow and partly to the confusion in recognizing the stage of shock, because the sequence of respective hemodynamic phenomenon is considerably different at the period of observation in this condition. The purpose of the present paper is to classify the relationship of hepatic blood flow and cardiac output throughout the entire course of hemorrhagic shock and its bearing on the irreversibility of shock. Oxygen utilization in the splanchnic viscera was also studied. The Relationship between Hepatic Blood Flow and Cardiac Output 1) Methods : Hepatic blood flow was measured by modified Frank's method. Twenty-two dogs ranging in weight from 8 to 20 kg were used and anesthetized with intravenous pentobarbital sodium of 25 mg/kg. A polyethylene catheter was passed through a right external jugular vein into the hepatic vein and then wedged into one of hepatic lobar veins. The outer end of the catheter in the hepatic vein was fixed at the level of the vena cava and allowed to drain continuously. The rate of hepatic outflow was measured by a graduated cylinder and stop watch. The blood collected was returned by transfer to an elevated burette draining into a left external jugular vein. Heparin (3-5 mg/kg) was given intravenously during the experiments. Cardiac output was calculated from indicator dilution curves according to the STEWART-HAMILTON principle. Two different procedures were used. In one method <SUP>32</SUP>P labelled red cells were used as an indicator and injected intravenously. Blood samples were collected successively from the femoral artery. In another cases, radio-iodinated serum albumin (RISA) was injected as an indicator. Cardiac output was calculated from radio-cardiograms by using a scintillation detector at the heart region and a rate-meter. 2) Results : We could distinguish the following two stages in hemorrhagic shock in relation to hepatic blood flow and cardiac output. a) The first stage : In the early stage of shock, hepatic blood flow decreases in parallel with the changes in cardiac output. b) The second stage : After same delay from the initial bleeding, hepatic blood flow decreases progressively without marked reduction in cardiac output. Consequently, a difference or "gap" appears in percentile changes of hepatic blood flow and cardiac output. c) Effects of transfusion : Complete recovery of arterial pressure, cardiac output and hepatic blood flow can be obtained by a transfusion performed in the first stage. Whereas, the transfusion made in the second stage give rise only transient recovery of arterial pressure and cardiac output.
著者
ONODERA T. FUJIWARA Hisayoshi TANAKA Masaru Wu Der-jinn HAMASHIMA Yoshihiro KAWAI Chuichi
出版者
社団法人日本循環器学会
雑誌
Jpn Circ J (ISSN:00471828)
巻号頁・発行日
vol.50, pp.614-618, 1986
被引用文献数
1

An autopsied patient who showed typical dilated cardiomyopathy (DCM)-like features and was pathologically diagnosed with hypertrophic cardiomyopathy (HCM) is presented. The patient, a 60-year-old male at the time of death, died of intractable congestive heart failure. At autopsy the heart weighted 570g and showed marked left ventricular (LV) dilatation with a thin wall (ventricular septum/free wall of the LV=7mm/8mm). There was no evidence of significant stenosis in the extramural coronary arteries. Massive fibrosis was found in the middle and outer thirds of the ventricular septum and anterior wall of the LV (48% in the ventricular septum and 9% in the free wall of the LV). As myocytes were not present in the area with massive fibrosis, percent area of disarray was calculated excluding the area of massive fibrosis and found to be 30% in the ventricular septum. Based on the marked increase in the percent area of disarray, this case was diagnosed as HCM. The patient's 37-year-old son showed asymmetric septal hypertrophy on echocardiography (ventricular septum/posterior wall of the LV = 15mm / 11mm), marked LV hypertrophy on electrocardiography, and diffuse and marked disarray by endomyocardial biopsy. There were also LV dilatation (LV diastolic dimension =51 mm) and hypokinesis of the LV ; as a result, a diagnosis of HCM with features of DCM was made.
著者
Matsumori Akira Ohashi Naohiro Nishio Ryosuke Kakio Tadashi Hara Masatake Furukawa Yutaka Ono Koh Shioi Tetsuo Hasegawa Koji Sasayama Shigetake
出版者
社団法人日本循環器学会
雑誌
Japanese circulation journal (ISSN:00471828)
巻号頁・発行日
vol.63, no.6, pp.433-438, 1999-05-20
被引用文献数
2 23

The familial of hypertrophic cardiomyopathy(HCM)is attributed to mutations in the genes for contractile proteins, but the etiology of non-familial form remains unknown. This study was designed to examine the clinical features, histopathologic changes, and hepatitis C virus(HCV)genomes in patients with HCM associated with HCV infection. Anti-HCV antibody was present in the sera of 9 of 65 patients(13.8%)with HCM versus 2.41% in a control population of voluntary blood donors in Japan, a statistically significant difference (p<0.0001). Among these 9 patients, 6 had ace-of-spades-spaped deformities of the left ventricle with apical hypertrophy. Myocardial fibrosis was found in all patients, and mild cellular infiltration was observed in 5patients. Type 1b HCV RNA was present in the sera of 5 of the 9 patients. The copy number of HCV was 5.5×10^3-8.6×10^5 genomes/ml serum, and multiple clones of HCV were detected in the sera of each patient by an analysis of the hypervariable regions using fluorescent single-strand conformation polymorphism. Positive strands of HCV were found in the hearts of 5 patients, and negative strands in the hearts of 2 patients. A high prevalence of HCV infection was found in patients with HCM, particularly of the apical variety, suggesting that HCV is an important causal agent in the pathogenesis of the disease.
著者
TOYAMA J. HONJO HARUO OSAKA TOSHIYUKI ANNO TAKAFUMI HIRAI MAKOTO OHTA TOSHIKI KODAMA ITSUO YAMADA KAZUO
出版者
社団法人日本循環器学会
雑誌
Jpn. Circ. J. (ISSN:00471828)
巻号頁・発行日
vol.51, pp.163-171, 1987
被引用文献数
1

In order to clarify the role of Purkinje fibers in the occurrence of reperfusion arrhythmias, endocardial mapping was performed on perfused canine hearts by attaching 42 close bipolar electrodes to the endocardial surface of the left ventricular septum. Reperfusion with oxygenated Krebs-Ringer solution following 30 min of coronary occulusion induced ventricular tachycardia (VT) in 14 out of 23 preparations. These VT degenerated into ventricular fibrillation (VF) within 1 min after the reperfusion in all but 3 cases. Endocardial mapping revealed that the excitations during VT were always initiated by the Purkinje activities and that myocardial excitations were expanded in a centrifugal manner through Purkinje-muscle junctional area. Furthermore, this excitation pattern was preserved, in the early phase of VT, even though the propagation pattern was distorted. VF was always induced by reperfusion following 30 min of ischemic condition, that is, coronary perfusion with a hyperkalemic (K=10 mM), acidic (pH=6.8) and hypoxic (PO_2=20-40 mmHg) solution (4/4 cases). Elimination of hyperkalemia from the ischemic condition markedly prevented occurrence of VF (1/6 cases) during reperfusion but it did not affect occurrence of VT (4/6 cases); this implies that hyperkalemia causes the onset of VF but has less effect on the occurrence of VT. It has been separately confirmed by micro-electrode experiment, using the dissected papillary muscle of the canine right ventricle, that abnormal impulse formation during re-oxygenation was triggered in Purkinje fibers around Purkinje-muscle junction.
著者
TOCHIKUBO O. MIYAZAKI Naomichi KANEKO Yoshihiro OCHIAI Hisao
出版者
社団法人日本循環器学会
雑誌
Jpn Circ J (ISSN:00471828)
巻号頁・発行日
vol.50, pp.808-817, 1986
被引用文献数
5

For statistical analysis of 24-hour recordings of arterial pressure (AP) and heart rate (HR), it is necessary to establish a theoretical probability density function of the distributions. In the present study, 24-hour recordings of direct AP and HR were performed in 15 normotensives (NT) and 39 patients with essential hypertension (EH) by means of a new portable device with a digital memory for analyzing frequency histograms. In both NT and EH, frequency histograms of AP(systolic and diastolic AP) and HR during a 24-hour period showed bi-modal curves, whereas those made during sleep and waking produced asymmetrical patterns resembling Gamma distribution. From the AP and HR histograms made fro each subject during sleep and waking, such parameters as mean (M), standard deviation, mode(Mo), skewness (Sk), kurtosis (K) and minimum values (Mi) were calculated. The Sk and M minus Mo were positive, and K was greater than 3 in both HR and AP histograms ; the AP and HR histograms during sleep can be more correctly analyzed with Gamma distribution (mean parameter errors were less than 7.3%) than with Gaussian distribution (in this : S=0, M minus Mo=0, K=3). The Mi would accord with the location parameter of the Gamma distribution.
著者
宮崎 學
出版者
社団法人日本循環器学会
雑誌
日本循環器學誌 (ISSN:00471828)
巻号頁・発行日
vol.22, no.11, pp.822-831, 1959-02-20

The CERO_2,composite factor of the balance of supply and demand of oxygen in the brain, not only expresses the presence and intensity of cerebral anoxia objectively but also seems to be an important compensatory factor in the maintenance of the normal cerebral function. The circulation and metabolic function in the brain were regulated in two ways by the blood flow regulating mechanism in and outside of the brain and by the compensatory change in CERO_2 of the cerebral tissue itself. The metabolic disturbance of the brain appears to be evoked by the insufficient compensation of CERO_2. Taking into consideration that the results obtained by N_2O method which state that the decrease in cerebral metabolism of the aged people is a secondary phenomenon of the decrease in oxygen supply to the brain, mainly the decrease in CBF, it is supposed that CERO_2 in the aged people should be greater than normal young people. Usually an inverse correlation is assumed between CBF and CERO_2. As the CBF of the aged is mainly due to the asteriosclerosis of the brain, a certain kind of correlation is inferred between CERO_2 and cerebral arteriosclerosis.The present author, to study the correlation between CERO_2 and cerebral arteriosclerosis, has measured CERO_2 in healthy and anemic aged subjects, examined the clinical significance of CERO_2 by comparing to the results obtained by N_2O method, retinal findings, cerebral symptoms and autoptical findings, and obtained the following results.1. The increase in CERO_2 was observed in about 70% of healthy aged subjects. Such frequent cerebral anoxia in healthy older people suggests the possibility of cerebral anoxia and cerebral metabolic disturbance in the aged.2. The assumption that the difference in CERO_2 in healthy aged subjects in caused by the abnormality of cerebral hemodynamics (decrease in CBF and increase in CVR) was confirmed experimentally and by the results obtained by N_2O method.3. The fact that this increase in CVR reflects mainly the attitude of the sclerotic cerebral arteries was examined by the correlation between retinal findings, cerebral symptoms and CERO_2.4. From the above stated results it is inferred that CERO_2 may be applied clinically as a measure of the intensity of cerebral arteriosclerosis.5. However, when anemia, disturbance in pulmonary function, abnormality of cerebral oxygen supply due to other CBF diminishing factors than cerebral arteriosclerosis, or primary metabolic abnormality of the brain is present, some considerations to such abnormal conditions are required.6. In anemic aged people, when the abnormality of the cerebral circulation is intense, anemia acts facilitatory to cerebral anoxia.7. Therefore, in this case, it is supposed that the CBF estimated from CERO_2 takes a value lower than the actual level and that CVR and the intensity of arteriosclerosis take higher levels.8. The intensity of cerebral arteriosclerosis increased in parallel with CERO_2 in the autoptical findings and the hypertensive subjects who increased in CERO_2 has a tendency of cerebral apoplexy. From this results it is inferred that CERO_2 may be applied as a critical signal of cerebral apoplexy too.