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  • 1
    Electronic Resource
    Electronic Resource
    Arrhythmogenic Right Ventricular Dyspiasia/Cardiomyopathy: (2000)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of cardiovascular electrophysiology 11 (2000), S. 0 
    Details
    ISSN: 1540-8167
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy. Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a heart muscle disease characterized by peculiar right ventricular involvement and electrical instability that precipitates ventricular arrhythmias and sudden death. The purpose of the present consensus report of the Study Group of the European Society of Cardiology and the Scientific Council on Cardiomyopathies of the World Heart Federation is to review the considerable progress in our understanding of the etiopathogenesis, morbid anatomy, and clinical presentation of ARVD/C since its first description in 1977. This article will focus on the important hut still unanswered issues, mostly regarding risk stratification, clinical outcome, and management of affected patients. Because ARVD/C is relatively uncommon and any one center may have experience with only a few patients, an international registry is being established to accumulate information and enhance the numbers of patients that can be analyzed to answer the pending questions. The registry also will facilitate pathologic, molecular, and genetics research on the etiology and pathogenesis of the disease. Furthermore, availability of an international database will enhance awareness of this largely unrecognized condition among the medical community. Physicians are encouraged to enroll patients in the International Registry of ARVD/C.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1540-8167.2000.tb00059.x
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  • 2
    Electronic Resource
    Electronic Resource
    Genetics of Right Ventricular Cardiomyopathy (2005)
    350 Main Street , Malden , MA 02148-5018 , USA , and 9600 Garsington Road , Oxford OX4 2DQ , UK . : Blackwell Science Inc
    Journal of cardiovascular electrophysiology 16 (2005), S. 0 
    Details
    ISSN: 1540-8167
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart muscle disease that may result in arrhythmia, heart failure, and sudden death. The hallmark pathological findings are progressive myocyte loss and fibrofatty replacement, with a predilection for the right ventricle. However, variants of ARVC that preferentially affect the left ventricle are increasingly recognized. ARVC is distinguished from dilated cardiomyopathy by a propensity toward ventricular arrhythmia and sudden death in the absence of significant ventricular dysfunction.In the majority of families, ARVC shows autosomal dominant inheritance with incomplete penetrance. Recessive forms are also described, often in association with cutaneous disorders. Causative mutations have so far been identified in plakoglobin, desmoplakin, and plakophilin, all of which encode key components of the desmosome. Desmosomes are protein complexes that anchor intermediate filaments to the cytoplasmic membrane in adjoining cells, thereby forming a three-dimensional scaffolding that provides tissues with mechanical strength. Unraveling of the genetic etiology of ARVC has elicited a new model for pathogenesis. Impaired functioning of cell adhesion junctions during exposure to shear stress may lead to myocyte detachment and death, accompanied by inflammation and fibrofatty repair. At least three mechanisms contribute to the arrhythmic substrate: bouts of myocarditis, fibrous and adipose infiltrates that facilitate macroreentry, and gap junction remodeling secondary to altered mechanical coupling. The latter may underlie arrhythmogenicity in early disease.Although ARVC can be considered a disease of the desmosome, a variety of other genetic defects give rise to phenocopies, which may ultimately enhance our understanding of the broad phenotypic spectrum.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1540-8167.2005.40842.x
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  • 3
    Electronic Resource
    Electronic Resource
    Pitfalls of Pacemaker Treatment for Hypertrophic Cardiomyopathy (1996)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of interventional cardiology 9 (1996), S. 0 
    Details
    ISSN: 1540-8183
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Permanent dual chamber pacing with a shortened AV delay has been increasingly utilized as a therapeutic approach to the problem of drug refractory symptomatic obstructive hypertrophic cardiomyopathy. Such patients are a small proportion of the total patient population, potentially limiting the applicability of pacing. Attention has focused on the effect of pacing on indices of systolic function, primarily the left ventricular outflow tract gradient. The effects of pacing on diastolic function are uncertain, although recent acute catheterization studies suggest an adverse effect. Studies have concentrated on subjective symptomatic improvement. The available data on objective improvement suggests modest improvements at best. The limitations of current pacemakers together with the role of adjunctive techniques such as radiofrequency (RF) ablation of the atrioventricular node and coronary sinus pacing are also reviewed. Current ongoing randomized trials of DDD pacing in hypertrophic cardiontyopathy will resolve many issues, although data comparing myectonty versus pacing is as yet unavailable.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1540-8183.1996.tb00649.x
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  • 4
    Electronic Resource
    Electronic Resource
    Is QT Dispersion Associated with Sudden Cardiac Death in Patients with Hypertrophic Cardiomyopathy? (2001)
    Oxford, UK : Blackwell Publishing Ltd
    Annals of noninvasive electrocardiology 6 (2001), S. 0 
    Details
    ISSN: 1542-474X
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: QT dispersion is significantly greater in patients with hypertrophic cardiomyopathy (HCM) than that in healthy subjects. Few data exist regarding the prognostic value of QT dispersion in HCM. In this study, we retrospectively investigated the association between QT dispersion and sudden cardiac death in 46 patients with HCM (mean 33.1 ±; 15.5 years, 32 men). The case group consisted of 23 HCM patients who died suddenly, and the control group consisted of 23 HCM patients who survived uneventfully during follow-up. Study patients were pair-matched for age, gender, and maximum left ventricular wall thickness. QT dispersion (maximum minus minimum QT interval) was manually measured on early 12-lead ECGs using a digitizing; board. An in-house program was used for calculating QT interval, QT dispersion, JT interval, and JT dispersion (maximum minus minimum J point to T end interval). Patients in the case group tended to have shorter RR intervals than those in the control group (777 ±; 171 vs 856 ±; 192 ms, P = 0.08). Maximum corrected QT and JT intervals did not discriminate the case group from controls (489 ±; 29 vs 479 ±; 27 ms, P = NS; 375 ±; 36 vs 366 ±; 22 ms, P = NS, respectively). Greater QT dispersion and JT dispersion were found in the case group compared with controls (74 ±; 28 vs 59 ±; 21 ms, P = 0.02 and 76 ±; 32 vs 59 ±; 26 ms, P = 0.03, respectively). The measurements of maximum QT, JT, and T peak to T end intervals, precordial QT and JT dispersion, and T peak and T end dispersion were all comparable between the two groups (P = NS for all). No systematic changes in ECG measurements were found from late ECGs of the case group compared to those from early ECGs (P = NS). No correlation between maximum left ventricular wall thickness and QT dispersion, JT dispersion, maximum QTc or JTc intervals was observed (r 〈 0.29, P 〉 0.05 for all). Our results; show that increased QT dispersion and JT dispersion is weakly associated with sudden cardiac death in the selected patients with HCM. A.N.E. 2001; 6(3):209–215
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1542-474X.2001.tb00110.x
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  • 5
    Electronic Resource
    Electronic Resource
    T Wave Complexity in Patients with Hypertrophic Cardiomyopathy (1998)
    Oxford, UK : Blackwell Publishing Ltd
    Pacing and clinical electrophysiology 21 (1998), S. 0 
    Details
    ISSN: 1540-8159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The complexity of the T wave assessed by principal component analysis (PCA) has been proposed to reflect obnormal repolarization, which may be arrhythmogenic. To determine whether PCA can differentiate patients with hypertrophic cardiomyopathy (HCM) from normal subfects and whether PCA is of prognostic importance in HCM, 112 patients with HCM (41 ±14 years, 64 males) and 72 healthy subjects (39 ± 9 years, 41 males) were studied. Patients with sinus node dysfunction, AV conduction block, flat T waves, QRS 〉 140 ms, and those 〈 15 years were excluded from this study. Standard 12-lead ECGs were recorded digitally using the MAC-VU system (Marquette Medical Systems). PCA parameters were computed using the QT Guard software package by Marquette. PCA ratio was significantly greater in HCM patients than in normal controls (23.9%± 12.4% vs 16.1%± 7.6%, P 〈 0.0001) and was correlated with QT-end dispersion (r = 0.24. P = 0.01) and QT peak (Q point to T peak) dispersion (r = 0.35, P 〈 0.0001). HCM patients with syncope (n = 23) had increased PCA ratios compared with those without syncope (29.1%± 11.5% vs 22.5%± 12.3%, P = 0.01). PCA ratio was similar in patients with and without nonsustained ventricular tachycardia on Holter (25.9%± 11.4% vs 22.7%± 12.1%, P = 0.2), as well as in patients treated with amiodarone or sotalol versus those not on therapy. In conclusion, assessment of the complexity of the T wave by PCA differentiates HCM patients from normal subjects. PCA ratio correlated with QT dispersion and an increased PCA ratio was associated with a history of syncope in HCM.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1540-8159.1998.tb01186.x
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  • 6
    Electronic Resource
    Electronic Resource
    Arrhythmias in Hypertrophic Cardiomyopathy (1991)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of cardiovascular electrophysiology 2 (1991), S. 0 
    Details
    ISSN: 1540-8167
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Arrhythmias in Hypertrophic Cardiomyopathy. Arrhythmias, particularly atrial fibrillation and nonsustained ventricular tachycardia, are common in adult patients with hypertrophic cardiomyopathy. Atrial fibrillation has long been thought to herald an ominous prognosis, but this is probably not the case, and in the majority of patients atrial fibrillation can be controlled without accelerated symptomatic deterioration. Uncontrolled observations indicate that low dose amiodarone may be the most useful drug in paroxysmal and chronic atrial fibrillation. The detection of nonsustained ventricular tachycardia on ambulatory electrocardiogram monitoring remains the single most useful indicator of the risk of sudden death in the adult patient, and the treatment of choice is again low dose amiodarone. The mechanism of sudden death, and the mode of action of amiodarone in preventing it, are not known for certain in the majority of patients. The risk of sudden death is higher in children and adolescents, but arrhythmias are less common, and no useful predictive marker of increased risk has been found. The role of invasive electrophysiological studies and the place of the implantable cardioverter-defibrillator in hypertrophic cardiomyopathy have not yet been established.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1540-8167.1991.tb01354.x
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  • 7
    Electronic Resource
    Electronic Resource
    Dual Chamber Pacing in Hypertrophic Cardiomyopathy: Long-Term Effects on Diastolic Function (2002)
    Oxford, UK : Blackwell Futura Publishing, Inc.
    Pacing and clinical electrophysiology 25 (2002), S. 0 
    Details
    ISSN: 1540-8159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: BETOCCHI, S., et al.: Dual Chamber Pacing in Hypertrophic Cardiomyopathy: Long-Term Effects on Diastolic Function. To assess the effects of chronic dual chamber pacing (DDD) on LV diastolic function in obstructive hypertrophic cardiomyopathy (HCM), 21 patients with obstructive HCM paced for refractory symptoms were studied at baseline and at 3 and 12 months. HCM patients were matched to 21 patients with obstructive HCM on conventional treatment. Left atrial fractional shortening was calculated by M-mode echocardiography; this index reflects LV end-diastolic pressure. LV outflow tract gradient decreased 65 ± 21% with DDD pacing and the NYHA class improved (P = 0.033). Left atrial fractional shortening worsened with DDD pacing (P 〈 0.001). Patients with abnormal baseline left atrial fractional shortening (〈 16%) were older, had a higher NYHA class, and had more severe mitral regurgitation. In this subgroup, left atrial fractional shortening did not worsen with DDD pacing and the NYHA class improved more than in patients with normal left atrial fractional shortening (P = 0.033). In conclusion, chronic DDD pacing reduces obstruction but impairs diastolic function in HCM. In patients with normal diastolic function, the untoward effects of pacing on diastolic function are more evident than in patients with abnormal diastolic function at baseline. This suggests that DDD pacing might be beneficial in a subgroup of patients with obstructive HCM and abnormal diastolic function.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1460-9592.2002.01433.x
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  • 8
    Electronic Resource
    Electronic Resource
    Mutations in the cardiac myosin binding protein–C gene on chromosome 11 cause familial hypertrophic cardiomyopathy (1995)
    [s.l.] : Nature Publishing Group
    Nature genetics 11 (1995), S. 434-437 
    Details
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Clinical evaluation of two unrelated families (designated NNand CD, Fig. 1) revealed individuals with classical features of FHC in both, two individuals with an incomplete FHC phenotype in Family NN and only one instance of sudden death in Family CD (see Methods for details). Genetic analyses in ...
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1038/ng1295-434
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  • 9
    Electronic Resource
    Electronic Resource
    Role of Invasive EP Testing in the Evaluation and Management of Hypertrophic Cardiomyopathies (2000)
    Springer
    Cardiac electrophysiology review 4 (2000), S. 86-88 
    Details
    ISSN: 1573-725X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1023/A:1009903622503
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  • 10
    Electronic Resource
    Electronic Resource
    Dilated cardiomyopathy: Pathogenesis and the recognition of early disease (1996)
    Springer
    Heart failure reviews 1 (1996), S. 87-96 
    Details
    ISSN: 1573-7322
    Keywords: dilated cardiomyopathy ; immunology ; pathogenesis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Idiopathic dilated cardiomyopathy causes longterm morbidity in young people and results in premature death. The underlying cause remains unknown, but there is now evidence of humoral and cellular immune activation in a proportion of patients and their asymptomatic relatives, raising the possibility of future targeted immunotherapy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00126373
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