Search

Dilated cardiomyopathy is a heart muscle disease of unknown aetiology, characterized by left ventricular dilatation and impaired systolic function. Data on the incidence and prevalence of the disease is ambiguous, due to geographic variations, patient selection and the diagnostic criteria adopted.Methods All the post-mortem and clinical cases observed in a consecutive series of 5252 patients resident in Trieste during the period November 1987–November 1989 were studied.Results Incidence of the disease discovered at autopsy was estimated at 4·5/100 000/year (24 cases), while clinical incidence in the same period was 2·45/100 000/year (13 cases). This is a total incidence of 6·95/100 000 new cases a year. A possible family history of heart muscle disease was found in three patients (12·5%). In 15 patients (62·5%) deaths were due to cardiological complications. Endocardial thickening (P=0·03), fatty infiltration (P=0·01) and arterial involvement (P=0·04) were found more frequently in older patients (>65 years).Conclusions The study confirms that dilated cardiomyopathy in Europe has a higher incidence than previously suggested and emphasizes the need for greater diagnostic sensitivity, particularly since pharmacological treatment is now so effective. [ABSTRACT FROM PUBLISHER]

The application of molecular genetics in cardiology is currently producing important results in the study of the pathogenetic mechanisms underlying cardiomyopathies. Recent clinical surveys have indicated that genetic factors play a major pathogenetic role in idiopathic dilated cardiomyopathy (IDC). Familial IDC is frequent (20-30%) and is probably a heterogeneous entity, as suggested by the clinical variability and the different pattern of inheritance in the affected families. Molecular genetic studies have demonstrated the existence of heterogeneity also at the genetic level. In a series of families with X-linked IDC, the disease gene has been identified as the dystrophin gene. In familial right ventricular cardiomyopathy (or right ventricular dysplasia), a new nosological entity characterized by isolated right ventricular involvement that can mimic IDC, the disease gene has been localized in the long arm of chromosome 14. In families with matrilineal transmission, the cardiomyopathy could be linked to mitochondrial DNA alterations. Autosomal dominant familial IDC, considered to be the most frequent form, is currently under active investigation. Our preliminary data have excluded a large series of candidate genes, among which are the cardiac f3-myosin heavy chain and several other genes encoding for cardiac contractile proteins, genes of the HLA region, and about 60 genes involved in the immune regulation. [ABSTRACT FROM PUBLISHER]

The aetiology of right ventricular dysplasia/cardiomyopathy is presently unknown. A genetic background has been suggested, but myocarditis may play a part in its pathogenesis. Two familial cases of right ventricular dysplasia, one of whom had also a diagnosis of myocarditis, are reported. Both patients presented with ventricular arrhythmias. The father subsequently had a "flu-like" syndrome, heart failure, and biventricular dysfunction; "active" myocarditis was found at endomyocardial biopsy. Then the patient died suddenly. The daughter developed progressive biventricular dysfunction; then she was resuscitated from a cardiac arrest, and subsequently died suddenly. In both patients necropsy showed severe right ventricular atrophy and fibro-adipose substitution, associated with biventricular fibrosis. Inflammatory infiltration was also present in the first patient. This study shows the association of right ventricular dysplasia and myocarditis in the same family. These cases may represent a link between inherited and acquired ("inflammatory") forms of the disease. [ABSTRACT FROM AUTHOR]

Sixty-five patients with dilated cardiomyopathy underwent 24 hour electrocardiographic monitoring: 62 (95.4%) showed ventricular arrhythmias and 52 (80%) complex ventricular arrhythmias (multiform ventricular extrasystoles, paired ventricular extrasystoles and ventricular tachycardia). Complex ventricular arrhythmias correlated significantly with some haemodynamic indices of ventricular dysfunction: patients with multiform and paired ventricular extrasystoles and with ventricular tachycardia had lower values of ejection fraction (31.9 +/- 11.8%, P = 0.002) and of cardiac index (2.9 +/- 0.7 litres/min/m2, P = 0.029) than the others (41.1 11.1% and 3.5 +/- 0.9 litres/min/m2 respectively). Patients were followed for a period of 30 +/- 18 months (20 days to 64 months). During follow-up 19 died and mortality was higher among patients with multiform and paired ventricular extrasystoles and/or ventricular tachycardia. Complex ventricular arrhythmias are frequent in dilated cardiomyopathy: ventricular tachycardia and multiform and paired ventricular extrasystoles seem to be related to a more depressed ventricular function and to a poor prognosis. The importance of antiarrhythmic treatment in these patients has still to be evaluated. [ABSTRACT FROM PUBLISHER]

Idiopathic dilated cardiomyopathy (IDC) is a heart disease of unknown aetiology characterised by impaired ventricular function usually associated with dilatation of the cardiac chambers. In order to test the hypothesis of an immunological cause for the disease at the genetic level, we performed linkage analysis between the putative disease locus and some of the potential candidate genes involved in the immune response or coding for the targets for autoantibodies in a large multigeneration family (63 members) from southern Italy with autosomal dominant transmission of the disease. Twenty-nine polymorphic markers on 18 different chromosomal locations were investigated, including markers linked to the genes coding for the HLA antigens, the immunoglobulin heavy and light chains, the receptors for the immunoglobulin Fc fragments, the subunits of the T cell receptor and the associated CD3, CD4, CD8, and CD45 antigens, interleukins 1, 3, 4, 5, 6, 9, and 11, the interleukin 1 and 2 receptors, and the genes coding for the beta 1 adrenoreceptor, the adenine nucleotide translocator-1, and the cardiac alpha and beta myosin heavy chains. No evidence for genetic linkage to IDC was found at any of these candidate loci. These results indicate that the still unidentified IDC gene maps outside several loci involved in the regulation of immune reactivity. [ABSTRACT FROM PUBLISHER]

Chest thump is a simple method of treatment of some paroxysmal arrhythmias. Its therapeutic efficacy, electrophysiological bases and clinical utility have been studied in 17 patients during 45 episodes of ventricular tachycardia (VT). Thumping the precordium interrupted the VT in 22 episodes. Three types of interruption of VT have been observed: (1) In 15 episodes, single ventricular premature beats induced by the blow, occurring randomly in the cycle, stopped the arrhythmia; (2) In 5 episodes, a run of premature beats, induced by a rapid succession of blows, interrupted the tachycardia; (3) In 2 episodes, chest thump caused a short period of asystole followed by sinus rhythm. Chest thump is an antiarrhythmic treatment of definite clinical utility. The complications are rare, although there is a possibility of ventricular fibrillation. Therefore, it should be performed only under careful supervision. [ABSTRACT FROM AUTHOR]

BACKGROUND--Right ventricular dysplasia is a heart muscle disease of unknown cause that is often familial and is anatomically characterised by adipose or fibroadipose infiltration of the right ventricular myocardium. It is generally regarded as a selective disorder of the right ventricle. AIM--To investigate the prevalence and characteristics of left ventricular involvement in two families in which at least one member had right ventricular dysplasia confirmed at necropsy. METHODS AND RESULTS--Eight patients were found to be affected by right ventricular dysplasia. In three of them this was confirmed at necropsy. Echocardiography or angiography or both showed left ventricular involvement in seven. This ranged from localised wall motion abnormalities to moderate or severe left ventricular dysfunction. The disease was progressive in four cases. At necropsy the left ventricular myocardium showed predominant fibrosis and degenerative changes of the myocardial cells. There were areas of myocardial thinning with fatty infiltration at the apex in two patients. CONCLUSIONS--Familial right ventricular dysplasia can be a progressive disorder that affects the left ventricle. Advanced disease may be clinically confused with dilated cardiomyopathy. [ABSTRACT FROM PUBLISHER]

The results of the medical treatment of primary pulmonary hypertension are usually very poor. A case of severe primary pulmonary hypertension in chronic heart failure was treated with the calcium antagonist nifedipine, a powerful vasodilator which acts directly on vascular smooth muscle. Nifedipine, administered in a dose of 20 mg sublingually, caused a pronounced fall in pulmonary and systemic vascular resistances (54 and 49%, respectively) and a rise in cardiac output (90.3%). Improvement was maintained over a three-month period. These data suggest that nifedipine can be used in the treatment of this condition. [ABSTRACT FROM PUBLISHER]

Many studies on the natural history of dilated cardiomyopathy show high probability of death or of cardiac transplantation in a large percentage of patients. These studies have several methodological limitations. Our prospective study, carried out from 1971, and which evaluated 120 patients, showed improved survival in more recent years. Survival 3 years after diagnosis changed from 30% (1971–67sol;1981) to 88·4% (7/1986–1/1989).Thirty patients were investigated by haemodynamic exercise test to assess their haemodynamic behaviour during exercise, to evaluate the effects of pharmacological treatment and to define parameters of prognostic value. Different haemodynamic behaviours were observed. Haemodynamic investigation during exercise is useful to assess the effect of treatment and may have prognostic value.Myocarditis presents a spectrum of clinical symptoms and echocardiographic abnormalities. In patients with congestive heart failure left ventricular dysfunction is common. Patients with atrioventricular block or chest pain usually have good left ventricular function. During follow-up, improvement is possible but persistent left ventricular dysfunction is associated with a high mortality rate. When left ventricular function is good at presentation and does not deteriorate during follow-up the prognosis is good. [ABSTRACT FROM PUBLISHER]

In order to investigate the anatomic substrate of ‘idiopathic’ ventricular tachycardia (VT) 10 patients with chronic recurrent VT and no apparent sign of heart disease underwent an echocardiographic, haemodynamic and histologic study (5 males, 5 females: mean age=40±11 years). In the patients with a left bundle branch block morphology of VT (7 cases), four showed findings compatible with an arrhythmogenic right ventricular dysplasia or a right ventricular cardiomyopathy. In the other three all examinations were normal with the exception of endomyocardial biopsy, which showed slight non specific changes in two. Of the remaining 3 cases (characterized by a right bundle branch block morphology of VT or by the presence of polymorphic VT) one had histologic evidence of myocarditis while another developed dilated cardiomyopathy. Macroscopic and/or microscopic ventricular abnormalities are frequently found in patients with VT which appears idiopathic. In these cases myocardial disease is frequently progressive, despite optimal control of VT. [ABSTRACT FROM PUBLISHER]

To determine whether afterload reduction with nifedipine is effective both at rest and during stress tests (rapid atrial pacing and contrast material overload), 14 patients with chronic severe isolated aortic insufficiency (10 asymptomatic) underwent right and left cardiac catheterization. Forty-five minutes after 20 mg of nifedipine (sublingually), left ventricular end-diastolic pressure, peak aortic pressure, systemic vascular resistance and double product decreased significantly at rest, at peak paced rate, and after angiography (P values from <0.05 to <0.001). Cardiac index increased at resting heart rate (P<0.01) but was unchanged during pacing. The reduction of systemic vascular resistances was inversely correlated with its initial value (r = −0.69). After nifedipine, average regurgitant fraction did not change; however its variations were significantly correlated with those of systemic vascular resistance (r = 0.69). It is concluded that in severe aortic insufficiency, nifedipine induces an effective reduction of left ventricular pre- and afterload, accompanied by an enhanced mechanical efficiency (unchanged or increased cardiac index with lower double product), both at rest and during stress tests. [ABSTRACT FROM PUBLISHER]

Nifedipine provides a rapid and marked ventricular unloading in patients with chronic heart failure due to congestive cardiomyopathy. We postulate that the improvement of cardiac performances is related to the effect of the drug on the peripheral circulation. However, further studies are needed to evaluate whether the acute hemodynamic effect will persist during long-term treatment. [ABSTRACT FROM PUBLISHER]

The changes in the ECG were studied during 357 angiocardiographies with injections in tile left atrium, left ventricle, or the aorta in 195 patients. In 238 Instances Urografin 76% was used and in 119, Isopaque 60%. The ECG changes were always transient and with six exceptions slight. In four of these cases tile changes were caused by obvious mechanical factors and only in two were then caused by the constrast medium (Urografin 76%). Slight and transient S-T and T changes were seen somewhat more frequently after Isopaque 60% than after Urografin 76%. However, in a comparative study in 20 patients where identical injections of the two media were performed this difference between the two contrast agents was not found. [ABSTRACT FROM AUTHOR]

The upstroke time of the pulmonary artery curve in children with various types of pulmonary stenosis was calculated and compared with that from children with innocent murmurs. The upstroke time was normal or low normal in cases with infundibular stenosis or combined infundibular and valvular stenosis and prolonged in valvular stenosis. [ABSTRACT FROM PUBLISHER]

Active myocarditis was diagnosed in 17 out of 295 patients who underwent endomyocardial biopsy. To evaluate the effects of prednisone (50 mg m−2 day−1, then tapered) and azathioprine (75 mg m−2 day−1), patients were controlled with endomyocurdial biopsy and ejection fraction measurement during the minimum treatment period of 6 months. After 2 months of treatment, persistent myocarditis was present in only three patients while healing or healed myocarditis was present in eight and six patients, respectively. Continuous treatment was associated with the disappearance of the histological signs of myocarditis in 14 patients. The subsequent reduction or interruption of the treatment was associated with a worsening histology in ten patients. Ejection fraction estimated at the end of the treatment period (six patients) or at the latest follow-up (11 patients) improved from 37.8 ± 14% to 44.8 ± 17.6%, P = 0.031. Thus, immunoSuppressive treatment was associated with a reduction of the histological signs of myocarditis in most patients. A concomitant, significant improvement of ejection fraction was observed. [ABSTRACT FROM PUBLISHER]

The characteristics of left ventricular (LV) involvement in 31 patients with right ventricular cardiomyopathy (RVCMP) were studied by two-dimensional echocardiography (2D echo). Two groups were identified: in group 1 (19 patients) the LV appeared normal initially, but became abnormal in four patients during follow-up. LV involvement was present at diagnosis in the 12 patients of group 2; LV function worsened in one of them. In conclusion, LV involvement is frequent in RVCMP. At least in some cases the disease appears to be progressive. [ABSTRACT FROM PUBLISHER]