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Biomedical subjects

F Camerini

Publications and source records attributed to F Camerini.

At least 19 recordsLinked to original sources

Magnetic resonance imaging in right ventricular dysplasia.

Fifteen patients with right ventricular dysplasia were investigated by T1-weighted spin- and gradient-echo pulse sequences, using a protocol that enabled both a subjective analysis of myocardial signal intensity and a quantitative/qualitative analysis of right and left ventricular function. In 8 patients, 3 investigators independently recognized abnormally hyperintense areas in the anatomic sites usually affected by the disease. In 7 of these patients, these areas showed an overlap with a-dyskinetic areas imaged by both magnetic resonance imaging (MRI) and echocardiography. In 1 patient who underwent a cardiac transplant, MRI of the explanted heart showed an excellent correlation between the distribution of the lesions and the in vivo/in vitro features. The data were compared with those from an equivalent sample of patients affected by dilated cardiomyopathy. In the latter patients, no focal hyperintensities were attributed to any anatomic sites in the right ventricule, and no focal a-dyskinetic foci were observed. Furthermore, the 2 groups of patients were significantly different in regard to dimensional and functional quantitative parameters. The results suggest that MRI is useful in integrating echocardiographic data and can be helpful in diagnosing this disease in late stages.

Adipose Tissue

Ventricular arrhythmias in dilated cardiomyopathy as an independent prognostic hallmark. Italian Multicenter Cardiomyopathy Study (SPIC) Group.

Prevalence and characteristics of ventricular arrhythmias (VA) on Holter monitoring were evaluated in 218 patients with invasively documented idiopathic dilated cardiomyopathy to clarify their relation to pump dysfunction, and their prognostic role. VA were observed in 205 patients (94%) and were high grade (ventricular pairs or tachycardia) in 130 (60%). No simple or multiform ventricular premature complexes were present in 88 patients (group 1; 41%), ventricular pairs in 63 (group 2; 32%), and ventricular tachycardia in 67 (group 3; 27%). Only echocardiographic right ventricular dimensions (p less than 0.05) and prevalence of VA during effort (8% in group 1, 15% in group 2, and 14% in group 3; p = 0.0005) differed significantly between groups. VA severity, and number of ventricular premature beats and tachycardia episodes were not correlated to right/left ventricular dimensions and pump function indexes. During a mean follow-up of 29 +/- 16 months, 27 patients died from cardiac events, and 16 received transplants. Three-year survival probability was lower in groups 2 (0.82) and 3 (0.81) than in group 1 (0.94). By Cox multivariate analysis, VA severity (p less than 0.01) was a major independent predictor of prognosis after markers of ventricular dysfunction such as left ventricular ejection fraction (p less than 0.001) and stroke work index (p less than 0.001).

Adolescent

Left ventricular involvement in right ventricular dysplasia.

Right ventricular dysplasia, a heart muscle disease of unknown cause, anatomically characterized by variable replacement of myocardial muscle with adipose or fibroadipose tissue, is usually considered a selective disorder of the right ventricle. However, concomitant left ventricular involvement has been noted in a few cases. The aim of this study was to evaluate the prevalence and characteristics of left ventricular involvement in right ventricular dysplasia, as well as possible progression of the disease. Thirty-nine patients with right ventricular dysplasia were studied by M-mode and two-dimensional echocardiography; 28 of them also underwent cardiac catheterization, and in 25 endomyocardial biopsy was performed. On first examination the left ventricle was normal in 25 patients, whereas in the remaining 14 right ventricular abnormalities were associated with left ventricular involvement, characterized by asynergic areas (12 patients) or diffuse mild hypokinesis (two patients). During follow-up (27 patients, 84.1 +/- 66.1 months) 10 patients showed worsening of right ventricular function; in nine the appearance or worsening of left ventricular abnormalities was observed. Five patients died (four in congestive heart failure and one suddenly). Results of postmortem examination (available in two patients) showed atrophy of myocells and a massive fatty and fibrous infiltration of the right ventricular wall, associated with degenerative changes and fibrosis of the left ventricle. In conclusion, right ventricular dysplasia may be associated with left ventricular involvement and the disorder appears to be progressive in some instances.

Adult

Electrocardiography of myocarditis revisited: clinical and prognostic significance of electrocardiographic changes.

To clarify the clinical and prognostic value of the ECG, an ECG review was undertaken in 45 consecutive patients with a histologic diagnosis of active myocarditis (29 men and boys and 16 women and girls; age, 36.8 +/- 15 years; idiopathic myocarditis, 39 cases). In patients (21) with symptoms of recent onset (less than or equal to 1 month) AV block and repolarization abnormalities were the prevailing ECG features at the time of admission, and a pseudoinfarction pattern (Q waves plus ST-segment elevation) frequently heralded a rapidly fatal course ("fulminant myocarditis"). Left atrial enlargement and atrial fibrillation, left ventricular hypertrophy and LBBB, which prevailed in patients who had symptoms for longer periods, corresponded to the most severe degree of left ventricular dysfunction during the initial hemodynamic and echocardiographic evaluation. The overall mortality rate after 58 +/- 24 months from the time of diagnosis was 29%. Abnormal QRS complexes and LBBB were markers of poor survival, independently of initial indexes of left and right ventricular function, both of which indicate an increased propensity for sudden cardiac death.

Adult

[Clinical and prognostic significance of echocardiographic parameters in dilated cardiomyopathy: a prospective study on 225 patients. The Italian Multicenter Study of Cardiomyopathies Group].

To assess the prognostic role of echocardiographic indexes and their relation to clinical conditions, 225 patients with dilated cardiomyopathy were studied prospectively. All cases had a normal coronary angiogram and non specific endomyocardial biopsy findings. 163 men (72.4%) and 62 women (27.6%), mean age 41.5 +/- 12.3 (range 8-61), were studied. Clinical, electrocardiographic and echocardiographic parameters, normalized for body surface area, were tested according to NYHA class and presence of segmental or diffuse wall motion abnormalities. One hundred-four patients were in NYHA class I-IIa, 94 were in class IIb-III and 27 were in class IV. Left ventricular end systolic diameter index, right ventricular end diastolic diameter index, left atrial diameter index, left ventricular fractional shortening and ejection fraction, and radius to wall thickness ratio were significantly more impaired in patients with more severe symptoms. Twenty-eight patients (13%) showed segmental wall motion abnormalities and had smaller left ventricular end systolic and left atrial diameter index and higher left ventricular fractional shortening and ejection fraction. During a mean follow up of 23 +/- 15 months (range 1-67 months), 25 patients (11.1%) died from cardiac causes and 16 (7.1%) underwent heart transplant because of refractory heart failure. Prognostic evaluation was performed separately for cardiovascular mortality alone and for cardiac events (cardiovascular mortality and heart transplantation). At Cox multivariate analysis only right ventricular end diastolic diameter index (p < 0.005) predicted cardiovascular mortality, while left atrial diameter index (p < 0.001), right ventricular end diastolic diameter index (p < 0.01) and left ventricular ejection fraction (p < 0.05) were significant independent predictors of cardiac events.

Adolescent

[Beta blocking agents in the treatment of dilated cardiomyopathy: review of the literature and clinical experience with 67 patients].

BACKGROUND: Several reports suggest that chronic beta blockade, most often with the beta 1 selective agent metoprolol, may improve hemodynamic and clinical function in patients with idiopathic dilated cardiomyopathy. However, controlled trials are limited and some studies have not shown beneficial effects in short term trials. Mechanisms of effectiveness are still debated and probably concern the capacity to avoid toxic myocardial damage by catecholamines, to induce receptor up-regulation, to contribute to the control of arrhythmias, to improve diastolic relaxation and other mechanisms. METHODS: After a revision of the literature, a preliminary clinical experience with metoprolol in dilated cardiomyopathy diagnosed according to the WHO definition is reported. Sixty-seven patients symptomatic for congestive heart failure or with complex ventricular arrhythmias associated with severe left ventricular dysfunction were submitted to test dose with metoprolol 5 mg bid for 2-7 days. All patients were completely studied, including coronary angiography and endomyocardial biopsy to exclude ischemic heart disease and active myocarditis. Four pts (6%) did not tolerate the first test dosage of metoprolol and twenty-two patients were excluded from analysis because of inadequate follow-up or because they were enrolled in an international trial. Forty-one patients underwent long-term treatment with metoprolol at a final mean dosage of 150 mg a day (range 50-200 mg) and are presently analyzed. The dosage was gradually increased during the first seven weeks. RESULTS: After 6 +/- 2 months and 12 +/- 2 months, 34 patients were stable or ameliorated (Group 1) and experienced an overall significant improvement of functional class (all pts in class I-II NYHA), of left and right ventricular ejection fraction (from 28 +/- 8.8% to 35.8 +/- 13.7% to 33.2 +/- 12.3% and from 38.6 +/- 11.8% to 42.4 +/- 5.8% to 45.2 +/- 12.2% respectively), of clinical signs of congestive heart failure, of cardiothoracic index, of left ventricular diameters and of arrhythmic pattern. Furthermore, the rate of ventricular couplets > 20/24h and of non-sustained ventricular tachycardia changed respectively from 46% and 54% to 4% and 21% at 12 +/- 2 months. None in Group 1 died nor is any waiting for heart transplantation. Eleven patients (Group 2) did not tolerate the drug acutely (4 pts) or deteriorated during the first 6 +/- 2 months (7 pts) of the treatment. In this group a worsening or an insignificant variation of all clinical and instrumental parameters was observed. During follow-up four patients of this group underwent heart transplantation (one died shortly after the operation because of infective complications), one died while waiting, two are currently waiting for heart transplantation, and three are still in heart failure (class III NYHA). No cases of sudden death occurred in any group of patients (15 pts with follow-up > 12 mo). CONCLUSIONS: Our uncontrolled study seems to confirm the beneficial effect of betablockers in a subgroup of patients with idiopathic dilated cardiomyopathy. The characterization of responders to this therapy is still undefined and will constitute the aim of future analyses.

Adolescent

Left ventricular involvement in right ventricular cardiomyopathy.

Right ventricular cardiomyopathy is generally considered a disease with isolated right ventricular involvement, although there are some case reports of concomitant abnormalities of the left ventricle. In order to evaluate the prevalence, characteristics and natural history of left ventricular involvement in this disease, 39 patients with right ventricular cardiomyopathy were studied by 2-dimensional echocardiography. At first evaluation 25 patients (group 1) showed right ventricular abnormalities and a normal left ventricle. In the remaining 14 patients (group 2) the right ventricular abnormalities were associated with left ventricular involvement (asynergic areas and/or mild diffuse hypokinesia). During the follow-up, left ventricular involvement appeared in 6 patients of group 1 and worsened in 4 patients of group 2. Five patients died. Pathological examination (available in 4 cases) showed, at the level of the right ventricle, marked atrophy of myocytes and fibro-fatty replacement, and predominant fibrosis at left ventricular level. Left ventricular involvement is thus frequent in right ventricular cardiomyopathy. At least in some cases the disease appears to be progressive.

Cardiomyopathies

[The pathogenesis of dilated cardiomyopathy: current progress].

The pathogenesis of dilated cardiomyopathy (DCM) is still unknown; however, some factors that seem to play an important role in the development of the disease have recently been identified: they are enteroviral infections, immune mechanisms and genetic factors. Enteroviral infection (particularly due to Coxsackie virus B) has long been suspected to be the cause of myocarditis and subsequent DCM. However, only recent techniques of genetic engineering have been able to demonstrate the presence of enteroviral RNA in endomyocardial biopsy of patients with DCM. The role of the viral particles contained in the myocardium is still undetermined. Changes in the immune system concerning cell-mediated and humoral immunity have been recently detected. It has been suggested that an autoimmune process could be the actual cause of DCM in some patients, rather than the consequence. The immune system is strictly related to the major histocompatibility complex. As in some autoimmune diseases, a relationship between DCM and HLA class II phenotype has been found: particularly the DR4 antigen seems to be associated with a high risk of disease. Besides immunogenetic factors, other genetic factors seem to play a role in the pathogenesis of DCM. In 6-8% of cases a familial history of cardiomyopathy has been observed. In clinical studies on familial DCM different phenotypes have been shown, suggesting that different genetic mechanisms are involved in the pathogenesis of the disease. At least two main mechanisms can be hypothesized: the transmission of "predisposing" factors or a defect in proteins essential for the cardiac muscle cell function. Viral agents, autoimmune reactions, immunogenetic and genetic factors seem to cause myocardial damage individually or with complex interactions: the research should be devoted to these topics in the future.

Antibody Formation

Quantitative texture analysis in echocardiography: application to the diagnosis of myocarditis.

Altered myocardial texture associated with inflammatory infiltration or fibrosis of the myocardium has already been described using qualitative and subjective analysis of two-dimensional echocardiograms. The aim of this work is to test whether quantitative analysis of regional image texture in two-dimensional echocardiograms would be an accurate method to identify myocarditis and myocardial fibrosis. A set of 20 two-dimensional studies with endomyocardial biopsy evaluation was examined in 13 patients. Biopsy-proven myocarditis was present in 8 studies; myocarditis and fibrosis in 4; fibrosis in 3; healing/healed myocarditis in 5. A control group of 8 normal subjects was also studied by echocardiography. After quantitative texture analysis of the first order, entropy appeared to consistently differentiate myocarditis from controls. Among second-order parameters, patients affected by myocarditis or fibrosis showed a decreased entropy and higher angular second moment versus controls. We conclude that myocarditis and fibrosis induce similar image texture alterations in ultrasonic images, with increased spatial heterogeneity of the gray level distribution, which can be differentiated from normal structures with digital image analysis techniques.

Adult

The use of associated propafenone in patients with amiodarone-resistant ventricular tachycardia.

To clarify the risk-benefit ratio involved in association of antiarrhythmic drugs, a combined therapy of amiodarone and propafenone was tested by means of continuous electrocardiographic monitoring, analysis of levels of the drug in the plasma and programmed electrical stimulation in a selected group of 10 patients who had left ventricular dysfunction and spontaneous relapses of sustained ventricular tachycardia despite treatment with amiodarone. Induction of sustained ventricular tachycardia, possible in each case during treatment with amiodarone, was suppressed after addition of propafenone in 2 patients (responders), who had the best ejection fractions of the entire group (greater than 45%). Worsening of spontaneous tachycardias developed in 4 cases during the combined therapy. These ventricular arrhythmias, although generally at a low rate, sometimes had the potential to degenerate into ventricular fibrillation and disappeared after both discontinuation of propafenone or increase of its dosage (1 patient). Of the six cases undergoing chronic combined treatment, only the responders to premature electrical stimulation were completely protected from recurrences of arrhythmia. Three cases, on the other hand, needed permanent endocardial pacing for symptomatic bradyarrhythmias. The combination of treatment with amiodarone and propafenone, although potentially useful in limiting dosages of and toxicity from amiodarone, is frequently associated with undesirable, and occasionally has severe, side-effects. The best candidates for this pharmacological association seem to be patients without severely depressed left ventricular function who have a greater probability of not presenting the inducibility of ventricular tachycardia after the addition of propafenone to the regimen for treatment.

Adult

Efficacy of continuous and intermittent transdermal treatment with nitroglycerin in effort angina pectoris: a multicentric study. The Collaborative Nitro Group.

Eighty-eight patients (84 men and 4 women; mean age 59.3 years) with stable exercise-induced angina pectoris were enrolled in this within-patient, placebo-controlled study aimed at comparing the efficacy of the continuous and intermittent (12 hour on, 12 hour off) application of transdermal nitroglycerin. Eighty-one patients completed the study. After a 1-week placebo run-in period, during which the stability of angina was assessed on a bicycle ergometer, the patients received continuous treatment (two 10 mg/24 hour patches twice daily, at 8 a.m. and 8 p.m.), intermittent treatment (two 10 mg/24 hour patches at 8 a.m. and two placebo patches at 8 p.m.) and placebo (two placebo patches twice daily, at 8 a.m. and 8 p.m.), each given for one week in a double-blind randomised sequence, according to a 3 x 3 latin-square design. A cycloergometric exercise test was performed at the end of each period of treatment, 4 and 10 hours after the application of the morning patch. In comparison with placebo, both schedules of the active treatment induced a significant increase in both the ischemic (duration of exercise to 1 mm ST segment depression) and the angina threshold (duration of exercise to mild angina) at the 4th and at the 10th hours after-dosing. A significant difference was also found between continuous and intermittent treatment at the same times of observation, in favour of the intermittent schedule. The limited number of anginal attacks recorded during placebo prevented any clinical evaluation of the treatments. This study shows that the efficacy of transdermal nitroglycerin is more pronounced when it is given following an intermittent schedule.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged

Clinical aspects and haemodynamics in the follow-up of dilated cardiomyopathy and myocarditis.

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% (1971-6/1981) 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.

Cardiomyopathy, Dilated

Clinical and pathologic study of familial dilated cardiomyopathy.

To evaluate the occurrence of familial cases of dilated cardiomyopathy (DC), 165 consecutive patients were studied. Diagnosis of myocardial disease was based on clinical, hemodynamic, bioptic, postmortem or a combination of these criteria. Twelve patients (7% of cases) showed evidence of myocardial disease in greater than or equal to 1 relative; 27 patients with myocardial disease were detected in the 12 families, but a suspected history of myocardial involvement was present in a further 16 cases. In 6 families proband and relatives were affected by DC (total 14 cases); in 1 of these families the disease began with an atrioventricular block. In 4 families the relatives showed the presence of myocarditis at the endomyocardial biopsy. In 2 families the relatives presented a right ventricular cardiomyopathy. The mode of inheritance was autosomal dominant in 7 families, recessive in 4; X-linked pattern may be hypothesized in 1. Nine patients died under the age of 45 years: 2 of sudden death, 6 of chronic heart failure and 1 of cerebral embolism. Familial transmission is not rare. Different modes of genetic transmission (autosomal dominant, recessive and X-linked) and different forms of myocardial disease suggest that familial DC may be a multifactorial disease.

Adolescent

[Peripartum cardiomyopathy: clinical, echocardiographic and biopsy aspects].

In order to characterize the features of peripartum cardiomyopathy, clinical, echocardiographic and right ventricular endomyocardial bioptic data obtained from 6 patients (pts) (age range: 34.1 +/- 5.5. yrs) who fulfilled peripartum cardiomyopathy diagnostic criteria, were analyzed. Five of these pts had clinical and non-invasive studies for a mean period of 41.6 +/- 59.6 months. All but one were multiparous. Five developed heart failure one month before delivery. Three patients had active myocarditis at endomyocardial biopsy and were treated with immunosuppressive therapy. At follow-up, 3 pts improved by two or three grades of the NYHA functional classification. All 3 showed a reduction in the left ventricular end diastolic diameter (from 3.9 +/- 0.4 to 3.4 +/- 0.3 cm/m2) and a normalization of the shortening fraction in 2 (from 15 to 36% and from 13 to 46% respectively) at echocardiography. These two patients had active myocarditis at the first endomyocardial biopsy. At follow-up, all 3 pts with active myocarditis showed histological evidence of healed myocarditis. In conclusion, peripartum cardiomyopathy is characterized by a high incidence of active myocarditis and improvement seems more frequent in this subgroup of pts.

Adult

Small vessel disease (SVD) in patients with unexplained ventricular arrhythmia and dilated congestive cardiomyopathy.

Small vessel coronary disease is not commonly found in endomyocardial biopsies (EMB) of nondiabetic patients with clinical evidence of ischemic heart disease, congestive heart failure, or ventricular arrhythmia (VA). In 166 patients studied by heart catheterization and multiple right ventricle EMB, we identified 4 patients with histologic evidence of small vessel disease and normal coronary arteriograms. Two patients were suffering from VA without an obvious cardiac cause. Of the other two, one initially showed frequent ventricular tachycardia attacks followed by congestive heart failure alone. In both patients, the cardiac chambers were dilated and the left ventricular function seriously impaired. In all four patients, the histologic examination of right EMB revealed a wall thickening of the precapillary arterioles and capillary vessels. At ultrastructural examination, an increased thickening (four or five times normal) of the subendothelial lamina and of the basement membrane of capillary pericytes was found. Degenerative changes of cardiac myocells were found in all cases.

Adult

Correlation between histomorphometric findings on endomyocardial biopsy and clinical findings in idiopathic dilated cardiomyopathy.

Multivariate analysis was used to analyze the morphometric data of endomyocardial biopsies (area, perimeter and minor diameter) of myocardial cells obtained at light microscopy by a computerized approach with 16 clinical parameters and prognosis in 52 patients with idiopathic dilated cardiomyopathy. The best morphometric parameter was "area" (R2 = 0.47). A positive correlation was found with age (p less than 0.02), interval between first symptoms and diagnosis (p less than 0.02), left ventricular end-diastolic volume (p less than 0.02), cardiac index (p less than 0.05) and echocardiographic end-diastolic diameter (p less than 0.1). A negative correlation was found with prognosis (p less than 0.02), ejection fraction (p less than 0.02), shortening fraction (p less than 0.05), echocardiographic end-systolic diameter (p less than 0.06) and mitral regurgitation presence (p less than 0.1). The parameters that provided no correlation were New York Heart Association class, left ventricular end-diastolic pressure, right atrial pressure, cardiothoracic ratio, presence or absence of heart failure, fever or alcohol intake. These findings suggest that endomyocardial biopsy may provide prognostic information and confirm clinical diagnosis.

Adult

Immunosuppressive treatment in myocarditis.

Twenty patients (13 males and seven females) with a biopsy-proven diagnosis of myocarditis underwent a period of treatment with prednisone and azathioprine. The primary objective of the study was the observation of histologic changes which occur during treatment and after treatment withdrawal. The secondary objective was the detection, if any, of changes in left ventricular ejection fraction. Multiple endomyocardial biopsies were obtained and the treatment was adjusted in order to achieve complete disappearance of the myocardial inflammation. The histologic status was improved in all patients, although complete disappearance of the signs of active disease was seen in 15 patients only. Two patients died during the observation period. A clear relationship between histologic status and immunosuppression was established in some patients (50% of all cases showed a worsening after withdrawal from the treatment). An overall improvement of the ejection fraction was observed (from 0.37 +/- 0.14 to 0.46 +/- 0.17), but a direct effect of the treatment on the recovery of ventricular function cannot be stated. In some patients, however, a direct relationship between the histological changes and the changes in ejection fraction was seen. These data suggest that treatment with prednisone and azathioprine may be beneficial in some patients with biopsy-proven myocarditis and depressed ventricular function.

Adolescent