T-lymphocyte subsets in idiopathic dilated cardiomyopathy

Idiopathic dilated cardiomyopathy (IDC) is a common clinical problem in Africa. To determine if there is a defect of immune regulation in patients with IDC, the percentage of total T-cells (OKT3 positive), helper/inducer cells (OKT4 positive) and suppressor/cytotoxic cells (OKT8 positive) were measured using monoclonal antibodies in 20 patients with IDC and in 20 age-matched normal control subjects. The percentage of helper/inducer cells was significantly higher in the IDC patients (45 +/- 2% mean +/- standard error) than in the normal subjects (33 +/- 2%) and 8 of the 20 IDC patients had a helper/suppressor cell ratio (OKT4/OKT8) higher than the normal range. Of the 8 patients with this abnormality, 7 were studied within 3 months of the onset of their illness. Results suggest that an excessive immune reaction is part of the pathogenesis of IDC in Africans.     

T-lymphocyte subsets in patients with idiopathic dilated cardiomyopathy

T-cell subsets were measured in the peripheral blood of 33 patients with heart failure from idiopathic dilated cardiomyopathy, 22 patients with heart failure from other causes, and 33 normal controls. Mean T-suppressor cell percentage was 30% in normals, 21% in patients with idiopathic dilated cardiomyopathy whose duration of symptoms was less than 1 year (P = 0.0005), and 26% in those with symptoms for greater than 1 year (P = 0.05). Similarly, percentage of T-suppressor cells in the group with heart failure from causes other than idiopathic dilated cardiomyopathy was significantly lower (23%; P = 0.005) in those with short duration of symptoms. When both heart failure groups were combined those with symptoms for less than 1 year had significantly lower T-suppressor frequencies (22%) than those with symptoms for more than 1 year (P = 0.015). Multivariate analysis identified duration of symptoms and age as the only independent predictors of T-suppressor cell frequencies. Decreased percentage of T-suppressor cells in patients with idiopathic dilated cardiomyopathy may be an epiphenomenon related to duration of heart failure. This should be taken into account in assigning an etiologic mechanism for T-suppressor cells in idiopathic dilated cardiomyopathy.     

Humoral immunity and lymphocyte subpopulations in patients with dilated cardiomyopathy

The incidence of serum organ and non-organ specific autoantibodies, the peripheral blood lymphocyte blastogenic response to phytohaemagglutinin, concanavalin A and pokeweek mitogen, and the surface markers of peripheral T and B lymphocytes were studied in 15 patients affected by coronary artery disease and in 21 patients with dilated cardiomyopathy. In the latter group there was a significantly impaired blastogenic response to concanavalin A with respect to both the normal control group and patients with coronary artery disease (P less than 0.01). The percentage of peripheral blood lymphocytes with cytotoxic/suppressor activity was also reduced in patients with dilated cardiomyopathy in comparison to normal subjects and patients with coronary artery disease (P less than 0.01 and P less than 0.05, respectively). These data may reflect an in vivo defect in suppressor cell function in patients with dilated cardiomyopathy.     

Prognostic indexes in primary dilated cardiomyopathy

The purpose of this study was to investigate, if besides the hypocontractility, which is the main finding in Primary Cardiomyopathy (PDC) there was some other mechanism in the development of heart failure and if this fact could influence in it's prognosis. We studied 13 patients with PDC in the hemodynamic cardiac laboratory from January 1982 to January 1988, these with systemic arterial hypertension. Coronary heart disease, myocarditis, primary valvular lesion, infiltrative disease, nephropathy, congenital heart disease, diabetes and alcoholism, were excluded. The control group was formed by 12 healthy subjects, which were studied for another purpose. We analyzed nine variables, including ejection fraction, peripheral vascular resistance, systolic and diastolic circumferential stress, left ventricular mass, left ventricular end diastolic and systolic volumes as well as force-velocity and force-fiber length relationship. The patients were followed up from 8 to 60 months (average 39 months). The cases with PDC were divided in two groups, "compensated" and "decompensated". The last ones with low ejection fraction and significantly increases systolic stress. We investigated which was the mechanism of compensation and decompensation through the force-velocity and force-fiber length relation. We found that compensation is associated with great increase of the after-load forces, the more end systolic volume at the end of the systole is not only controlled by the "force", but the decompensation is developed when the hypocontractility is added to the incompetence to compensate the after load. We found that the three deaths in this study had these hemodynamic characteristics, being the cause of death: the presence of heart failure in two patients and ventricular fibrillation in one.(ABSTRACT TRUNCATED AT 250 WORDS)     

Comparison of long-term outcome of alcoholic and idiopathic dilated cardiomyopathy.

AIMS: The outcome of alcoholic cardiomyopathy is thought to be better than idiopathic dilated cardiomyopathy if patients abstain from alcohol. The aim of this study was to compare the long-term clinical outcome of alcoholic and idiopathic dilated cardiomyopathy. METHODS AND RESULTS: Of 134 patients with dilated cardiomyopathy and normal coronary angiography, 50 had alcoholic cardiomyopathy; they were compared serially to 84 patients with idiopathic dilated cardiomyopathy. Left ventricular end-diastolic diameter, left ventricular ejection fraction and cardiac index, severity of ventricular arrhythmias, measurement of heart rate variability and results of signal-averaged ECG were similar in both groups. Although alcohol withdrawal was strongly recommended but observed in only 70% of patients with alcoholic cardiomyopathy, both groups had similar outcome in terms of cardiac death after follow-up treatment of 47+/-40 months. Multivariate analysis in the entire cohort demonstrated that increased pulmonary capillary wedge pressure (P=0. 003), alcoholism and lack of abstinence during follow-up (P=0.006) and decreased standard deviation of all normal-to-normal RR intervals (P=0.02) were independent predictors of cardiac death. CONCLUSION: In contrast with previous studies, patients with alcoholic cardiomyopathy did not have a better outcome than patients with idiopathic dilated cardiomyopathy. Alcoholism without abstinence was a strong predictor of cardiac death. This suggests that a more aggressive approach to alcohol cessation is needed in these patients.     

Prenalterol in primary dilated cardiomyopathy: hemodynamic and angiographic evaluation

The hemodynamic effects of an acute infusion of prenalterol(PN), a new inotropic beta-adrenoceptor agonist, have been evaluatedby cardiac catheterization in 10 patients with primary dilated(congestive) cardiomyopathy. A single dose of20µg/kg wasadministered over 5 min after basal hemodynamic and angiographicmeasurements. The administration of prenalterol caused a significantincrease in mean cardiac index, from 2.3 to 3.3 l/min/m² (P< 0.01) and mean stroke volume, from 47 to 62 ml (P <0.01) without a change in heart rate. Mean left ventricularend-diastolic pressure was reduced from 19 to 13 mm Hg (P <0.05) and left ventricular dp/dt rose from 902 to 1089 mm Hg/s(P < 0.01). Stroke work index increased from 27 to 40 gm/m²(P < 0.01) and ejection fraction from 31 to 36% (P < 0.05).Mean blood pressure did not change and the systemic vascularresistance decreased from 24 to 17 RU (P < 0.01). The favorableeffect of prenalterol on left ventricular relaxation was shownby an increase of peak negative left ventricular dp/dt from946 to 1159 mm Hg/s and by a decrease of the time constant ofleft ventricular pressure fall from 49 to 39 s. These results demonstrated a positive inotropic effect of prenalterolon patients with diffuse and severely reduced contractility.     

Results of programmed ventricular stimulation in primary dilated cardiomyopathy

Programmed ventricular stimulation (PVS) has been advocated as being capable of identifying patients with idiopathic non obstructive dilated cardiomyopathy (NOCM) and at high risk of sudden death. We have studied the results of that method in 56 patients aged from 29 to 69 years (mean 53 years) presenting with idiopathic NOCM. The patients were divided into two groups according to the presence or absence of ventricular tachycardia (VT). Group I (controls) comprised 23 patients without documented VT. Group II consisted of 33 patients with documented VT which was sustained in 5 cases. Finally, 7 patients from both groups experienced losses of consciousness. Ventricular stimulation was performed on 2 sites of the right ventricle, using 1 to 3 extrastimuli on 2 imposed cycles. It was repeated under isoprenaline on 25 occasions. PVS induced non sustained ventricular tachycardia (NSVT) in only 2 patients of group I; it reproduced the sustained ventricular tachycardia (SVT) observed in the 5 patients with spontaneous SVT. PVS was negative in 14 of the 28 patients with NSVT; it induced NSVT in 8/28 and SVT in 6/28 (including 4 with more than 280 beats/min). The isoprenaline test failed to induce SVT. 7 patients died suddenly: 3 presented with SVT and 4 had syncopes and NSVT; ventricular stimulation induced SVT in 3 of these 4 patients. It is concluded that induction of sustained VT is uncommon in NOCM, but a history of syncope should prompt a search for SVT. Patients with spontaneous symptomatic NSVT and inducible SVT must be considered at high risk of sudden death.     

Regression of dilated cardiomyopathy in a chronic alcoholic patient after abstinence from alcohol

The authors report the case of a 28 year old alcoholic who was admitted to hospital for cardiac failure in 1982 due to a dilated cardiomyopathy. The clinical and paraclinical signs disappeared after cessation of alcohol intake. Three years after abstaining from alcohol, the electrocardiogram, echocardiogram and isotopic ventriculography are normal. This case illustrates the necessity of absolute cessation of alcohol intake in patients with dilated cardiomyopathies.     

Causes and prognosis of syncope in patients with primary dilated cardiomyopathy

The causes of adverse prognosis of patients with primary dilated cardiomyopathy remain controversial. Classically, it is thought that syncope is associated with an increased risk of mortality. The aim of this study was to try and identify the causes and prognostic significance of syncope in patients with primary dilated cardiomyopathy. Sixty-five patients aged 31 to 80 with primary dilated cardiomyopathy were admitted for investigation of syncope. The average ejection fraction was 27 +/- 10%. Invasive and non-invasive investigations including complete electrophysiological investigations, were performed. Sustained monomorphic ventricular tachycardia was induced in 14 patients (21.5%), ventricular flutter or fibrillation was induced in 9 patients (14%), a supraventricular arrhythmia in 17 patients (26%), and a conduction defect alone or associated with another arrhythmia in 7 patients (11%). A pathological result of tilt testing was observed in 5 patients (8%). No cause of syncope could be demonstrated in 15 patients (23%). During follow-up (4 +/- 2 years) there was a mortality of 15% which was only correlated with the reduction in left ventricular ejection fraction. The authors conclude that there are many causes of syncope in primary dilated cardiomyopathy: ventricular arrhythmias represent only 35% of cases and do not impact on the prognosis; above all, left ventricular ejection fraction is the most important prognostic factor.     

T-lymphocyte subpopulations in children's atopic asthma

A group of children with atopic asthma (42) and the control group of normal children (30) were examined in order to determine the level of suppressor and helper T lymphocyte subpopulations in each group by using monoclonal antibodies. The asthmatic children were divided into two subgroups: one group received no therapy (30) and the other was treated with specific hyposensitization (12). Suppressor T lymphocytes were significantly lower in the subgroup of non-treated asthmatic children (p less than 0.01) and significantly higher (p less than 0.01) in the subgroup of children treated with immunotherapy. The stimulation index using mitogens was higher in the group of asthmatic children. The results suggest that the reduction and functional damage of suppressor T lymphocytes may have an influence on the pathogenesis of asthma and that immunotherapy may be beneficial in the treatment of atopic asthma in children.     

Familial dilated cardiomyopathy in patients transplanted for idiopathic dilated cardiomyopathy

OBJECTIVE: To evaluate the prevalence, clinical features, and pattern of inheritance of familial dilated cardiomyopathy (DCM) in heart transplant patients. PATIENTS AND METHOD: Patients with idiopathic DCM who had undergone heart transplantation were invited to participate. Patients with alcohol abuse were excluded. A clinical evaluation, 12-lead ECG, echocardiogram, blood tests, and DNA extraction were performed in patients and relatives. Familial DCM was defined as the presence of at least one relative with idiopathic DCM. Possible familial DCM was considered when at least one relative had left ventricular enlargement (LVE) (> 112% predicted LVEDD). RESULTS: One hundred and ninety-nine relatives of 43 families were studied. DCM was familial in 11 probands (25.6%) and possibly familial in 11 (25.6%). Fifteen relatives had DCM (7.5%), 26 (13.1%) LVE, and 5 (2.5%) hypertrophic cardiomyopathy. The pattern of inheritance was autosomal dominant in most families. Five probands (3 with familial DCM) had antecedents of consanguinity and possible recessive inheritance. Six probands (14%, 1 with familial DCM) had relatives with conduction system defects. Creatine kinase was moderately increased in 9 relatives (4.5%), 3 of them with LVE. Fifteen patients had at least moderate alcohol intake. Three of them had familial DCM (relatives without alcohol abuse) and 6 had possible familial DCM. CONCLUSIONS: The prevalence of familial DCM is high in patients who undergo heart transplant. Left ventricular enlargement, conduction system abnormalities, and elevated creatine kinase may be early markers of familial disease. Hypertrophic cardiomyopathy is present in some relatives of patients with idiopathic DCM. Familial DCM is present in patients with a previous diagnosis of alcoholic DCM.     

Peripheral T-lymphocyte subpopulations in different clinical stages of chronic HBV infection correlate with HBV load

AIM： To characterize the peripheral T-cell subpopulation profiles and their correlation with hepatitis B virus （HBV） replication in different dinical stages of chronic HBV infection.
METHODS： A total of 422 patients with chronic HBV infection were enrolled in this study. The patients were divided into three stages： immune-tolerant stage, immune active stage, and immune-inactive carrier stage. Composition of peripheral T-cell subpopulations was determined by flow cytometry. HBV markers were detected by enzyme-linked immunosorbent assay. Serum HBV DNA load was assessed by quantitative real-time poiymerase chain reaction.
RESULTS： CD8^＋ T-cells were significantly higher in patients at the immune-tolerant stage than in patients at the immune-active and -inactive carrier stages （36.87 ± 7.58 vs 34.37 ± 9.07, 36.87 ± 7.58 vs 28.09 ± 5.64, P 〈 0.001）. The peripheral blood in patients at the immune-tolerant and immune active stages contained more CD8^＋ T-cells than CD4^＋ T-cells （36.87 ± 7.58 vs 30.23 ± 6.35, 34.37 ± 9.07 vs 30.92 ± 7.40, P 〈 0.01）, whereas the peripheral blood in patients at the immune- inactive carrier stage and in normal controls contained less CD8^＋ T-cells than CD4^＋ T-cells （28.09 ± 5.64 vs 36.85 ±6.06, 24.02 ± 4.35 vs 38.94 ± 3.39, P 〈 0.01）. ANOVA linear trend test showed that CD8^＋ T-cells were significantly increased in patients with a high viral load （39.41 ± 7.36, 33.83 ± 7.50, 31.81 ± 5.95 and 26.89 ± 5.71, P 〈 0.001）, while CD4^＋ T-cells were significantly increased in patients with a low HBV DNA load （37.45 ± 6.24, 33.33 ± 5.61, 31.58 ± 6.99 and 27.56 ± 5.49, P 〈 0.001）. Nultiple regression analysis displayed that log copies of HBV DNA still maintained its highly significant coefficients for T-cell subpopulations, and was the strongest predictors for variations in CD3^＋, CD4^＋ and CD8^＋ cells and CD4^＋/CD8^＋ ratio after adjustment for age at HBV-infection, maternal HBV-infection status, presence of hepatitis B e antigen and HBV mutation.
CONCLUSION： Differences in peripheral T-cell subpopulation profiles can be found in different clinical stages of chronic HBV infection. T-cell impairment is significantly associated with HBV load.     

Right ventricular dilated cardiomyopathy associated with primary biliary cirrhosis

A case of right ventricular dilated cardiomyopathy associatedwith primary biliary cirrhosis is described. The patient wasa middle aged woman, who initially complained of fatigue anditching. The diagnosis of primary biliary cirrhosis was madebased on clinical, biochemical and histological evidence ofthe disease. Seven years later severe right-sided heart failuredeveloped. The diagnosis of right ventricular dilated cardiomyopathywas made based on echocardiographic and angio graphic evidenceof a globally dilated and poorly contracting right ventricle.Left ventricular function was within normal limits. Autoimmuneserology screening at this time revealed the presence of organ-speccardiac antibody (titre 1/20) and of antinuclear antibody (titre1/80) by indirect immunofluorescence. There were no findingsof mitochondrial antibody or other non-organ specific or organ-specificantibodies. Overall, this assessment demonstrates autoimmunityin both hepatic and heart muscle disease in a patient with primarybiliary cirrhosis and right ventricular dilated cardiomyopathy.     

Differentiation between primary dilated cardiomyopathy and ischemic cardiomyopathy based on right ventricular performance.

The differentiation of primary dilated cardiomyopathy from ischemic cardiomyopathy, though important, is difficult clinically and may require coronary angiography or metabolic imaging. Both patient groups have severe left ventricular dysfunction and severe wall motion abnormality. This study examined the differences in right ventricular performance between the two groups. There were 90 patients with a left ventricular ejection fraction less than 30% who had coronary angiography and multigated radionuclide angiography (MUGA). Of these, 69 had ischemic cardiomyopathy and 21 had primary cardiomyopathy. The left ventricular ejection fraction was similar; 22 +/- 6% in ischemic cardiomyopathy and 21 +/- 6% in primary cardiomyopathy. However, the right ventricular ejection fraction was higher in ischemic cardiomyopathy (38 +/- 16% versus 29 +/- 12%, p less than 0.01). There were 59 patients with right ventricular ejection fraction greater than or equal to 30%, of whom 50 patients (85%) had ischemic cardiomyopathy. The left ventricular and right ventricular volumes were determined by a count-based method. The right ventricular end-diastolic volume/left ventricular end-diastolic volume ratio was 0.57 in ischemic cardiomyopathy and 1.07 in primary cardiomyopathy (p less than 0.05). Thus assessment of right ventricular function may help differentiate primary from ischemic cardiomyopathy; a preserved right ventricular performance is highly suggestive of ischemic cardiomyopathy.