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An original case study of a myocardial involvement in a patient with adult Still's disease is presented. The fibrinoid necrosis appearance of a myocardial vessel is an unusual finding at histology.

Plasma noradrenaline is little used in evaluating the prognosis of cardiac failure because of the theoretical necessity of interrupting treatment for a few days before blood sampling. The present study reevaluated the prognostic value of this parameter with blood sampling performed during treatment and then 48 hours after withdrawal of treatment in 192 patients with chronic stable cardiac failure at an advanced stage (64% of patients in Classes III or IV with an average ejection fraction of 28.5 +/- 13.5%). During follow-up (average 43 months) there were 51 deaths and 17 transplants. None of the patients were lost to follow-up. Univariate analysis of 52 variable observers during the initial phase of evaluation found in decreasing order of predictive value for death plasma noradrenaline levels before and after withdrawal of treatment for 48 hours. Serum sodium, age, systolic mean and diastolic pulmonary artery pressures. In multivariate analysis: noradrenaline with or without withdrawal of treatment, hyponatraemia and systolic pulmonary artery pressure. Actuarial survival curves distinguished the following parameters: noradrenaline levels became predictive at concentrations of over 210 pg/mL and there was a significant difference in survival with respect to 4 levels of serum noradrenaline (with or without treatment)300 pg/mL, 300 to 600 pg/mL and900 pg/mL. This serum noradrenaline measured without withdrawal of treatment (especially angiotensin converting enzyme inhibitors) is a powerful predictor of mortality, carrying a progressively poorer prognosis as the concentration increases.

The history of the treatment of heart failure may be divided into three stages, the consequences of different conceptions of the physiopathology of the disease, with diuretics to counteract salt and water retention, vasodilators to improve conditions of cardiac load, angiotensin converting enzyme inhibitors to limit the effect of neurotumoral and sympathetic activation. There are two main reasons for using calcium antagonist in heart failure, the first being arterial vasodilatation leading to improve systolic function and the second being the beneficial effect on ventricular relaxation. However, their use has been controversial because of the results obtained with the first generation of these drugs. New molecules derived from dihydropyridine have been developed. Clinical trials with these second generation calcium antagonists are analysed.

The influence of classical atherosclerosis risk factors (male sex, smoking, hypercholesterolemia, diabetes, overweight and hypertension on peri-hospital mortality and morbidity were studied in 83 patients (68 men and 15 women) aged 63 +/- 13 years and undergoing peripheral arterial surgery. None of these factors was statistically correlated with peri-operative complications with the exception of hypertension but the correlation was slight (0.5 less than p less than 0.10). In contrast, there was a powerful statistical (p less than 0.0003) link between age and morbidity and mortality associated with this type of surgery.

Multicentric reticulohistiocytosis is a rare disorder of the skin and joints that possesses distinctive histologic findings. A case is reported with typical features, and ulceration, an unusual outcome of "skin" lesions.

It has been hypothesized that dilated cardiomyopathy (DCM) is of dysimmune origin. Conventional immunological studies have provided no evidence that a primary disregulation of immune mechanisms is involved. In the present study, the possibility of an individual predisposition to DCM resting on a preferential distribution of HLA system antigens has been investigated. Typing of the HLA system antigens A and B was performed in a group of 38 DCM patients who were heavy drinkers. The results were compared with those obtained in: (a) 57 alcoholic patients without cardiopathy, and (b) a population of 306 healthy subjects. All subjects were caucasians. Compared with alcoholic patients without cardiac disease, DCM patients had a prevalence of B8 allele. The relative risk of developing DCM was 2.83 in the presence of the B8 antigen. This result suggests a genetic predisposition to DCM: the B8 allele, prevalent among our patients, is associated with the phenotype of numerous autoimmune diseases. This study therefore supports the theory that DCM is of dysimmune origin, but this must be confirmed by further investigations conducted on a larger number of cases.

Forty-nine diabetic patients (26 type I and 23 type II), free of cardiovascular disease symptoms, were compared with 32 controls in a noninvasive study of left ventricular (LV) function. Absence of ischemic cardiopathy was confirmed by routine investigations and an exercise electrocardiogram using 12 leads with a thallium-201 myocardial scintigraphy. Diabetic patients had (1) a significantly prolonged mean isovolumetric relaxation time (IVRT) assessed by M-mode echocardiography and phonomechanography; (2) a significantly reduced E-F slope; (3) an increased mean Weissler index (pre-ejection period/LV ejection time). The IVRT and E-F slope abnormalities reflect increased myocardial stiffness and impaired LV compliance. The increased Weissler index reflects impaired myocardial contractility. These abnormalities were not related to sex, age, duration of diabetes or to the presence or extent of complications. No significant difference was found between diabetic patients and controls for mean diastolic and systolic LV diameters, thickness of the posterior wall or of the interventricular septum, assessed by echocardiography, or for the ejection fraction, determined by radionuclide angiocardiography. Finally, more than half of the patients with a frankly abnormal IVRT, Weissler index and E-F slope had had diabetes for less than 5 years, some even less than 2 years, without complications. These data show: (1) evidence of LV dysfunction specific to diabetes and unrelated to ischemic cardiopathy and hypertension; (2) the possible involvement of a metabolic factor in this early asymptomatic LV abnormality rather than microangiopathy.

Striated skeletal muscle from 20 patients with primary cardiomyopathies were examined histologically by light microscopy. The average age of the patients was 51 years (range 34-71 yr). Seventeen of them (16 men, 1 woman) had dilated cardiomyopathy, 9 of which were associated with chronic alcoholism, and 3 men had hypertrophic cardiomyopathy. All underwent routine peripheral muscle biopsies, without clinical or biological signs of neuromuscular involvement. Minor anomalies were observed in the dilated cardiomyopathy patients: 4 in chronic alcoholics and 1 in a diabetic patient. All 3 biopsies were normal in the hypertrophic cardiomyopathy group. Comparison of these results and those reported in the literature showed that our limited findings were not only due to the study protocol and that the histological lesions were not specific to cardiomyopathy.

A case of acquired immunodeficiency syndrome associated with myocarditis is reported. The myocarditis was peculiar in that it heralded the syndrome and followed a subacute course. It was most probably of infectious origin, although no pathogen has positively been identified; it may have been caused by a cytomegalovirus or even by the human immunodeficiency virus itself on a background of genetic predisposition. This case shows that while cardiologists are concerned with cardiac disorders occurring during AIDS, they must also consider the possibility of AIDS when confronted with a myocarditis.

In order to test the hypothesis of the role of a suppressor/cytotoxic T lymphocyte deficit in the pathogenesis of dilated cardiomyopathies (DCM), 20 patients (11 alcoholic-A; 9 primary-P) were compared with 24 normal controls (N) and 10 patients with chronic cardiac failure (CCF). The percentage of OKT 3, a global assessment of the T lymphocytes, did not differ significantly between the groups. The percentage of OKT 4 (helper T lymphocytes) was significantly lower in DCM (43 +/- 8.1 p. 100) compared to N (51.92 +/- 8.1 p. 100), p less than 0.001. The percentage of OKT 4 was also lower in CCF (45.3 +/- 3.91 p. 100) compared to N (p less than 0.05). There was a very significant decrease in the percentage of OKT 8 (suppressor/cytotoxic T lymphocytes) in DCM (17.23 +/- 4.78 p. 100) compared to N (26.42 +/- 5.72 p. 100) (p less than 10(-8)). A reduction of OKT 8 was also observed in CCF compared to N (p less than 0.05). The ratio of OKT 4/OKT 8 was significantly higher in DCM (2.7 +/- 0.97) compared to N (2.08 +/- 0.6) (p less than 0.05). This difference was not observed in CCF (2.19 +/- 0.48). There were no differences between DCM A and P. These results indicate that chronic cardiac failure is associated with an equal reduction in the percentage of OKT 4 and OKT 8 lymphocytes. Dilated cardiomyopathy is associated with a large reduction in the OKT 4 and especially in the of OKT 8 with a statistically significant increase in the OKT 4/OKT 8 ratio. Although chronic cardiac failure seems to affect lymphocytes, these results are compatible with a deficit of suppressor/cytotoxic T lymphocytes in dilated cardiomyopathies.

Between November, 1978 and August, 1983, 22 patients aged from 28 to 65 years with idiopathic dilated cardiomyopathy diagnosed on the basis of clinical and haemodynamic criteria were investigated. All these patients, who consumed alcohol in excessive amounts, were followed up for 1 to 58 months. The overall mortality rate was 10 p. 100 at 4 months and 25 p. 100 at 58 months. Six patients were clinically improved with reduction of heart size (group A); 10 patients showed deterioration with 3 deaths (group C) and 6 patients followed an intermediate course (group B). When the characteristics of the groups were evaluated, it was found that compared to group C patients those in groups A and B had a lower cardiothoracic ratio: 0.56 +/- 0.04 (B) vs 0.64 +/- 0.06 (C) (P less than 0.02), a lower indexed end-diastolic diameter and systolic diameter on TM echo: 3.20 +/- 0.50 cm/m2 (A) vs 4.13 +/- 0.39 cm/m2 (C) (P less than 0.02), and 2.72 +/- 0.37 cm/m2 (A) vs 3.57 +/- 0.47 cm/m2 (C) (P less than 0.02) respectively, and a lower indexed end-diastolic volume as evaluated by angiography: 121 +/- 61 ml/m2 (A) vs 202 +/- 65 ml/m2 (C) (P less than 0.06). Dilated cardiomyopathy associated with excessive alcohol consumption has a better prognosis when the patients stop drinking and when their heart dilatation is mild to moderate.

24-hour continuous electrocardiographic ECG monitoring and standardized tests were performed to detect cardiac autonomic neuropathy in diabetic patients. Thirty-eight patients, with a mean duration of diabetes of 10 years, twenty-five IDDM and thirteen NIDDM, and thirty-two controls, with no illness or treatment which could alter the heart rate (HR), were studied. Five standardized tests were performed. Three tests investigated parasympathetic function: variations of HR during Valsalva manoeuvre, deep breathing and standing. The other two tests investigated sympathetic function: detection of orthostatic hypotension and blood pressure response to sustained handgrip. Parasympathetic HR control was impaired in twenty-nine patients, together with impaired sympathetic cardiovascular control in seven. According to the 6 indices studied, 24-hour ECG monitoring detected abnormalities in only eight patients. Mean minimum 24-hour HR and mean sleeping HR were elevated in the group of patients whose five standardized tests were normal and in the group of patients with impairment of both parasympathetic and sympathetic cardiovascular control, but not in the group of patients with only impaired parasympathetic HR control. This study suggests that 24-hour ECG monitoring is a less sensitive test of cardiac autonomic neuropathy than standardized tests. Moreover, it shows HR abnormalities that are not specific to cardiac autonomic neuropathy.

Apparently primary dilated cardiomyopathy poses a difficult aetiological problem. There is epidemiological, biological and histological evidence that some of them are the long-term results of acute myocarditis especially due to Coksackie virus. In order to establish whether cytomegalovirus infection could also have the same consequences 67 cases of dilated cardiomyopathy were reviewed to look for inflammatory changes and CMV serology. The results indicate a possible link between acute CMV myocarditis and dilated cardiomyopathy (7 p. 100 of cases) but this is difficult to prove in the chronic stage of the disease.

The case of a 71 year old woman with an idiopathic hypereosinophilic syndrome is reported. Bidimensional ultrasonography was in favor of endomyocardial involvement. This case was original as nephrotic syndrome developed, due to membranous glomerulopathy which was complicated with renal vein thrombosis. Both eosinophilia and nephrotic syndrome greatly improved with corticosteroid treatment. Nonetheless, the patient died suddenly after 2 years, and this demise is in keeping with the overall poor prognosis of idiopathic eosinophilic syndrome.

The authors report 2 observations of auriculoventricular blockage developed during a mediastino-pulmonary sarcoidosis, requiring a heart pacer in the first case and corticosteroid treatment in the second. The frequency of the disorders of intracardiac conduction was evaluated by a retrospective study of 412 cases of mediastino-pulmonary sarcoidosis. Seventy two ECG were considered abnormal, a frequency of 17.2%. The disorders of auriculo-ventricular and intra-ventricular conduction represented a third of cases. What is known on the risk of lethal evolution of cardiac sarcoidosis in which those abnormalities are significant, justifies an electrophysiological investigation. The indications for a definitive cardiac stimulation and corticosteroid treatment are debated. On this final point, it seemed necessary to plan a prospective study to confirm the merits of the treatment and to define its modalities.

In order to detect evidence of cardiac autonomic neuropathy, 24-hour continuous electrocardiographic monitoring was carried out on fifty-one diabetic patients (thirty-one IDD, twenty NIDD) and twenty-two healthy controls taking no treatment which could alter the heart rate. In the diabetic patients the minimum 24-hour heart-rate and the mean sleeping heart rate were significantly higher, and the maximum 24-hour heart rate and the ratio [(maximum-minimum heart rates)/minimum heart rate] were significantly lower. Evidence in one diabetic of cardiac autonomic neuropathy was found only as the difference (maximum-minimum heart rates). This index was found to be below 38/min (mean-2 SD of the controls) in seven diabetics, but only one of the nine diabetics with signs of autonomic neuropathy had this abnormal index. The mean values for the minimum and the mean sleeping heart rates were high in the IDD with or without signs of peripheral neuropathy and without signs of autonomic neuropathy but were not high in IDD with signs of autonomic neuropathy. These findings suggest the presence of cardiac autonomic neuropathy in diabetics. However, the possibility of insulin-induced tachycardia should be considered this tachycardia is probably related to stimulation of the sympathetic nervous system, which would explain the absence of abnormalities in IDD with autonomic neuropathy.

A forty year old man with malignant pheochromocytoma of Zuckerkandl's body is reported. The case history is a severe hypertension with acute episodes, tachycardia and psychiatric disorders. The tumor extra adrenal site was got by abdominal scanning and its malignant type was demonstrated by pathological study and metastasis of lymph nodes occurring 4 months after surgery. The surgical treatment was associated to antimitotic and alpha blocking therapy. Clinical and biological status is still good after a twelve months follow-up.