Basal thinning of the interventricular septum (IVS) and atrioventricular block (AVB) are characteristic features of cardiac sarcoidosis. Since the conduction system passes along IVS, it has been ...considered that a close connection exists between basal thinning of IVS and AVB. However, neither the incidence of cases showing basal thinning of IVS nor the relation between it and AVB has been clarified. We thus investigated to elucidate these two issues.
Thirty-five patients with cardiac sarcoidosis were selected for this study and underwent echocardiographic examination. The wall thickness of IVS was measured at a site 1 cm below the aortic valve inserted point of IVS. Thickness of this site < or = 5 mm was defined as thinning. Twelve-lead and Holter electrocardiograms were obtained to determine the presence/absence and degree of AVB.
Basal thinning of IVS was noted in 7 of the 35 patients (20%). AVB was present in 4 of these 7 (57%), and was first degree in 3 (43%) and third degree in one (14%). AVB was not present in 3 patients. Basal thinning of IVS was not apparent in 28 of the 35 patients (80%). AVB was observed in 14 of the 28 patients, 3 had first degree block, 2 had second degree block, and 9 had third degree block. AVB was not observed in 14 of the 28 patients.
These results clarified that basal thinning of IVS is not as common as previously thought in cardiac sarcoidosis, basal thinning of IVS and the presence/absence and degree of AVB are not necessarily correlated.
In patients with primary atrioventricular (AV) conduction disturbances, lesions are commonly believed to be limited to the conduction system. In the present study, myocardial lesions were analyzed ...histologically in patients with AV conduction disturbances to determine the presence of myocarditis. We studied 50 patients with second or third degree AV block in whom the cause was not clear (AVB group). Endomyocardial biopsy was performed from the right ventricle and examined by light microscopy. Tissue specimens also were obtained from 12 normal hearts at autopsy as a control group (N group). The diagnosis of myocarditis was based on the Dallas criteria. The myocyte transverse diameter was 15.4±4.1 μm in the AVB group and 11.7 ±3.1 μm in the N group (P<0.01). The fibrosis area ratio also was significantly higher in the AVB group than in the N group (10.1±6.7% vs 5.1±2.0%). The mean number of lymphocytes per 400-fold magnified field was significantly greater in the AVB group than in the N group (1.9±1.6 vs 1.3±0.4). In addition, disorganization of the cardiac myocytes was noted in 8 patients in the AVB group (16%), myocyte disarrangement in 39 patients (78%), myocytolysis in 23 patients (46%), and nuclear deformity in 21 patients (42%). Myocarditis was diagnosed in 3 of the 50 patients (6%). The present study demonstrates that myocardial lesions can be detected in a large proportion of patients with AV conduction disturbances. Furthermore, myocarditis is present in 6% of the cases.
Tetrahydrobiopterin (BH
4) is an essential cofactor of nitric oxide synthase, and GTP cyclohydrolase I (GCHI) is a rate-limiting enzyme in the biosynthesis of BH
4. The expression of inducible nitric ...oxide synthase (iNOS) was earlier demonstrated in the ventricles of patients with dilated cardiomyopathy (DCM) although that of GCHI was not clarified. The present study was designed to determine the GCHI mRNA expression as well as to confirm iNOS mRNA expression in endomyocardial biopsy specimens from patients with DCM.
Clinical details were assessed in 19 patients with DCM and in 9 control subjects. The real-time reverse transcription polymerase chain reaction (PCR) was performed on total RNA extracted from endomyocardial biopsy specimens. Glyceraldehyde 3-phosphate dehydrogenase (GAPDH) mRNA was quantified for use as an internal control.
iNOS/GAPDH for the DCM samples was 4.8-fold greater than that for the control ones (
P<0.01), whereas the GCHI/GAPDH for the DCM samples was reduced to 31.1% of the control (
P<0.05).
The increased expression of iNOS mRNA was confirmed in endomyocardial biopsy specimens from patients with DCM. The GCHI mRNA level was suppressed in these specimens.
Background: Tetrahydrobiopterin (BH4) is an essential cofactor of nitric oxide synthase, and GTP cyclohydrolase I (GCHI) is a rate-limiting enzyme in the biosynthesis of BH4. The expression of nitric ...oxide synthase was earlier demonstrated in the ventricles of patients with dilated cardiomyopathy (DCM), although that of GCHI was not clarified. The present study was designed to determine the GCHI mRNA expression in endomyocardial biopsy specimens from patients with DCM.
Methods: Clinical details were assessed in 19 patients with DCM and in 9 control subjects. Patients with arrhythmia and whose biopsy samples were histologically normal and free from organic cardiovascular findings were used as the control. The real-time reverse transcription polymerase chain reaction was performed on total RNA extracted from endomyocardial biopsy specimens. Glyceraldehyde 3-phosphate dehydrogenase (GAPDH) mRNA was quantified for use as an internal control.
Results: GCHI/GAPDH for the DCM samples was reduced to 31.1% of the control (P < 0.05). BH4 levels were not determined because of limitations of the amount of the sample. However, it is reasonable to assume that BH4 production in the DCM heart would be markedly reduced under the condition of decreased GCHI mRNA expression in the tissue.
Conclusions: In the present study, we show for the first time that the GCHI mRNA level was suppressed in endomyocardial biopsy specimens from patients with DCM.
Purpose to compare the old patients with chronic heart failure with the new patients in terms of prognosis.
Subject & Method: The clinical findings, drugs and prognosis, compared in a group of ...hospitalized with chronic heart failure over one year 1995(old group, n
=
88, age 72.1 ± 12.9 years) and another group for one year 2002(new group, n
=
150, age 67.2 ± 14.3 years).
Result: There were no significant differences in either group in the LVEF and NYHA. The drugs administered were carperitide (0 vs 84.7 %), ACE-I/ARB (37.5 vs 88.0 %) and beta-blockers (6.8 vs 64.7 %), which were given the new group at a significantly higher. The cumulative survival rate was significantly higher in the new group (P < 0.0001:Fig1).
Conclusion: The therapy with carperitide, ACEI/ARB and beta-blockers resulted in a distinct improvement in the prognosis for chronic heart failure.
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A 46-year-old man was admitted for further evaluation of exertional chest discomfort. One family member had experienced sudden death, and 2 others had died of heart failure, including 1 known to have ...had Fabry’s disease. The patient was also diagnosed with Fabry’s disease, based on reduced leukocyte a-galactosidase A activity, 2.0 nmol/mg protein/hour, as well as endomyocardial biopsy findings of marked sarcoplasmic vacuolization of cardiac muscle cells by light microscopy and lamellated “zebra bodies” in the cytoplasm shown by electron microscopy. Echocardiography disclosed marked left ventricular hypertrophy and systolic anterior motion of the mitral leaflets. On cardiac catheterization, a left ventricular peak systolic outflow gradient of 50 mm Hg was noted; this decreased to 10 mm Hg following intravenous administration of 100 mg of cibenzoline. It is imperative to recognize the existence of cases with Fabry’s disease associated with left ventricular outflow obstruction.
Apical hypertrophic cardiomyopathy has been divided into two entities: apical asymmetric septal hypertrophy (apical ASH) and apical symmetric hypertrophy (AH). The latter differs clinically from ...hypertrophic cardiomyopathy (HCM) with ASH, and it is unclear whether AH represents a distinct subtype of HCM. In the present study, the presence or absence and the extent of cardiac muscle cell disorganization, a histologic characteristic of HCM, were compared in patients with AH (n = 10) and ASH (n = 29) in whom cardiac biopsy specimens were obtained from the left ventricular apex and interventricular septum. Disorganization was graded as (1+) in only 1 patient in the AH group and (−) in the remaining 9. In contrast, in the ASH group disorganization was graded as (1+) in 15 patients, (2+) in 7, (3+) in 3, and (−) in only 4 (P < 0.0001). Thus, it was observed that in AH disorganization is virtually absent or at most limited to a very narrow area. It is concluded from a histological stand point as well that the type of apical hypertrophic cardiomyopathy showing apical symmetric hypertrophy differs from usual HCM.
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