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PubMedDate not supplied

Cardiovascular involvement in metabolic diseases.

Cardiomyopathies are a group of disorders that result in dysfunction of the contractility of the myocardium anda decrease in cardiac function. Cardiomyopathies may be primary or idiopathic or secondary due to an underlying definable systemic disorder. Cardiomyopathies may be further defined as according to the World Health Organization (WHO) as: 1) congestive or dilated; 2) obstructive as in hypertrophic cardiomyopathy; 3) restrictive as in infiltrative disorders such as storage diseases. Two additional categories have been added that include arrhythmogenic right ventricular cardiomyopathy and unclassified cardiomyopathies. A number of metabolic disorders result in cardiomyopathy and are frequently the cause of death in these disorders.

Cardiomyopathies↗
PubMed1980

Value of radionuclide imaging techniques in assessing cardiomyopathy.

Radionuclide imaging techniques add an important dimension to the diagnosis, classification and management of myocardial disease. The gated blood pool scan provides information allowing determination of the functional type of cardiomyopathy (congestive, restrictive or hypertrophic) as well as evaluation of ventricular performance. Myocardial perfusion imaging with thallium-201 is useful in distinguishing congestive cardiomyopathy from severe coronary artery disease and also in depicting septal abnormalities in hypertrophic cardiomyopathy. Radionuclide techniques also prove useful in following progression of disease and in evaluating the efficacy of therapeutic interventions.

Cardiomyopathies↗
PubMed1996

[The restrictive syndrome in endomyocardial fibrosis and the effect of enalapril based on acute drug test data].

6 hours after the second dose (5 mg) of enalapril 9 males and 6 females (mean age 34.93 +/- 1.03 years) with endomyocardial fibrosis (EMF) underwent ECG and pulsed Doppler echocardiography to study enalapril effect on cardiohemodynamics. As shown by ventricular diastolic function and systolic flow in the pulmonary artery with estimation of mean pressure in the pulmonary artery according to Kitabatake, enalapril (renitek) affects positively cardiohemodynamics of EMF patients: improvement of ventricular diastolic function occurred in line with a significant fall in the pulmonary artery pressure. It is noted that application of Doppler echocardiography is essential for detection of restriction syndrome.

Adult↗
PubMed2004

Predictors of cardiovascular death in patients with a left ventricular restrictive filling pattern of the mitral inflow.

The survival, clinical and echocardiographic variables and the predictors of cardiovascular death were determined for a group of 168 patients (mean age 63+/-13 years; 65 females; mean left ventricular ejection fraction 32+/-10%) with restrictive filling of the left ventricle and depressed systolic function after a mean follow-up period of 2.7+/-1 years. Shorter deceleration time (DT) of the mitral inflow was the only variable significantly different between survivors and nonsurvivors (p<0.05) and the only predictor of death found by multivariate logistic regression analysis (odds ratio 2.2, 95% confidence interval 1.7-3.6). In this patient population, a DT of the early wave of the mitral inflow <140 ms identified the patients with the highest risk of cardiac death. DT is a practical echocardiographic parameter for risk stratification of patients with significant left ventricular systolic dysfunction and restrictive filling of the left ventricle.

Cardiomyopathy, Restrictive↗
PubMed1988

Demonstration of restrictive ventricular physiology by Doppler echocardiography.

In patients with restriction of cardiac filling of various origins, cardiac catheterization has been traditionally used as part of the diagnostic evaluation to verify the presence of restrictive/constrictive hemodynamics. In an attempt to determine whether this "restrictive" physiology could be demonstrated noninvasively, 14 patients who had a history, physical examination, two-dimensional echocardiogram and catheterization data compatible with a restrictive myocardial process were studied with pulsed wave Doppler ultrasound. Forty normal subjects served as a control group. The Doppler ultrasound evaluation included measurement of peak mitral and tricuspid flow velocities and flow velocity integrals, mitral and tricuspid deceleration times and central venous flow patterns during apnea and inspiration. The flow velocity recordings across the mitral and tricuspid valves in patients manifesting restriction were markedly different from those in normal subjects, showing shortened deceleration times across both valves, which indicated both an abrupt premature cessation of ventricular filling and the presence of a diastolic dip-plateau contour in ventricular pressure recordings. In addition, abnormal central venous flow velocity reversals with inspiration and diastolic mitral and tricuspid regurgitation were frequently observed, also suggesting the reduced myocardial compliance characteristic of a restrictive myocardial process.

Adult↗
PubMedDate not supplied

The relative protective effects of moderate dietary restriction versus dietary modification on spontaneous cardiomyopathy in male Sprague-Dawley rats.

The relative protective effects of modifying dietary protein, fat, fiber, and energy content vs moderate food or dietary restriction (DR) on spontaneous cardiomyopathy of Charles River male Sprague-Dawley (SD) rats was evaluated at 1 and 2 years. For 2 years, SD rats were fed Purina Rodent Chow 5002 (21.4% protein, 5.7% fat, 4.1% fiber, 3.1 kcal/g) or a modified rodent chow 5002-9 (13.6% protein, 4.6% fat, 15.7% crude fiber, 2.4 kcal/g) ad libitum (AL) or by moderate DR at approximately 65% of the caloric intake of the AL group fed the 5002 diet. Serum lipids, carcass composition, and organ weights were evaluated and hearts were qualitatively and quantitatively examined microscopically for male SD rats at 1 and 2 years. Cardiomyopathy was characterized by the colocalization of myocardial degeneration, the development of subepicardial, perivascular, subendocardial, and interstitial fibrosis, and mononuclear inflammatory cell infiltration that increased by incidence and severity in an age-dependent manner from 1 to 2 years. SD rats fed the 5002 diet AL had the greatest heart weights and the most severe cardiomyopathy, with the highest myocardial fibrotic index. These parameters were relatively decreased in the AL 5002-9 diet, the DR 5002 diet, and the DR 5002-9 diet rats at 1 and 2 years. Regardless of the type of diet fed, both AL groups had the most severe cardiomyopathy by 2 years. Moderate DR allowed isocaloric comparisons of the relative effects of modified diets on survival, obesity, and heart disease. Only slight improvements in the severity and progression of spontaneous cardiomyopathy were seen by modification of the protein, fiber, fat, and energy content of the diet if fed AL. However, moderate DR with either diet was more effective than changing the diet composition in preventing and controlling the progression of cardiomyopathy in male SD rats.

Aging↗
PubMed1984

Hypertropic cardiomyopathy and hyperthyroidism in the cat.

In a 21/2-year period, hypertrophic cardiomyopathy was found at necropsy of 23 cats that died (13 cats) or were euthanatized (10) because of problems associated with hyperthyroidism. Of these, 4 (17%) also had evidence of cardiac failure (pulmonary edema or pleural effusion). The mean body weight of the cats with hyperthyroidism and hypertrophic cardiomyopathy was significantly less (P less than 0.001) than that of clinically normal cats and cats with primary cardiomyopathy (congestive or restrictive) or excessive moderator band cardiomyopathy. In addition, the ratio of heart weight to body weight was significantly greater (P less than 0.001) in the 23 hyperthyroid cats than in the normal cats and cats with primary cardiomyopathy. Twenty (87%) of the cats had symmetric hypertrophy of the ventricular septum and left ventricular free wall, whereas the remaining 3 cats had disproportionate thickening of the ventricular septum, compared with the free wall, similar to what is found in cats with asymmetric hypertrophic cardiomyopathy. Histologic cardiac abnormalities included large, hyperchromatic nuclei, interstitial fibrosis, endocardial fibroplasia, fibrosis of the atrioventricular node, and marked disorganization of cardiac muscle cells. The study showed that hypertrophic cardiomyopathy develops in most hyperthyroid cats, some of which also develop congestive heart failure. Although the signs of heart disease in primary myocardial disease and thyrotoxic disease are similar, the characteristic signalment and clinical signs of hyperthyroidism should lead one to suspect the association of hypertrophic cardiomyopathy with the hyperthyroidism.

Animals↗
PubMed2005

Autophagy in cardiac myocyte homeostasis, aging, and pathology.

Autophagy, an intralysosomal degradation of cells' own constituents that includes macro-, micro-, and chaperone-mediated autophagy, plays an important role in the renewal of cardiac myocytes. This cell type is represented by long-lived postmitotic cells with very poor (if any) replacement through differentiation of stem cells. Macroautophagy, the most universal form of autophagy, is responsible for the degradation of various macromolecules and organelles including mitochondria and is activated in response to stress, promoting cell survival. This process is also involved in programmed cell death when injury is irreversible. Even under normal conditions, autophagy is somewhat imperfect, underlying gradual accumulation of defective mitochondria and lipofuscin granules within aging cardiac myocytes. Autophagy is involved in the most important cardiac pathologies including myocardial hypertrophy, cardiomyopathies, and ischemic heart disease, a fact that has led to increasing attention to this process.

Adaptation, Physiological↗
PubMedDate not supplied

Twenty years of progress and beckoning frontiers in cardiovascular pathology: cardiomyopathies.

In the last 20 years, with the advent of cardiac transplantation and the availability of molecular biology techniques, major advancements were achieved in the understanding of cardiomyopathies. Novel cardiomyopathies have been discovered (arrhythmogenic right ventricular, primary restrictive, and noncompacted myocardium) and added in the update of WHO classification. Myocarditis was also included with the name "inflammatory cardiomyopathy." Adenoviruses and parvoviruses were found to be frequent cardiotropic viruses in addition to enteroviruses. The extraordinary progress accomplished in molecular genetics of inherited cardiomyopathies allowed to establish hypertrophic and restrictive cardiomyopathies as sarcomeric ("force generation") diseases, dilated cardiomyopathies as cytoskeleton ("force transmission") disease, and arrhythmogenic right ventricular cardiomyopathy (ARVC) as cell junction disease. If we consider also cardiomyopathy as ion channel disease (long and short QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia), because they are diseases of the myocardium associated with electrical dysfunction, then a genomic/postgenomic classification of inherited cardiomyopathies may be put forward: cytoskeletal cardiomyopathy, sarcomeric cardiomyopathy, cell junction cardiomyopathy and ion channel cardiomyopathy.

Adenovirus Infections, Human↗
PubMed2000

[Hypereosinophilia with myocardial involvement due to toxocariasis. Diagnosis of regional myocardial perfusion abnormalities by pulsed tissue Doppler echocardiography].

CASE REPORT: The case of a 57-year-old woman is reported who was admitted for peripheral hypereosinophilia. DIAGNOSIS: Detailed investigations revealed first of all a hypereosinophilic syndrome with infiltration of bone marrow and lung. The patient suffered more and more from angina pectoris with signs of heart failure. Coronary angiography was therefore carried out which showed normal coronary arteries. With suspicion of myocardial involvement endomyocardial biopsies were performed which revealed the presence of Löfflers endocarditis parietalis fibroplastica. Finally, serological studies for parasites disclosed a positive ELISA test for Toxocara, confirmed later to be rising. CONCLUSION: Myocardial involvement of hypereosinophilia, caused by Toxocara is not described until now. Further diagnostic by means of pulsed wave tissue Doppler echocardiography provided regional differentiation of a restrictive filling pattern which documented the importance of this new diagnostic tool in myocardial illness.

Animals↗
PubMed2001

Restrictive left ventricular filling pattern as a strong predictor of depressed baroreflex sensitivity in heart failure.

BACKGROUND: The aim of this study was to test the hypothesis that a restrictive left ventricular diastolic filling pattern, as an index of elevated pulmonary wedge pressure, would predict a depressed baroreflex sensitivity (BRS) in patients with chronic heart failure. METHODS: A total of 189 consecutive patients with an ejection fraction < or = 40% at echocardiography, in sinus rhythm and clinically stable for at least 1 month in oral therapy, underwent clinical examination, echo-Doppler study and the phenylephrine test. RESULTS: The correlations between the NYHA functional class, echo-Doppler variables and BRS were weak, although significant (r ranging from -0.15 to 0.40). However, patients with a deceleration time < 140 ms as an expression of restrictive filling, compared to those with a deceleration time > or = 140 ms, had a lower BRS (3 +/- 4 vs 6 +/- 4 ms/mmHg, p < 0.00001), a lower ejection fraction (20 +/- 6 vs 28 +/- 7%, p < 0.00001), greater left ventricular (end-diastolic volume index 137 +/- 43 vs 113 +/- 45 ml/m2, p < 0.00001) and left atrial dimensions (25 +/- 6 vs 20 +/- 5 cm2, p < 0.00001), more severe mitral regurgitation (3 +/- 1 vs 2.3 +/- 1, p < 0.00001) and were in a higher NYHA class (2.3 +/- 0.6 vs 1.8 +/- 0.5, p < 0.00001). Medications at the time of the study were similar in the two groups. At stepwise regression analysis, the deceleration time emerged as the most powerful independent predictor of a depressed BRS (< 3 ms/mmHg), followed by mitral regurgitation, age, and NYHA class (all data p = 0.0001). CONCLUSIONS: In patients with chronic heart failure, the presence of a restrictive left ventricular filling pattern is highly predictive of autonomic derangement as expressed by low values of BRS.

Age Factors↗
PubMed1996

[Restrictive cardiac involvement in a patient with dyserythropoietic anemia and secondary hemochromatosis].

We report a case of a 63 year old woman diagnosed with dyserythropoietic anemia who was admitted to our hospital with congestive heart failure. Iron's metabolism disturbance, CT-Scan and magnetic resonance imaging allowed us to make the diagnosis of acquired hemochromatosis due to multiple transfusions. The echocardiographic study was compatible with a myocardial restrictive pattern caused by iron deposits.

Anemia, Dyserythropoietic, Congenital↗
PubMed2003

Restrictive left ventricular filling in hypereosinophilic syndrome as a result of partial cavity obliteration by an apical mass: a strain/strain rate study.

We present a case of hypereosinophilic syndrome with a large apical mass, which markedly reduced end-systolic left ventricular cavity volume. Standard blood pool Doppler findings showed a restrictive filling pattern. However, ultrasound-derived regional strain rate and strain parameters suggested that wall deformation was not compromised, inferring that the restrictive filling pattern in this case was solely as a result of the reduction in end-systolic cavity size.

Blood Flow Velocity↗
PubMed1990

[Doppler echocardiographic features of the atrial and ventricular filling modes and their significance in restrictive myocardial diseases].

The filling modes into the right atrium and both ventricles were observed using pulsed Doppler echocardiography in six cases of restrictive myocardial diseases, and these were compared with those of 13 cases of constrictive pericarditis, six cases of lone atrial fibrillation and 16 healthy subjects. Special attention was paid to the mechanical properties of the cardiac walls which might be reflected in the filling modes. 1. In the restrictive cases, right atrial filling from the superior caval vein during ventricular systole was reduced in velocity and duration, but the atrial filling during ventricular diastole was not appreciably changed. This flow pattern was similar to that of lone atrial fibrillation, indicating reduced distensibility or impaired contraction and ejection fraction of the right atrium. In constrictive pericarditis, the right atrial filling time was shortened both in ventricular systole and diastole, reflecting stiffening of the pericardium. 2. In the restrictive cases, the first half of the left ventricular rapid filling wave was steep and the skirt of the descending limb was prolonged, while there was no such tendency in the right ventricle. In constrictive pericarditis, the rapid filling time was shortened in the right ventricle, and was not significantly changed in the left ventricle. 3. The differences in the atrial and ventricular filling patterns between restrictive myocardial disease and constrictive pericarditis may serve to distinguish these two disease entities.

Adult↗