Cardiomyopathy, Dilated ; etiology ; pathology ; therapy ; Humans ; Myocardium ; pathology

No abstract available.
Cardiomyopathy, Dilated/etiology/*pathology ; Endomyocardial Fibrosis/*radiography ; Gadolinium ; Heart Ventricles/*physiopathology ; Humans ; Magnetic Resonance Imaging/*methods ; Ventricular Dysfunction, Left/*physiopathology

OBJECTIVESTo study the pathologic features of arrhythmogenic right ventricular cardiomyopathy (ARVC) in the phase of heart failure.

METHODSEight cases underwent heart transplantation in Fuwai Hospital during the period from May, 2004 to July, 2007 with pathologic diagnosis of ARVC were studied. The age of patients ranged from 15 to 54 years. They had history of palpitation and syncope for 1 to 22 years. Severe heart failure was diagnosed according to the New York Heart Association Classification System. The recipient hearts were examined and the following parameters were evaluated: weight of heart, presence of cardiac dilatation, myocardial hypertrophy, fatty infiltration, fibrosis, parietal thrombosis and myocarditis. The degree of left ventricular involvement was also analyzed.

RESULTSOf the 8 cases studied, 7 cases with prominent right ventricular lesion (fibrofatty replacement) were classified as classic type. One case with prominent left ventricle lesion and mild right ventricle involvement was classified as left predominant type. No biventricular type and no pure fatty infiltration were found. The cases of classic type showed moderate to severe dilatation of right ventricle, sometimes with aneurysm formation. Left ventricle was involved in 6 cases, which showed diffuse interstitial fibrosis, patchy fibrous replacement and subepicardial fatty infiltration. Mild to moderate dilatation of left ventricle, myocardial hypertrophy and vacuolation were also observed in these cases. The case of left predominant type had severe hypertrophy and dilatation of left ventricle, with prominent diffuse interstitial fibrosis and transmural fatty infiltration. Besides, 3 cases showed left ventricular hypertrophy and parietal thrombosis in both ventricles. Focal lymphocytic myocarditis was noted in 1 case.

CONCLUSIONSLeft ventricular involvement is common in the heart failure phase of ARVC. Extensive interstitial fibrosis, marked hypertrophy and degeneration of myocardial fibers, as well as severe cardiac dilatation with organized thrombi, represent the major pathologic changes which resembles dilated cardiomyopathy.

Adipose Tissue ; pathology ; Adolescent ; Adult ; Arrhythmias, Cardiac ; Arrhythmogenic Right Ventricular Dysplasia ; complications ; pathology ; physiopathology ; Cardiomyopathy, Dilated ; etiology ; Female ; Fibrosis ; etiology ; Heart Failure ; complications ; Humans ; Male ; Middle Aged ; Myocarditis ; etiology ; pathology ; Myocardium ; pathology ; Young Adult

OBJECTIVETo investigate the importance of autoimmunity against beta(1)-adrenoreceptor in the pathogenesis of dilated cardiomyopathy (DCM).

METHODSFourteen rabbits were divided equally into two groups. Rabbits in the immunized group (n = 7) were immunized monthly for one year with synthetic peptide corresponding to the second extracellular loop of the beta(1)-adrenoreceptor and adjuvant. Control rabbits received the mixture with the same procedure as described except with a substitution of saline for the corresponding peptide. During the study period, all rabbits were bled to assay the titers of antipeptide antibody and left ventricular ejection fractions (LVEFs) were measured by emission computed tomography. At the end of experiment, invasive cardiac function was measured and morphologic examinations were done.

RESULTSHigh titers of antipeptide antibody were found in the sera from immunized rabbits throughout the study period in contrast to those from control rabbits. LVEFs were significantly higher in immunized rabbits than those of the control group at the 4th and 6th month. At the end of the experiment, the maximal rates of rise and decline of ventricular pressure of the immunized group were significantly lower than those of the control group. Morphological changes were found in immunized rabbits such as the enlargement of ventricles, myofibrillar lysis and necrosis, mitochondria swelling and condensation. No obvious alterations were noted in hearts of control rabbits.

CONCLUSIONAutoimmunity against the beta(1)-adrenoreceptor may be involved in the pathogenesis of dilated cardiomyopathy and beta(1)-adrenoreceptor antibody may play a role in the process.

Animals ; Cardiomyopathy, Dilated ; etiology ; immunology ; pathology ; Heart ; drug effects ; physiopathology ; Immunization ; Male ; Microscopy, Electron ; Myocardium ; pathology ; ultrastructure ; Peptide Fragments ; administration & dosage ; chemical synthesis ; immunology ; Rabbits ; Receptors, Adrenergic, beta-1 ; administration & dosage ; chemistry ; immunology ; Ventricular Dysfunction, Left ; etiology ; physiopathology

An 82-yr-old man was presented with fever and cough accompanied by generalized erythematous rash. He had taken mexiletine for 5 months, as he had been diagnosed with dilated cardiomyopathy and ventricular arrhythmia. Laboratory studies showed peripheral blood eosinophilia and elevated liver transaminase levels. Chest radiographs showed multiple nodular consolidations in both lungs. Biopsies of the lung and skin lesions revealed eosinophilic infiltration. After a thorough review of his medication history, mexiletine was suspected as the etiologic agent. After discontinuing the mexiletine and starting oral prednisolone, the patient improved, and the skin and lung lesions disappeared. Subsequently, mexiletine was confirmed as the causative agent based on a positive patch test. Drug-induced hypersensitivity syndrome is a severe adverse reaction to drugs and results from treatment with anticonvulsants, allopurinol, sulfonamides, and many other drugs. Several cases of mexiletine-induced hypersensitivity syndrome have been reported in older Japanese males with manifestation of fever, rash, peripheral blood eosinophilia, liver dysfunction without other organ involvement. Here, we report a case of mexiletine-induced hypersensitivity syndrome which presented as eosinophilic pneumonia in a Korean male.
Aged, 80 and over ; Anti-Arrhythmia Agents/*adverse effects ; Arrhythmias, Cardiac/drug therapy ; Cardiomyopathy, Dilated/drug therapy ; Drug Hypersensitivity/*diagnosis/etiology ; Exanthema/pathology ; Humans ; Lung/pathology/radiography ; Male ; Mexiletine/*adverse effects ; Pulmonary Eosinophilia/*chemically induced/*diagnosis ; Syndrome ; Tomography, X-Ray Computed

OBJECTIVEIn order to explore the possible roles played by the autoimmune mechanism in the progression of myocarditis into dilated cardiomyopathy (DCM) using an animal model, we investigated whether autoimmune myocarditis might develop into DCM.

METHODSExperimental Balb/C mice (n = 20) were immunized with cardiac myosin with Freund's complete adjuvant at days 0, 7 and 30. The control Balb/C mice (n = 10) were immunized with Freund's complete adjuvant in the same mannere. Serum and myocardium samples were collected after the first immunization at days 15, 21 and 120. The anti-myosin antibody was examined by enzyme-linked immunosorbent assay and immunoblotting.

RESULTSPathological findings demonstrated that there was myocardial necrosis or inflammatory infiltration during acute stages and fibrosis mainly in the late phase of experimental group, but the myocardial lesions were not found in the control group. Autoimmunity could induce myocarditis and DCM in the absence of viral infection. High titer anti-myosin IgG antibodies were found in the experimental group, but not in the control group. Furthermore, the anti-myosin heavy chain (200 KD) antibody was positive in 21 of 48 patients with DCM and viral myocarditis, but only 4 of 20 patients with coronary heart disease, including 1 case and 3 cases that reacted with heavy and light chains (27.5 KD), respectively. The antibodies were not detected in healthy donors.

CONCLUSIONCardiac myosin might be an autoantigen that provokes autoimmunity and leads to the transformation of myocarditis into DCM. Detection of anti-myosin heavy chain antibody might contribute to diagnosis for DCM and viral myocarditis.

Adult ; Aged ; Animals ; Autoantibodies ; blood ; Autoimmune Diseases ; complications ; Cardiac Myosins ; immunology ; Cardiomyopathy, Dilated ; etiology ; immunology ; Female ; Humans ; Male ; Mice ; Mice, Inbred BALB C ; Middle Aged ; Myocarditis ; complications ; Myocardium ; pathology

OBJECTIVETo explore the role of interleukin-17 (IL-17) in the evolution of viral myocarditis (VMC) into dilated cardiomyopathy (DCM).

METHODSA mouse model of VMC was established in 100 male Balb/c mice by intraperitoneal injection of coxsackievirus B3. The expression of IL-17 protein in the cardiac tissue of the mice was detected immunohistochemically, and IL-17 mRNA in the splenocytes was examined by reverse transcription-polymerase chain reaction (RT-PCR). IL-17 levels in the plasma, peripheral blood mononuclear cell (PBMC) culture supernatants, and phytohemagglutinin (PHA)-stimulated PBMC culture supernatants were measured in 30 DCM patients, 26 non-DCM patients and 20 normal adults using enzyme-linked immunosorbent assay (ELISA), and IL-17 mRNA expression in the PBMCs was detected using RT-PCR.

RESULTSThe levels of IL-17 mRNA in the splenocytes of the mice with VMC were significantly higher at 4 and 6 weeks than those at 8 weeks (P<0.01), but not detected at 2 weeks. No IL-17 expression was found in the ventricular tissue of the mice at 2 weeks, but peaked at 4 weeks followed by gradual decrease (P<0.01). IL-17 level in PHA-stimulated PBMC culture supernatants but not the plasma, and its mRNA level in PHA-stimulated PBMCs but not the PBMC culture supernatants, were significantly elevated in DCM patients as compared with those in non-DCM patients and normal control subjects.

CONCLUSIONSThe mouse model of VMC in the chronic phase and DCM patients express high levels of IL-17, which may contribute to the transition from VMC to DCM.

Adult ; Animals ; Cardiomyopathy, Dilated ; etiology ; metabolism ; pathology ; Coxsackievirus Infections ; complications ; metabolism ; Enterovirus B, Human ; Female ; Humans ; Interleukin-17 ; genetics ; metabolism ; Male ; Mice ; Mice, Inbred BALB C ; Middle Aged ; Myocarditis ; complications ; metabolism ; virology ; RNA, Messenger ; genetics ; metabolism