scholarly journals Survival of patients with cardiomyopathies

2021 ◽  
Vol 27 (4) ◽  
pp. 7-15
Author(s):  
Monika Shumkova ◽  
Kiril Karamfiloff ◽  
Raya Ivanova ◽  
Kristina Stoyanova ◽  
Dobrin Vassilev
Keyword(s):  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Myocardial Damage ◽  
Restrictive Cardiomyopathy ◽  
Genetic Mutations ◽  
Ventricular Cardiomyopathy ◽  
Pathogenetic Mechanism ◽  
Dilative Cardiomyopathy ◽  
Factors Associated ◽  
Different Types ◽  
Life Threatening

Cardiomyopathies are a heterogeneous group of diseases. The main pathogenetic mechanism is myocardial damage due to genetic mutations. Cardiomyopathies are one of the leading causes of heart failure, sudden cardiac death, and life-threatening arrhythmias. Certain factors associated with poor prognosis determined the prognosis in this group of patients. Survival in different types of cardiomyopathies depends on the time of diagnosis and initial treatment. The types of cardiomyopathies discussed in this review are hypertrophic cardiomyopathy, dilative cardiomyopathy, restrictive cardiomyopathy, left ventricle non-compaction, and arrhythmogenic right ventricular cardiomyopathy.

Restrictive cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy

2016 ◽  
Author(s):  
Perry Elliott ◽  
Kristina H. Haugaa ◽  
Pio Caso ◽  
Maja Cikes
Keyword(s):  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Restrictive Cardiomyopathy ◽  
Left Ventricular ◽  
Muscle Disease ◽  
Left Ventricular Volumes ◽  
Ventricular Cardiomyopathy ◽  
Ventricular Wall Thickness ◽  
Muscle Disorder ◽  
Desmosomal Protein

Restrictive cardiomyopathy is a heart muscle disorder characterized by increased myocardial stiffness that results in an abnormally steep rise in intraventricular pressure with small increases in volume in the presence of normal or decreased diastolic left ventricular volumes and normal ventricular wall thickness. The disease may be caused by mutations in a number of genes or myocardial infiltration. Arrhythmogenic right ventricular cardiomyopathy is an inherited cardiac muscle disease associated with sudden cardiac death, ventricular arrhythmias, and cardiac failure. It is most frequently caused by mutations in desmosomal protein genes that lead to fibrofatty replacement of cardiomyocytes, right ventricular dilatation, and aneurysm formation.

Heart muscle diseases

2012 ◽  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Right Ventricular ◽  
Dilated Cardiomyopathy ◽  
Fabry Disease ◽  
Heart Muscle ◽  
Cardiac Amyloidosis ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Restrictive Cardiomyopathy ◽  
Left Ventricular ◽  
Ventricular Cardiomyopathy

Classification 418Dilated cardiomyopathy 420Dilated cardiomyopathy: treatment 422Hypertrophic cardiomyopathy 424Hypertrophic cardiomyopathy: investigations 428Hypertrophic cardiomyopathy: treatment 430Restrictive cardiomyopathy 432Cardiac amyloidosis 434Cardiac amyloidosis: treatment 436Fabry disease 438Arrhythmogenic right ventricular cardiomyopathy (ARVC) 440ARVC: management 442Left ventricular non-compaction ...

scholarly journals Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC/D): A Systematic Literature Review

2013 ◽  
Vol 7 ◽  
pp. CMC.S10940 ◽  
Author(s):  
Jorge Romero ◽  
Eliany Mejia-Lopez ◽  
Carlos Manrique ◽  
Richard Lucariello
Keyword(s):  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Young Athlete ◽  
Ventricular Cardiomyopathy ◽  
Icd Implantation ◽  
Life Threatening ◽  
The Right ◽  
Ablative Procedures ◽  
Myocardial Atrophy ◽  
Genetic Form

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a genetic form of cardiomyopathy (CM) usually transmitted with an autosomal dominant pattern. It primary affects the right ventricle (RV), but may involve the left ventricle (LV) and culminate in biventricular heart failure (HF), life threatening ventricular arrhythmias and sudden cardiac death (SCD). It accounts for ll%-22% of cases of SCD in the young athlete population. Pathologically is characterized by myocardial atrophy, fibrofatty replacement and chamber dilation. Diagnosis is often difficult due to the nonspecific nature of the disease and the broad spectrum of phenotypic variations. Therefore consensus diagnostic criteria have been developed and combined electrocardiography, echocardiography, cardiac magnetic resonance imaging (CMRI) and myocardial biopsy. Early detection, family screening and risk stratification are the cornerstones in the diagnostic evaluation. Implantable cardioverter-defibrillator (ICD) implantation, ablative procedures and heart transplantation are currently the main therapeutic options.

scholarly journals Angiotensin-Converting Enzyme Inhibitors/Angiotensin Receptor Blockers: Anti-arrhythmic Drug for Arrhythmogenic Right Ventricular Cardiomyopathy

2021 ◽  
Vol 8 ◽  
Author(s):  
Bin Tu ◽  
Lingmin Wu ◽  
Lihui Zheng ◽  
Shangyu Liu ◽  
Lishui Sheng ◽  
...  
Keyword(s):  
Ventricular Arrhythmia ◽  
Right Ventricular ◽  
Disease Progression ◽  
Mixed Model ◽  
Linear Mixed Model ◽  
Angiotensin Receptor Blockers ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Cardiomyopathy ◽  
Life Threatening

Background: Current treatment guidelines for arrhythmogenic right ventricular cardiomyopathy (ARVC) mainly emphasize on prevention of ventricular arrhythmic events. Despite the progressive nature of ARVC, therapeutic options focusing on decelerating disease progression are scarce.Methods and Results: This retrospective observational cohort study included 311 patients [age, 39.1 ± 14.4 years; male, 233 (74.9%)] with a definite diagnosis of ARVC as determined by the 2010 Task Force Diagnostic Criteria. Among them, 113 patients (36.3%) received ACEI/ARB treatment. Disease progression was evaluated according to repeat transthoracic echocardiograms with a linear mixed model. Patients receiving ACEI/ARB treatment were associated with slower disease progression reflected by a gradual decrease in tricuspid annular plane systolic excursion than those not receiving ACEI/ARB treatment (0.37 vs. 0.61 mm per year decrease, P < 0.001) and slower dilation of right ventricular outflow tract (0.57 vs. 1.06 mm per year increased, P = 0.003). Cox proportional hazard regression models were used to evaluate the association between life-threatening ventricular tachycardia events and ACEI/ARB treatment. A reduced risk of life-threatening ventricular arrhythmia was associated with ACEI/ARB treatment compared to that without ACEI/ARB treatment (adjusted HR: 0.71, 95% CI: 0.52–0.96, P = 0.031).Conclusions: ACEI/ARB treatment is associated with slower disease progression and lower risk of life-threatening ventricular arrhythmia in patients with ARVC. Delaying disease progression may pave way for reducing life-threatening ventricular arrhythmia risk.

scholarly journals Arrhythmogenic right ventricular cardiomyopathy: asymptomatic to life threatening as illustrated by the cases of two sisters

2007 ◽  
Vol 15 (10) ◽  
pp. 348-353 ◽  
Author(s):  
L. C. Otterspoor ◽  
C. L. A. Reichert ◽  
M. J. M. Cramer ◽  
Z. A. Bhuiyan ◽  
A. A. M. Wilde ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Life Threatening

scholarly journals Myocyte necrosis underlies progressive myocardial dystrophy in mouse dsg2-related arrhythmogenic right ventricular cardiomyopathy

2009 ◽  
Vol 206 (8) ◽  
pp. 1787-1802 ◽  
Author(s):  
Kalliopi Pilichou ◽  
Carol Ann Remme ◽  
Cristina Basso ◽  
Maria E. Campian ◽  
Stefania Rizzo ◽  
...  
Keyword(s):  
Transgenic Mice ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Fibrous Tissue ◽  
Myocardial Damage ◽  
Dominant Negative ◽  
Dominant Negative Effect ◽  
Ventricular Cardiomyopathy ◽  
Negative Effect ◽  
Explanted Heart

Mutations in the cardiac desmosomal protein desmoglein-2 (DSG2) are associated with arrhythmogenic right ventricular cardiomyopathy (ARVC). We studied the explanted heart of a proband carrying the DSG2-N266S mutation as well as transgenic mice (Tg-NS) with cardiac overexpression of the mouse equivalent of this mutation, N271S-dsg2, with the aim of investigating the pathophysiological mechanisms involved. Transgenic mice recapitulated the clinical features of ARVC, including sudden death at young age, spontaneous ventricular arrhythmias, cardiac dysfunction, and biventricular dilatation and aneurysms. Investigation of transgenic lines with different levels of transgene expression attested to a dose-dependent dominant-negative effect of the mutation. We demonstrate for the first time that myocyte necrosis is the key initiator of myocardial injury, triggering progressive myocardial damage, including an inflammatory response and massive calcification within the myocardium, followed by injury repair with fibrous tissue replacement, and myocardial atrophy. These observations were supported by findings in the explanted heart from the patient. Insight into mechanisms initiating myocardial damage in ARVC is a prerequisite to the future development of new therapies aimed at delaying onset or progression of the disease.

scholarly journals Arrhythmogenic left ventricular cardiomyopathy

2020 ◽  
Vol 6 (1) ◽  
pp. 20190079
Author(s):  
Seyedeh Mojdeh Mirmomen ◽  
Andrew Jay Bradley ◽  
Andrew Ernest Arai ◽  
Arlene Sirajuddin
Keyword(s):  
Right Ventricular ◽  
Cardiac Death ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Myocardium ◽  
Left Ventricular ◽  
Ventricular Cardiomyopathy ◽  
Cardiotoxic Effect ◽  
Life Threatening ◽  
Muscle Disorder ◽  
The Right

Arrhythmogenic ventricular cardiomyopathy (AVC) is a heritable heart muscle disorder characterized by fibrofatty infiltration of the myocardium. Intramyocardial fat deposition is considered arrhythmogenic and predisposes patients to life-threatening arrhythmias and sudden cardiac death. The classic subtype of AVC is characterized by fibrofatty replacement of the right ventricular myocardium (i.e. arrhythmogenic right ventricular cardiomyopathy). In advanced cases of arrhythmogenic right ventricular cardiomyopathy, the left ventricle may be involved as well. Predominantly left ventricular involvement by AVC is exceedingly rare and lack of specific diagnostic criteria as well as its potential cardiotoxic effect make its diagnosis challenging and of high importance.

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