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At least 685 records · Page 38Linked to original sources
PubMed1985

Endomyocardial biopsy: a review of the literature.

A review of the literature relating to endomyocardial biopsy (EMB) is presented. This is considered important at this time since EMB is being utilized with increasing frequency, particularly for the diagnosis of myocarditis. The development of the technique is briefly outlined. Emphasis is placed on the clinical application of EMB in the various primary cardiomyopathies (dilated, hypertrophic, restrictive, and obliterative), the infiltrative secondary cardiomyopathies (amyloidosis, sarcoidosis, hemochromatosis), myocarditis, as well as such conditions as adriamycin cardiotoxicity, cardiac transplant rejection, and Kawasaki disease. More controversial application of EMB in primary mitral valve prolapse (Barlow's syndrome), idiopathic ventricular arrhythmias, and the elucidation of the enigmatic finding of angina with angiographically normal coronary arteries is detailed. Experience with immunological and biochemical investigation of biopsy material, as well as with virus isolation and drug assays in the myocardium, is alluded to. Complications encountered with this procedure are also discussed, and its future role is contemplated.

Amyloidosis↗
PubMed1992

Interpretation of cardiac pathophysiology from pressure waveform analysis: pericardial compressive hemodynamics, Part III.

Failure of right atrial pressure to normalize after pericardial pressure is relieved suggests persistent effusive-constrictive physiology, myocardial failure, or cardiomyopathy which may have indistinguishable hemodynamic pressure waveforms. Clinical characteristics, ancillary testing, and endomyocardial biopsy may be required to obtain a definite diagnosis in such cases. The hemodynamic waveforms obtained during pericardiocentesis provide insight into the pathophysiologic processes, producing symptoms of dyspnea in this interesting patient group.

Adult↗
PubMed2006

B-type natriuretic peptide level in a patient with constrictive pericarditis.

We report the case of a 35-year-old man with constrictive pericarditis who had a B-type natriuretic peptide (BNP) level of 129 pg/dl despite a left ventricular end diastolic pressure of 35 mmHg. We discuss a possible explanation for the relatively low BNP level given this patient's markedly elevated intracavitary pressures in the setting of constrictive pericarditis.

Adult↗
PubMed2007

Acquired immune cytopenias post-cardiac transplantation respond to rituximab.

Hematologic abnormalities following solid organ transplantation are infrequently autoimmune in origin. We present a series of autoimmune cytopenias developing as a late complication of pediatric cardiac transplantation. Autoimmune cytopenias represented include autoimmune hemolytic anemia, acquired Glanzmann thrombasthenia, and idiopathic thrombocytopenic purpura. Standard therapies were used in each patient without sustainable results. Eventually, each patient was treated with and responded to rituximab. In this report, we review these cases, propose potential mechanisms for development of autoimmune cytopenias, and discuss our experience with rituximab in managing refractory autoimmune cytopenias.

Anemia, Aplastic↗
PubMed1993

Molecular genetic aspects of cardiomyopathy.

Cardiomyopathies are a major cause of mortality and morbidity and this spectrum of disorders tops the list of diseases leading to cardiac transplantation. While significant gains have been made during the past decade clinically, knowledge of the molecular aspects of these disorders has taken longer to advance. During the past 5 years, however, molecular genetic information on a variety of primary cardiomyopathies, such as familial hypertrophic cardiomyopathy and X-linked dilated cardiomyopathy, has been obtained. Other primary and secondary myocardial diseases are now under study with advances occurring more regularly. The purpose of this review is to outline the major advances thus far described for some primary and secondary cardiomyopathies, as well as detailing the slower progress seen for others.

Cardiomyopathies↗
PubMedDate not supplied

Cardiomyopathy in children in Ahmedabad.

A clinical study and follow up of 20 children with cardiomyopathies upto age of 16 years are presented. The DCM was most common variety followed by RCM and HCM in pediatric age group. SHMD presenting with cardiomyopathy were common in infancy and early childhood. Cardiomyopathies presented most frequently between 2-5 years and 10-16 years age group with DCM having almost equal distribution. Clinical presentation of various types is described, despite of vigorous decongestive and vasodilator treatment in advanced cases, course was rapidly downhill and prognosis is poor in general.

Adolescent↗
PubMed2005

[Arrhythmogenic right ventricular cardiomyopathy].

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a primary myocardial disorder that is characterized by localized or diffuse atrophy of predominantly right ventricular myocardium with subsequent replacement by fatty and fibrous tissue. Arrhythmias of right ventricular origin are the main clinical manifestation. Affected patients present with ventricular premature beats and nonsustained or sustained ventricular tachycardia demonstrating a left bundle branch block pattern. However, since ventricular tachycardia may also degenerate into ventricular fibrillation, sudden death may be the first manifestation of ARVC.In recent years, ARVC has been more and more recognized as an important and frequent cause of ventricular tachyarrhythmias and sudden cardiac death, particularly in young patients and athletes, with apparently normal hearts. Evidence of the disease is found in 30-50% of family members. ARVC is a genetically heterogeneous disease. The diagnosis is based on electrocardiographic abnormalities and the identification of regional or global right ventricular dysfunction and fibrolipomatosis. Although several potentially causative genes have been identified, currently, genetic testing is not part of the routine diagnostic work-up.An implantable cardioverter-defibrillator is indicated in selected high-risk patients with ARVC (i. e., patients with life-threatening ventricular tachycardia or survivors of sudden cardiac death). The clinical course of the disease is often characterized by progression. In individual patients heart transplantation may become necessary.

Arrhythmogenic Right Ventricular Dysplasia↗
PubMed2000

Magnetic resonance imaging in patients with cardiomyopathies: when and why.

Cardiac magnetic resonance imaging (MRI) is a noninvasive tool which is able to diagnose and differentiate cardiomyopathies in a single study. The assessment of essential information such as alterations of myocardial and ventricular geometry and function is possible with a high degree of accuracy and reproducibility, based on a small inter- and intraobserver variability. Thus, very small morphological and functional changes in different types of cardiomyopathy are detectable, thereby enabling the cardiologist to increase the safety of therapeutic decisions. Furthermore, MRI bears the potential to characterize tissue transformation in the different types of myocardial affections including ischemic, toxic, infiltrative or inflammatory forms.

Adult↗
PubMed1996

Levels of cerium in the tissues of rats fed a magnesium-restricted and cerium-adulterated diet.

Cerium, a lanthanide, is the most abundant rare earth element present in monazite and is biologically active. It has been postulated that cerium toxicity in conjunction with magnesium deficiency causes tropical endomyocardial fibrosis, a restrictive human cardiomyopathy (Valiathan et al. 1989; Valiathan and Kartha 1990). Generally, lanthanides are known to be poorly absorbed (Durbin et al. 1956; Evans 1990). The present study was carried out to ascertain whether magnesium deficiency promotes accumulation of cerium in various organs of rats.

Animals↗
PubMedDate not supplied

Disseminated peritoneal plasmacytoma following cardiac transplantation.

Posttransplant lymphoproliferative disorders (PTLD) are an important comorbidity in immunosuppressed transplant patients. We describe a unique case of PTLD, a disseminated peritoneal plasmacytoma in a 56-year-old cardiac transplant patient presenting with ascites. The associated computed tomographic findings include omental thickening, mesenteric stranding, and ascites.

Ascites↗
PubMed2003

Paradoxical regional myocardial uptake between 201Thallium and 123I-BMIPP SPECT in patients with cardiomyopathy.

The clinical significance of the paradoxical mismatched phenomenon between (201)Tl and (123)I-BMIPP is still unknown. We report two cases that revealed paradoxical regional myocardial uptake between two tracers in patients with cardiomyopathy. There may be abnormal myocardium in these patients where active transportation of (201)Tl is disturbed and passive transportation of (123)I-BMIPP is not disordered.

Adult↗
PubMed2005

Comparison of epirubicin and doxorubicin cardiotoxicity in children and adolescents treated within the German Cooperative Soft Tissue Sarcoma Study (CWS).

PURPOSE: Up to now, cardiotoxicity of epirubicin has been studied almost exclusively in adult cancer patients. The aim of this study was to investigate epirubicin in children and adolescents, in comparison with doxorubicin. METHODS: About 172 soft tissue sarcoma patients (mean age at diagnosis: 8.3 years), treated with epirubicin (median cumulative dose: 450 mg/m2) or doxorubicin (median cumulative dose: 240 mg/m2) within the high-risk group of the CWS-96 study, were examined in a prospective multicentre study. Heart function was analysed by echocardiography, measuring left-ventricular fractional shortening (FS). The median follow up was 27.7 months. RESULTS: Incidence of clinically manifest cardiomyopathy was 0% (0/60; 95% CI: 0-6.0%) in patients treated with epirubicin, and 0.9% (1/108; 95% CI: 0-5.1%) in patients treated with doxorubicin. A further three patients showed subclinical cardiomyopathy. There was no difference in FS between the two treatment arms. CONCLUSIONS: Cardiotoxicity was low in our study. For the short term, cardiotoxicity seems to be only a minor problem in patients treated with epirubicin as applied in this cohort.

Adolescent↗
PubMed1995

Heart transplantation in children and young adults: early and intermediate-term results.

The purpose of this article is to report our short- and intermediate-term follow-up of cardiac transplantation for congenital heart disease and cardiomyopathy in children (age greater than 6 months), adolescents, and young adults. Thirty patients (ages 8 months to 24 years) with end-stage heart failure have undergone cardiac transplantation in our program: 12 (40%) for postoperative end-stage heart failure, 9 (30%) as primary treatment for congenital heart disease, 5 (17%) for dilated cardiomyopathy, and 4 (13%) for restrictive/hypertrophic cardiomyopathy. Nineteen patients (63%) had undergone prior operations; 4 patients received transplants for failed Fontan procedures. Induction therapy with antithymocyte therapy was used routinely, and long-term immunosuppression was by cyclosporine and azathioprine alone. Rejection surveillance/diagnosis was based on echocardiographic criteria. Posttransplantation follow-up ranges from 3 to 78 months. Operative mortality was 3.3% (1/30). No patients have been diagnosed with either accelerated allograft atherosclerosis or posttransplantation lymphoproliferative disease. We conclude that cardiac transplantation may be performed with excellent early and intermediate-term results.

Adolescent↗
PubMed1995

Immunological characterization and functional importance of human heart mast cells.

Mast cells are present in normal and even more abundant in diseased human heart tissue and their localization is of particular relevance to their function. Within heart tissue mast cells lie between myocytes and in close contact with blood vessels. They are also found in the coronary adventitia and in the shoulder regions of a coronary atheroma. The density of cardiac mast cells is markedly higher in some patients with myocarditis and dilated cardiomyopathy than in accident victims without cardiovascular diseases. More importantly, in some of these conditions there is in situ evidence of mast cell activation. We have described an original technique to isolate and purify HHMC for in vitro study. This procedure gives viable cells and after stimulation with immunological or non-immunological stimuli they release performed (histamine and tryptase) and newly generated mediators (PGD2 and LTC4). We have demonstrated that HHMC differ from those in other anatomical districts in that they are activated by specific immunological and non-immunological stimuli, and in their relation to the arachidonic acid metabolism, suggesting that the local microenvironment can influence their phenotypic and biochemical characteristics. Our own and other findings suggest that HHMC have complex and significant roles in different pathophysiological conditions involving the cardiovascular system. Direct activation of HHMC by therapeutic and diagnostic substances injected intravenously explains some of the anaphylactoid reactions caused by these agents. HHMC possess Fc epsilon RI and IgE bound to the surface and C5a receptors, which could explain how cardiac mast cells are involved in systemic and cardiac anaphylaxis. Cardiac mast cells and those in human coronary arteries also play a role in the early and late stages of atherogenesis and during ischemic myocardial injury. In conclusion, although studies of HHMC are in their infancy, their in vitro isolation may be useful in identifying additional mediators synthesized and released, stimuli relevant to human pathophysiology, and pharmacological agents selectively modulating the activation of these cells and their mediators. Drugs specifically acting on HHMC or on their mediators may eventually be useful in treating different cardiovascular diseases.

Anaphylaxis↗