Search

Background: Dilated cardiomyopathy, a heart muscle disease of unknown cause, is characterized by high mortality and is a major cause of cardiac transplantation. It has become, therefore, increasingly important to identify patients at higher risk. The aim of this study was to assess which of the data obtained at the time of diagnosis are the best predictors of survival., Methods and Results: One hundred forty-four patients with dilated cardiomyopathy (118 men; mean age, 39 years) were assessed clinically, noninvasively, and hemodynamically. The effect of variables derived from the evaluation on outcome (death or heart transplantation) was examined. During a mean follow-up time of 4.1 years, 68 patients (47%) died and 9 (6%) underwent heart transplantation. The 1-, 2-, and 5-year transplant-free survival rate was 79, 69, and 44%, respectively. Cox multivariate regression analysis identified three variables as independent predictors of outcome: (1) pulmonary artery systolic pressure, P = .0001; (2) left ventricular ejection fraction, P = .0013; and (3) left ventricular end-diastolic dimension, P = .007. The prognostic index was constructed from regression coefficients and parameters significant in the Cox model. The minimal prognostic index in the study group was 1.4 and the maximal was 6.0 with a corresponding 1-year survival of 98 and 18%, respectively. The validity of the prognostic index was tested in the consecutive group of 81 patients, who were followed for a mean 2.3 years. The prognostic index of the poor outcome group differed significantly from that in survivors (3.7 vs 2.9, respectively, P < .01). The sensitivity and specificity of model predictions were 68 and 52%, respectively., Conclusions: The severity of pulmonary hypertension and left ventricular dysfunction provides an independent insight into the prognosis of patients with dilated cardiomyopathy. The prognostic index is useful when assessing prognosis and may be helpful in the timing of heart transplantation.

Organ- and disease-specific cardiac autoantibodies are found in a third of dilated cardiomyopathy patients from the U.K. and Italy and represent markers of autoimmune involvement. The role of autoimmunity may vary in dilated cardiomyopathy patients from different countries due to differences in genetic susceptibility to autoimmune diseases. The aim of this study was to assess the frequency of organ-specific cardiac autoantibodies detected by immunofluorescence in a consecutive series of patients with dilated cardiomyopathy and in disease and normal control subjects from Poland. The study groups included 79 patients with idiopathic (WHO criteria) dilated cardiomyopathy, 55 patients with other cardiac disease and 60 normal subjects. Cardiac antibody tests were performed by indirect immunofluorescence on human heart; skeletal muscle was used to identify cross-reacting antibodies. The frequency of organ-specific cardiac autoantibodies was higher in patients with dilated cardiomyopathy (21/79, 27%) than in controls with other cardiac disease (1/55, 2% P < 0.001) or in normal subjects (7/60, 12% P < 0.02). Conversely, cross-reactive antibodies were detected in similar proportions in patients with dilated cardiomyopathy (5/79, 6%), disease controls (7/55, 13%) and normal subjects (6/60, 10%, P = ns). The organ-specific antibody was more common in patients with dilated cardiomyopathy with insidious onset of disease (17/34, 50%) compared to those who did not exhibit this feature (4/45, 9%, P < 0.0001). Organ- and disease-specific cardiac autoantibodies were found in 27% of Polish patients with dilated cardiomyopathy at diagnosis; this is evidence for autoimmune involvement in a subset of patients from our country, as seen in a previously reported series of Western European origin. The association of antibody status with insidious onset of symptoms is in keeping with the long latency period observed in other autoimmune disorders.

Familial occurrence of dilated cardiomyopathy is estimated by 2-20%. We present a family with dilated cardiomyopathy inherited in an autosomal dominant way. We examined 9 members of the family, most of them are asymptomatic.

Unlabelled: The clinical profile of 19 patients with dilated cardiomyopathy from 2 to 18 years old (mean age 13.4 +/- 4 years) was reviewed to detect any factors that might be predictive for their survival. Follow-up study ranged from 5 to 105 months (mean 39 +/- 33 months). All patients received digitalis + diuretics, 12 were managed with immunosuppression, 16 with antiarrhythmics. There were 12 survivors and 7 nonsurvivors: the 1-year mortality was 21.2%, the 2-years mortality was 35.8%. All deaths were within first 2 years. In 12 patients who survived 2 years, significant improvement was noticed in 9 cases. Endomyocardial biopsy was performed in 16 patients. Four of them with histological diagnosis of myocarditis survived and in 3 of them a considerable improvement was noticed. Half of 12 patients with nonspecific histological findings died (p less than 0.05). There was no significant difference between survivors and nonsurvivors in all following parameters: the incidence of severe heart failure (NYHA class III-IV) and severe ventricular arrhythmias (Lown III-IV), relative heart volume, echocardiographic LVDD, haemodynamic parameters--CI, LVEF, LVEDP, LVEDVI., Conclusions: Clinical, electrocardiographic, echocardiographic and haemodynamic data are nonpredictive for survival. The most dangerous period are the first two years of illness. In long term, improvement was noticed in half of patients.

Unlabelled: Hypertrophic cardiomyopathy is characterized by a diverse clinical and morphological spectrum. In this report we describe five patients with atypical hypertrophic cardiomyopathy and significantly dilated atria. Diagnostic difficulties are stressed. All patients underwent clinical examination, noninvasive studies including 2-D echocardiogram, cardiac catheterization. In three patients endomyocardial biopsy was taken and examined by light microscopy. All but one patient were men, ranging in age from 28 years to 40 years at initial examination. In all patients disproportion between mildly elevated or, in two cases normal filling pressures and degree of dilatation of both atria was found. Two patients C.G. and D.T. had auscultatory and roentgenographic findings of mitral stenosis. Following echocardiographic and angiocardiographic studies an increase in left and right ventricular thickness associated with dilatation of both atria was found. Endomyocardial biopsy in both cases did not show endocardial thickening or infiltrative changes. In family S, two patients W.S. and Je.S had marginal left ventricular hypertrophy. The presence of left ventricular hypertrophy in patient Ja. S and finding, after family studies, hypertrophic, obstructive cardiomyopathy in patient A.S. enabled establishing the correct diagnosis. Beside varying degree of hypertrophy in family S, mild dilation of the right ventricle and incomplete right bundle branch block were found. In two patients of family S. restrictive cardiomyopathy was found, in patient Ja. S. during cardiac catheterization at initial presentation, in patient W.S. on doppler transmitral flow velocity examination at late follow-up. Paroxysmal atrial fibrillation was the first symptom in four patients, thromboembolic event in one patient. In four patients pacemaker requirement was found. During long-term follow-up (mean 4.8 years) slowly progressive heart failure associated with further dilatation of atria is observed. Mild doses of diuretics are effective in controlling congestive symptoms., Conclusion: in hypertrophic cardiomyopathy significantly dilated atria and clinical signs of mitral stenosis can be present. The presence of myocardial hypertrophy is not necessary to diagnose hypertrophic cardiomyopathy. Familial studies can be helpful in establishing the correct diagnosis.

24 endomyocardial biopsies were performed in children aged 4-18 (x = 12.9) without any complications. The endomyocardial biopsy was performed in 12 patients with the heart failure of unknown origin (6 - restrictive heart disease, 4 - dilated cardiomyopathy with arrhythmias, 2-post-inflammatory dilated cardiomyopathy), in 5 patients with a clinical diagnosis of myocarditis, in 1 with the hypertrophic cardiomyopathy, in 2 cases of the dilated cardiomyopathy without heart failure and in 1 girl with the recurrent exudative pericarditis. Active myocarditis with fibrosis was stated in 2 cases of a restrictive heart disease, mild inflammatory state in 2 patients with a clinical diagnosis of the myocarditis and in 3 others with the dilated cardiomyopathy. All of 7 patients with the myocarditis underwent the immunosuppressive therapy. In the patient with endomyocardial fibrosis the result of left ventricular biopsy corresponded with angiocardiographic diagnosis. Non-specific changes in biopsies were stated in 2 children with the restrictive cardiomyopathy and in 5 with the dilated cardiomyopathy. Bioptic, morphologic lesions in patients with the dilated cardiomyopathy did not correlate with hemodynamic parameters of contractility. Biopsies were normal in 6 subjects. Endomyocardial biopsy influenced on making a decision of the therapy in 9 of 21 patients (42.8%). Diagnosis was verified in 7 patients (38.3%) basing on endomyocardial biopsy. In 18 of 24 subjects (75%) endomyocardial biopsy contributed to the interpretation of the disease pathology.

Late results of 6-month immunosuppressive therapy (prednisone from 1.5 mg/kg b.w. and azathioprine 2 mg/kg b.w.) simultaneously performed with a conventional treatment were analyzed in 20 patients with heart failure of unknown origin and bioptic diagnosed myocarditis. Average patients' age was 33.8 +/- 10.7 years, mean disease duration--7.8 month, mean left ventricular ejection fraction--25.9 +/- 8.9%. Follow up period was at least 24 months in all patients. 1 patient died before the end of therapy. After 6-month immunosuppressive therapy improvement was stated in 10 patients (50%)--group A, stable disease course in 3 (15%)--group B and deterioration in remaining 6 (30%)--group C. After the next 18 month conventional therapy as many as in 8 of 10 group A patients deterioration was observed, further improvement (EF increase from 17% to 43%) in 1 female patient and a stable disease course in the another female. Of 3 group B patients in 1 further improvement was observed and a stable course in 2 remaining. Of 6 group C patients 4 died, 1 underwent cardiac transplantation, 1 female patient is still alive, but does not put herself to control examinations. Early improvement after 6-month immunosuppressive treatment does not prejudge the later prognosis.