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Cardiomyopathy Patients (cardiomyopathy + patient)
Selected AbstractsRight Ventricular Dimensions and Function in Isolated Left Bundle Branch Block: Is There Evidence of Biventricular Involvement?ECHOCARDIOGRAPHY, Issue 5 2008Jeroen Van Dijk M.D. Background: Isolated left bundle branch block (LBBB) may be an expression of idiopathic cardiomyopathy affecting both ventricles. The present study was conducted to evaluate right ventricular (RV) dimensions and function in asymptomatic LBBB patients with mildly depressed left ventricular (LV) function. Methods: Fifteen patients with asymptomatic LBBB in whom coronary artery disease, hypertension, and valvular pathology was excluded were studied. Fifteen healthy volunteers and 15 idiopathic dilated cardiomyopathy LBBB patients served as controls. RV long axis and tricuspid annulus diameter were obtained, as were tricuspid annular plane systolic excursion (TAPSE) and peak systolic velocity (Sm) of the RV free wall annulus. Tricuspid regurgitation (TR) jets (peak TR jets) were used for RV pressure assessment. Results: RV dimensions were comparable between the asymptomatic LBBB patients and controls. RV functions of healthy volunteers and asymptomatic LBBB patients were similar (TAPSE: 24 ± 3 and 24 ± 4 mm, Sm: 13 ± 2 and 13 ± 3 cm/s, respectively), whereas functional parameters in idiopathic dilated cardiomyopathy patients were significantly reduced (TAPSE: 19 ± 5 mm, Sm: 9 ± 2 cm/s, both P < 0.01 by analysis of variance [ANOVA]). For the three groups combined, a significant inverse correlation between RV pressure (peak TR jets) and RV function (Sm) was observed (r =,0.52, P = 0.017). Conclusions: In patients with an asymptomatic LBBB, RV dimensions and function are within normal range. The present study suggests that screening of RV functional parameters in asymptomatic LBBB patients is not useful for identification of an early-stage cardiomyopathy, and RV dysfunction is merely a consequence of increased RV loading conditions caused by left-sided heart failure and does not indicate a generalized cardiomyopathy affecting both ventricles. [source] A human phospholamban promoter polymorphism in dilated cardiomyopathy alters transcriptional regulation by glucocorticoids,HUMAN MUTATION, Issue 5 2008Kobra Haghighi Abstract Depressed calcium handling by the sarcoplasmic reticulum (SR) Ca-ATPase and its regulator phospholamban (PLN) is a key characteristic of human and experimental heart failure. Accumulating evidence indicates that increases in the relative levels of PLN to Ca-ATPase in failing hearts and resulting inhibition of Ca sequestration during diastole, impairs contractility. Here, we identified a genetic variant in the PLN promoter region, which increases its expression and may serve as a genetic modifier in dilated cardiomyopathy (DCM). The variant AF177763.1:g.203A>C (at position ,36,bp relative to the PLN transcriptional start site) was found only in the heterozygous form in 1 out of 296 normal subjects and in 22 out of 381 cardiomyopathy patients (heart failure at age of 18,44 years, ejection fraction=22±9%). In vitro analysis, using luciferase as a reporter gene in rat neonatal cardiomyocytes, indicated that the PLN-variant increased activity by 24% compared to the wild type. Furthermore, the g.203A>C substitution altered the specific sequence of the steroid receptor for the glucocorticoid nuclear receptor (GR)/transcription factor in the PLN promoter, resulting in enhanced binding to the mutated DNA site. These findings suggest that the g.203A>C genetic variant in the human PLN promoter may contribute to depressed contractility and accelerate functional deterioration in heart failure. Hum Mutat 29(5), 640,647, 2008. © 2008 Wiley-Liss, Inc. [source] QT Dispersion Does Not Represent Electrocardiographic Interlead Heterogeneity of Ventricular RepolarizationJOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, Issue 8 2000MAREK MALIK Ph.D. QT Dispersion and Repolarization Heterogeneity. Introduction: QT dispersion (QTd, range of QT intervals in 12 ECG leads) is thought to reflect spatial heterogeneity of ventricular refractoriness. However, QTd may be largely due to projections of the repolarization dipole rather than "nondipolar" signals. Methods and Results: Seventy-eight normal subjects (47 ± 16 years, 23 women), 68 hypertrophic cardiomyopathy patients (HCM; 38 ± 15 years. 21 women), 72 dilated cardiomyopathy patients (DCM; 48 ± 15 years, 29 women), and 81 survivors of acute myocardial infarction (AMI; 63 ± 12 years, 20 women) had digital 12-lead resting supine ECGs recorded (10 ECGs recorded in each subject and results averaged). In each ECG lead, QT interval was measured under operator review by QT Guard (GE Marquette) to obtain QTd. QTd was expressed as the range, standard deviation, and highest-to-lowest quartile difference of QT interval in all measurable leads. Singular value decomposition transferred ECGs into a minimum dimensional time orthogonal space. The first three components represented the ECG dipole; other components represented nondipolar signals. The power of the T wave nondipolar within the total components was computed to measure spatial repolarization heterogeneity (relative T wave residuum, TWR). OTd was 33.6 ± 18.3, 47.0 ± 19.3, 34.8 ± 21.2, and 57.5 ± 25.3 msec in normals, HCM, CM, and AMI, respectively (normals vs DCM: NS, other P < 0.009). TWR was 0.029%± 0.031%, 0.067%± 0.067%, 0.112%± 0.154%, and 0.186%± 0.308% in normals, HCM, DCM, and AMI (HCM vs DCM: NS. other P < 0.006), The correlations between QTd and TWR were r = -0.0446, 0.2805, -0.1531, and 0.0771 (P = 0.03 for HCM, other NS) in normals, HCM, DCM, and AMI, respectively. Conclusion: Spatial heterogeneity of ventricular repolarization exists and is measurable in 12-lead resting ECGs. It differs between different clinical groups, but the so-called QT dispersion is unrelated to it. [source] Activation Sequence Modification During Cardiac Resynchronization by Manipulation of Left Ventricular Epicardial Pacing Stimulus StrengthPACING AND CLINICAL ELECTROPHYSIOLOGY, Issue 1 2007USHA B. TEDROW M.D. Background: Success of cardiac resynchronization therapy (CRT) depends on altering electrical ventricular activation (VA) to achieve mechanical benefit. That increases in stimulus strength (SS) can affect VA has been demonstrated previously in cardiomyopathy patients undergoing ablation. Objective: To determine whether increasing SS can alter VA during CRT. Methods: In 71 patients with CRT devices, left ventricle (LV) pacing was performed at escalating SS. Timing from pacing stimulus to right ventricular (RV) electrogram, ECG morphology, and maximal QRS duration on 12 lead ECG were recorded. Results: Demographics: Baseline QRS duration 153 ± 25 ms, ischemic cardiomyopathy 48%, ejection fraction 24%± 7%. With increased SS, conduction time from LV to right ventricle (RV) decreased from 125 ± 56 ms to 111 ± 59 ms (P = 0.006). QRS duration decreased from 212 ± 46 ms to 194 ± 42 ms (P = 0.0002). A marked change in QRS morphology occurred in 11/71 patients (15%). The RV ring was the anode in 6, while the RV coil was the anode in 5. Sites with change in QRS morphology showed decrease in conduction time from LV to RV from 110 ± 60 ms to 64 ± 68 ms (P = 0.04). Twelve patients (16%) had diaphragmatic stimulation with increased SS. Conclusions: Increasing LV SS reduces QRS duration and conduction time from LV to RV. Recognition of significant QRS morphology change is likely clinically important during LV threshold programming to avoid unintended VA change. [source] Transcoronary Ablation of Septal Hypertrophy Does Not Alter ICD Intervention Rates in High Risk Patients with Hypertrophic Obstructive CardiomyopathyPACING AND CLINICAL ELECTROPHYSIOLOGY, Issue 4 2005THORSTEN LAWRENZ Introduction: Transcoronary ablation of septal hypertrophy (TASH) is safe and effectively reduces the intraventricular gradient in patients with hypertrophic obstructive cardiomyopathy (HOCM). To analyze the potential of anti- and proarrhythmic effects of TASH, we studied the discharge rates of implanted cardioverter defibrillators (ICD) in patients with HOCM who are at a high risk for sudden cardiac death. Methods: ICD and TASH were performed in 15 patients. Indications for ICD-implantation were secondary prevention in nine patients after resuscitation from cardiac arrest with documented ventricular fibrillation (n = 7) or sustained ventricular tachycardia (n = 2) and primary prevention in 6 patients with a family history of sudden deaths, nonsustained ventricular tachycardia, and/or syncope. All the patients had severe symptoms due to HOCM (NYHA functional class = 2.9). Results: During a mean follow-up time of 41 ± 22.7 months following the TASH procedure, 4 patients had episodes of appropriate discharges (8% per year). The discharge rate in the secondary prevention group was 10% per year and 5% in the group with primary prophylactic implants. Three patients died during follow-up (one each of pulmonary embolism, stroke, and sudden death). Conclusion: In conclusion, on the basis of ICD-discharge rates in HOCM-patients at high risk for sudden death, there is no evidence for an unfavorable arrhythmogenic effect of TASH. The efficacy of ICD treatment for the prevention of sudden cardiac death in HOCM could be confirmed, however, mortality is high in this cohort of hypertrophic cardiomyopathy patients. [source] Autologous cellular cardiomyoplasty for pediatric dilated cardiomyopathy patients: New therapeutic option for children with failing heart?PEDIATRIC TRANSPLANTATION, Issue 2 2010Kimimasa Tobita MD No abstract is available for this article. [source] Dynamics of Ventricular Repolarization in Patients with Dilated Cardiomyopathy Versus Healthy SubjectsANNALS OF NONINVASIVE ELECTROCARDIOLOGY, Issue 2 2005Jose Luis Alonso M.D. Background: Patients with impaired left ventricular function have a high risk of developing ventricular arrhythmias and sudden death. Among different markers of risk, the prolongation and regional heterogeneity of repolarization are of increasing interest. However, there are limited data regarding feasibility of analyzing repolarization parameters and their dynamics in 24-hour Holter ECG recordings. Methods: Dynamic behavior of repolarization parameters was studied with a new automatic algorithm in digital 24-hour Holter recordings of 60 healthy subjects and 55 patients with idiopathic dilated cardiomyopathy (IDC). Repolarization parameters included the mean value of QT and QTc durations, QT dispersion, and peaks of QT duration and QT dispersion above prespecified thresholds. Results: In comparison to healthy subjects, patients with IDC had lower heart rate variability, longer mean QT and QTc durations, higher content of QTc peaks >500 ms, longer QT dispersion and its standard deviation, and a higher content of peaks >100 ms of QT dispersion (P < 0.01 for all comparisons). These repolarization parameters were significantly higher in IDC patients after adjustment for age, sex, and heart rate variability. The parameters of repolarization dynamics correlated with SDNN in healthy subjects but not in dilated cardiomyopathy patients. Conclusions: The automatic assessment of repolarization parameters in 24-hour digital ECG recordings is feasible and differentiates dilated cardiomyopathy patients from healthy subjects. Patients with dilated cardiomyopathy have increased QT duration, QT dispersion, and increased variability of QT dispersion reflecting variations in T-wave morphology, the factors which might predispose them to the development of arrhythmic events. 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