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

The purpose of this study was to analyze left ventricular (LV) torsion and untwisting, and to evaluate the correlation between torsion and other components of LV contraction in children with dilated cardiomyopathy (DCM). Segmental and global rotation, rotational rate (Vrot) were measured at three levels of LV using the twodimensional (2D) speckle tracking imaging (STI) method in 10 DCM patients (range 0.6-15 yr, median 6.5 yr, 3 females) and 17 age- and sex-matched normal controls. Global torsion was decreased in DCM (peak global torsion; 10.9+/-4.6degrees vs. 0.3+/-2.1degrees, p<0.001). Loss of LV torsion occurred mainly by the diminution of counterclockwise apical rotation and was augmented by somewhat less reduction in clockwise basal rotation. In DCM, the normal counterclockwise apical rotation was not observed, and the apical rotation about the central axis was clockwise or slightly counterclockwise (peak apical rotation; 5.9+/-4.1degrees vs. -0.9+/-3.1degrees, p<0.001). Systolic counterclockwise Vrot and early diastolic clockwise Vrot at the apical level were decreased or abolished. In DCM, decreased systolic torsion and loss of early diastolic recoil contribute to LV systolic and diastolic dysfunction. The STI method may facilitate the serial evaluation of the LV torsional behavior in clinical settings and give new biomechanical concepts for better management of patients with DCM.
Adolescent ; Cardiomyopathy, Dilated/pathology/*physiopathology ; Child ; Child, Preschool ; Echocardiography, Doppler/methods ; Female ; Humans ; Infant ; Male ; Reproducibility of Results ; Ventricular Dysfunction, Left/pathology/*physiopathology

BACKGROUNDCurrent risk stratification of idiopathic dilated cardiomyopathy (IDC) lacks sufficient sensitivity and specificity. The objective of this study was to investigate the predictive role of frontal QRS-T angles in IDC.

METHODSA prospective study with 509 IDC patients was performed from February 2008 to December 2013 in the Affiliated Drum Tower Hospital, Nanjing University School of Medicine. Baseline values and changes in QRS-T angles were recorded. Follow-up was conducted every 6 months. Analyses by Cox Proportional Hazards model were performed to evaluate the association between QRS-T angle and outcomes. The primary outcome of interest was all-cause mortality.

RESULTSDuring a median follow-up of 34 months, 90 of 316 patients with QRS-T angles >90° died compared to 31 of 193 patients with QRS-T angles ≤90° (hazard ratio [HR] =2.4, P < 0.001). Cardiac death was more prevalent in patients with a wide QRS-T angle (HR = 2.4, P < 0.001), similar to heart failure rehospitalization (HR = 2.5, P < 0.001). After adjustment for potential prognostic factors, the QRS-T angle was independently associated with all-cause mortality (HR = 2.5, P < 0.05), cardiac mortality (HR = 1.9, P < 0. 05), and heart failure rehospitalization (HR = 2.3, P < 0.01). Optimized therapy significantly narrowed the frontal QRS-T angle (100.9 ± 53.4° vs. 107.2 ± 54.4°, P < 0.001). The frontal QRS-T angle correlated well with established risk factors, such as left ventricular ejection fraction, brain natriuretic peptide, and New York Heart Association functional class.

CONCLUSIONSThe frontal QRS-T angle is a powerful predictor of all-cause mortality, cardiac mortality, and worsening heart failure in IDC patients, independent of well-established prognostic factors. Optimized therapy significantly narrows the QRS-T angle, which might be an indicator of medication compliance, but this requires further investigation.

Aged ; Cardiomyopathy, Dilated ; pathology ; physiopathology ; Electrocardiography ; Female ; Heart Failure ; pathology ; physiopathology ; Humans ; Kaplan-Meier Estimate ; Male ; Middle Aged ; Prognosis ; Prospective Studies ; Risk Factors

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

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