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At least 577 records · Page 32Linked to original sources
PubMed2001

[Clinical manifestations of AL-amyloidosis and some other types of amyloidosis].

Amyloidosis is the term for specific pathological peptide deposits in various tissues. Amyloid substances may be the manifestation of the following nosological units: AL-amyloidosis, AA-amyloidosis, ATTR-amyloidosis, beta2-microglobulin amyloidosis and possibly othr familiar forms of amyloidosis. The most frequent symptoms of amyloidosis are: 1. proteinuria with nephrotic syndrome, 2. cardiac failure with restrictive cardiomyopathy, 3. unexplicable hepatomegaly, 4. idiopathic peripheral polyneuropathy, 5. haemorrhagic manifestations and symptoms of increased fragility of the capillaries while the number of thrombocytes is normal, 6. periorbital haematomas and 7. macroglossia. Diagnostic and therapeutic procedures differ according to the type of amyloidosis and are analyzed in detail in the text.

Amyloidosis↗
PubMedDate not supplied

Magnetic resonance imaging in the evaluation of the pericardium. A pictorial essay.

Magnetic resonance (MR) is an ideal technique for the evaluation of the pericardium since it enables the combination of high resolution anatomical images of the pericardial layers with functional information concerning the impact of pathology on diastolic heart function and cardiac filling in particular. In comparison with echocardiography, which remains the first choice technique for the study of the pericardium, MR provides larger fields of view allowing the visualisation of the entire chest, higher spatial and contrast resolution and greater reproducibility. The technique becomes particularly useful when ultrasound imaging does not provide adequate diagnostic information or requires further characterisation; ''non-echoic'' patients, loculated pericardial effusions, focal thickening of the layers and pericardial masses are usually better assessed with MR. The method also provides valuable diagnostic information for establishing the diagnosis of constrictive pericarditis and to differentiate this condition from restrictive cardiomyopathy. The aim of this paper is to present the role of MR imaging in the assessment of a patient with suspected pericardial disease, and discuss the MR technique, anatomy and the main pathological conditions.

Cardiomyopathies↗
PubMed2005

A case of primary malignant pericardial mesothelioma.

Primary malignant pericardial mesothelioma (PMPM) is an exceedingly rare tumour. One of the largest necropsy series gave an incidence of primary pericardial tumours of 0.0022%, of which mesothelioma is the most common type. In a Canadian epidemiological survey, the annual incidence of PMPM was reported to be one in 40 million. A male predominance of the disease has been described, and the majority of cases occur in the fourth to seventh decades of life. There has been no definite association between asbestos exposure and pericardial disease. Due to its generally late presentation and poor response to therapy, the prognosis is very poor. The present report discusses the case of a 43-year-old man who presented with cardiac tamponade and was subsequently diagnosed with PMPM. Cardiac tamponade is a known complication of the malignancy, but it is rarely the first manifestation of cancer. The patient's clinical course was a result of the aggressive nature of PMPM. Effusive constrictive pericarditis and restrictive cardiomyopathy were likely contributors to this patient's disease burden. These processes should be considered and managed appropriately in patients who do not respond to pericardiocentesis or pericardial window as treatment for pericardial tamponade.

Adult↗
PubMed2005

Primary amyloidosis as a cause of microvascular angina and intermittent claudication.

Primary systemic amyloidosis or AL amyloidosis is a rare condition characterized by extracellular deposits of fibrils composed of fragments of immunoglobulin light chains. Widespread deposition of this amyloid in tissues interferes with their normal function and leads to multiple organ failure. Clinical manifestations are highly variable due to the wide range of organs involved. The heart is affected in 90% of cases; in 30% of these, cardiac dysfunction is the form of presentation and in 50% it is the cause of death. The commonest form of cardiovascular manifestation is congestive heart failure due to restrictive cardiomyopathy caused by extensive interstitial infiltration of amyloid into the myocardium. Occasionally, it can present as angina, due to infiltration of amyloid into the walls of small vessels. The authors describe the case of a patient in whom the disease presented simultaneously with heart failure and effort angina, as well as intermittent claudication.

Aged↗
PubMedDate not supplied

Progressive heart failure in a patient after coronary artery bypass grafting.

A 60-year-old man suffered progressive heart failure caused by restrictive cardiomyopathy after coronary artery bypass grafting. The cardiomyopathy was due to cardiac amyloidosis coexisting with coronary artery disease. Repeated echocardiographic assessment of cardiac structure and function was crucial for diagnosis.

Coronary Artery Bypass↗
PubMed1991

Magnetic resonance imaging of great vessel, myocardial, and pericardial disease.

The outstanding ability of magnetic resonance imaging to provide anatomic information over a large field of view in any plane in patients of all sizes and body habitus has made it the current modality of choice for evaluating the thoracic aorta and for differentiating constrictive pericarditis from restrictive cardiomyopathy. The lack of radiation exposure makes it particularly advantageous for patients requiring serial studies. It serves as an important clinical adjunct to ultrasound in evaluating diseases of the abdominal aorta, other great vessels, the myocardium, and the pericardium. Myocardial function, thickness, and mass can be assessed without geometric assumptions. Myocardial tagging offers a unique way to study cardiac biomechanics. The combination of anatomic and quantitative functional information becoming available in clinically reasonable acquisition times makes magnetic resonance imaging an increasingly attractive noninvasive tool. The contribution of tissue characterization has been disappointing, but may improve with utilization of contrast agents; that of in vivo spectroscopy remains investigational.

Aorta, Abdominal↗
PubMed1991

Senile cardiac amyloidosis associated with homozygosity for a transthyretin variant (ILE-122).

Senile cardiac amyloidosis, also known as senile systemic amyloidosis, is a sporadic disease of late onset but with increasing incidence with age. Recently it has been shown in one case that amyloid deposits contained a transthyretin variant with an isoleucine for valine substitution at position 122. A second case with the transthyretin isoleucine 122 variant is reported here. This individual, who died with restrictive cardiomyopathy, was found to be homozygous for this transthyretin variant and his son heterozygous for the variant. A brother of the propositus was also homozygous for the variant but died of a cerebral vascular accident without being evaluated for amyloidosis. These studies show genetic transmission of the isoleucine-122 transthyretin variant associated with this form of cardiac amyloidosis.

Aged↗
PubMed1990

Doppler echocardiography in pericardial disease.

In conclusion, an understanding of the physiology of cardiac tamponade and pericardial constriction allows accurate interpretation of the changes in SVC and transatrioventricular valve Doppler flow velocities that characterize each abnormality. Meticulous attention to detail in obtaining the studies is essential for accurate diagnosis, because relative changes in flow velocities during respiration may be obscured or misinterpreted if poor-quality data are obtained. In our laboratory, SVC Doppler studies have proved to be the most technically feasible in patients with cardiac tamponade, but transvalvular studies provide important complementary data and are critical in constrictive pericarditis. Hepatic vein Doppler offers an alternative approach to the analysis of systemic venous return, particularly in stable patients with dilated hepatic veins. These studies must always be interpreted with attention to possible confounding variables such as previous pericardiotomy, significant tricuspid regurgitation, severe chronic lung disease, or restrictive cardiomyopathy. As physiologic rather than anatomic indicators, they are a valuable addition to echocardiography in assessing the hemodynamic significance of pericardial abnormalities.

Adult↗
PubMed1990

Coenzyme Q10: clinical benefits with biochemical correlates suggesting a scientific breakthrough in the management of chronic heart failure.

There are obviously several causes of myocardial dysfunction but energy deficiency of the myocytes may play a significant role and probably is a common mechanism during the progression of myocardial failure. Theoretically, a poor utilization efficiency of oxygen may be due to exhaustion of the myocardial stores of bioenergetics. In this report the authors review their biochemical results from measurements of coenzyme Q10 (CoQ10) levels in blood and human endomyocardial biopsies using an HPLC method from patients with suspected myocardial disease (n = 45). The levels of CoQ10, which has a key role in the respiratory chain and the synthesis of ATP, was found to be significantly decreased in various groups of patients with myocardial failure (dilated and restrictive cardiomyopathy and alcoholic heart disease) as compared to "normal" myocardium (0.42 +/- 0.04 micrograms/mg dry weight). The deficiency of CoQ10 was more pronounced with increasing symptoms; e.g. patients with dilated cardiomyopathy in NYHA Classes III and IV had lower tissue CoQ10 content than those of Classes I and II (0.28 +/- 0.04 vs. 0.37 +/- 0.06 micrograms/mg, p less than 0.001). Nearly two thirds of a series of 40 patients in severe heart failure (Classes III and IV) treated with CoQ10, 100 mg daily, in an open, controlled design showed subjective and objective improvement. Clinical responders were 69% and 43% of patients with cardiomyopathy and ischaemic heart disease, respectively. The results suggest that CoQ10 is a novel and effective breakthrough in heart-failure therapy and it appears safe, as no adverse reactions were registered. The through in heart-failure therapy and it appears safe, as no adverse reactions were registered.(ABSTRACT TRUNCATED AT 250 WORDS)

Chronic Disease↗
PubMed1990

Diastolic dysfunction during acute cardiac allograft rejection.

Left ventricular diastolic function was evaluated in 41 heart transplant patients during acute rejection by an analysis of echocardiograms and surgically implanted intramyocardial tantalum markers. In 35 patients, isovolumic relaxation time was calculated from M-mode tracings selected from two-dimensional echocardiographic recordings. A total of 84 biopsy findings of no rejection, moderate rejection, and severe acute rejection after treatment were correlated with measurements of isovolumic relaxation time. In six patients, end-diastolic volume, end-systolic volume, stroke volume, ejection fraction, and peak filling rate were obtained from biplanar cineradiographic images of intramyocardial markers. Data from 11 prerejection periods were compared with those of moderate acute rejection. All echocardiograms and marker images were analyzed without previous knowledge of biopsy findings. At times of acute rejection, isovolumic relaxation time decreased from 107 to 65 msec (p less than 0.01) and returned to 98 msec after immunosuppressive therapy. Ejection fraction and end-systolic volume did not change significantly with acute rejection, whereas stroke volume decreased from 76 to 67 ml (p less than 0.05). In contrast to the effects on systolic function, episodes of acute rejection were accompanied by a decrease in end-diastolic volume from 166 to 153 ml (p less than 0.01) and a reduction in peak filling rate from 514 to 460 ml/sec (p less than 0.05). These data suggest that acute cardiac rejection is associated with relative preservation of left ventricular systolic performance but with alterations in diastolic dynamics similar to those seen in "restrictive" cardiomyopathy.

Acute Disease↗
PubMedDate not supplied

[Idiopathic hypereosinophilic syndrome--differential diagnosis problems and a case report].

A case of male patient with hypereosinophilic syndrome is described. The diagnosis was suggested by clinical features, laboratory data, roentgenological and echosonographic findings and confirmed by cytologic and hematological findings obtained by bone, liver and heart biopsies. There was no evidence of other diseases causing hypereosinophilia. Clinically, the disease presented with cardiac decompensation due to restrictive cardiomyopathy, toxic hepatic lesion and varicosal leg ulcers. The patient improved after the institution of corticosteroids.

Diagnosis, Differential↗
PubMed1989

Endomyocardial fibrosis in the child.

Endomyocardial fibrosis is a rare disease in children and has been mainly observed in tropical Africa, seldom in Europe. Its precise aetiology remains unknown. New surgical procedures have recently led to better survival. Four children, a 5 year old Swiss boy and 3 African girls, aged 12 to 14 years, were submitted with global heart failure class III to IV of the NYHA classification. Two patients had eosinophilia on admission. A third had a history of transient eosinophilia. The echocardiographic examination showed a very typical picture. In 3 patients (2 f and 1 m) both ventricles were involved; in one girl only the left ventricle. Decreased ventricular distensibility with impaired filling of the left and/or right ventricle was present in all. Diastolic pressures ranged between 24 and 35 mmHg; the systolic function was satisfactory in 3 children (ejection fraction [EF] of 44 to 61%) and severely decreased in the fourth (EF 10%). Three patients underwent endocardectomy: both ventricles in 1 case, only the left in 2 cases. Mitral valve repair by means of the Carpentier ring was done once, valve replacement once (Starr-Edwards prothesis). One child died; the 2 surviving patients showed a distinct improvement in their clinical state and are now in class II of the NYHA classification. Echocardiographic and catheter investigations show, however, persistence of the restrictive cardiomyopathy. Thus, surgical intervention must be considered a palliative procedure in these cases. The diagnosis can clearly be made nowadays with bidimensional and Doppler echocardiography.

Adolescent↗
PubMed1987

[Cardiac amyloidosis. Invasive and noninvasive diagnosis].

Aim of this study is the analysis of clinical, morphologic and haemodynamic features of cardiac amyloidosis. Cardiac amyloidosis was demonstrated histologically in 7 of our patients: in 6 by endomyocardial biopsy during cardiac catheterization, in one at autopsy. The clinical picture was characterized in every patient by signs and symptoms of congestive heart failure. The electrocardiogram showed several non specific signs: low voltage of the QRS complexes, both in peripheral (4/7) and precordial leads (7/7 cases); marked leftward and upward deviation of the QRS axis (6/7 cases); first degree A-V block (5/7); abnormal Q waves (7/7). M-mode and two-dimensional echocardiography invariably demonstrated a typical pattern: a non dilated left ventricle with thickened and hyper-refractile walls, and usually a slight-moderate diffuse hypokinesia. Other common features were a thickening of right ventricular walls, interatrial septum, and atrioventricular and semilunar valves. Computerized analysis of the M-mode tracings disclosed a marked impairment of the indexes of both systolic and diastolic ventricular function in all patients. By correlating electrocardiographic and echocardiographic data, we found in every case a striking disproportion between the low QRS voltage and the high muscle cross-sectional area (an echocardiographic index of left ventricular mass): this pattern appears to be highly suggestive of infiltrative heart disease. Cardiac catheterization (performed in 6 cases) showed an increase of left ventricular (6/6) and right ventricular (5/6) end-diastolic pressure, with a dip plateau pattern in some cases (4/6 of the left, 2/6 in the right ventricle). The cardiac index was decreased in 3/6 cases. Left ventricular angiography confirmed the echocardiographic data of normal volumes and a slight-moderate decrease of the ejection fraction. We conclude that cardiac amyloidosis usually mimics a restrictive cardiomyopathy (severe congestive heart failure with increased ventricular filling pressures, in the absence of severe systolic ventricular dysfunction). This disease can be suspected clinically by the correlation of the clinical, electrocardiographic and echocardiographic data. The final diagnosis requires an endomyocardial biopsy.

Adult↗
PubMed1985

Cardiac amyloidosis: five cases diagnosed by endomyocardial biopsy.

Between October 1974 and December 1985, 350 patients have been subjected to endomyocardial biopsy (EMB). Five of these subjects clinically presented restrictive cardiomyopathy symptoms. EMB revealed diffuse cardiac amyloidosis. Light-electron-microscopic and morphometric data have been reported. The role of EMB in the diagnosis of cardiac disease is also emphasized.

Amyloid↗
PubMed1985

Mitral valve replacement in idiopathic hypereosinophilic syndrome.

Idiopathic hypereosinophilic syndrome is a rare systemic disease that can cause one form of endocardial fibrosis. Endocardial fibrous tissue with overlying thrombus formation in idiopathic hypereosinophilic syndrome leads to a restrictive cardiomyopathy that may produce mitral and/or tricuspid regurgitation. This report describes a patient with idiopathic hypereosinophilic syndrome who underwent mitral valve replacement and successful steroid therapy for an usually localized form of endocardial fibrosis.

Endocarditis↗
PubMed1985

[Acquired systemic amyloidosis associated with monoclonal gammopathy].

A case of acquired systemic amyloidosis with monoclonal gammopathy in a woman aged 67 years is reported. Clinical features included macroglossia, restrictive cardiomyopathy, cutaneous purpura and carpal-tunnel syndrome. A short review on amyloidosis appears in the case discussion.

Aged↗
PubMed1985

[Idiopathic hypereosinophilic syndrome. Current clinico-therapeutic state].

Idiopathic hypereosinophilic syndrome is a term labelling clinical illnesses characterized by blood hypereosinophilia and widespread infiltration of organs and tissues by mature eosinophils. Although any tissue can be affected the involvement of the nervous system and of the myocardium usually indicates worse prognosis. Histopathological studies carried out on myocardial tissues of hypereosinophilic patients, suggest that eosinophils are the main inducers of tissue damages. These cells seem to affect first the coronary endothelial cells, so leading to thrombi formation and then fibrosis. Eventually restrictive cardiomyopathy may occur. The pathogenetic events are not fully understood. However several studies have been focused on two eosinophil proteins: the major basic protein (MBP) as possible mediator of tissue damages and the cationic eosinophil protein (ECP) involved in thrombotic events. In the bone marrow a marked hyperplasia of the eosinophil series is usual, while in the blood only mature eosinophils circulate. Non specific abnormalities either of the cellular or humoral immunity have been described. Previous reports of a definitely incurable disease have been improved by modern therapeutic programs.

Adrenal Cortex Hormones↗
PubMed1978

Endomyocardial fibrosis with calcification of the myocardium.

A case of endomyocardial fibrosis with intramyocardial calcification was characterized by symptoms of restrictive cardiomyopathy resembling constrictive pericarditis. Pathological findings were typical of those found in classical Davies' disease, originally encountered and described in Uganda.

Calcinosis↗