[Etiopathogenesis of arrhythmogenic right ventricular dysplasia].

Arhythmogenic right ventricular dysplasia (ARVD) is a genetically determined cardiomyopathy with a dominant transmission mode and variable penetrance. Transdifferenciation of cardiomyocytes into adipocytes is likely to explain massive replacement of right ventricular and to a lesser extent left ventricular myocardium by adipose tissue. This phenomenon starts in the mediomural layers and extends into the epicardium.

Prenatal echocardiographic appearance of arrhythmogenic right ventricle dysplasia: a case report.

We report a case of arrhythmogenic right ventricular dysplasia (ARVD) diagnosed prenatally by echocardiography at 24 weeks gestation. The 4-chamber view showed a large outpouched area extending from below the tricuspid valve to the insertion of the moderator band; the affected wall appeared thin and akinetic, with absence of flow at color Doppler investigation and no evidence of cardiovascular failure. The size of the outpouched area was unchanged at subsequent controls (25 and 26 weeks gestation) when frequent extrasystoles occurred, probably of a ventricular origin.

Fat in the heart. A feature unique to the human species? Observational reflections on an unsolved problem.

Fat that is well demarcated from underlying muscle is found on the right ventricular free wall and around epicardial coronary vessels. Fat is not present in the left ventricle in normal subjects. In right ventricular dysplasia, fat and fibrosis may massively displace right ventricular myocardial tissue.

Arrhythmogenic right ventricular dysplasia.

Arrhythmogenic right ventricular dysplasia (ARVD) is a new form of cardiomyopathy probably more frequent than commonly reported. It is a rare but important cause of sudden arrhythmic death in young, otherwise healthy persons, as well as a subtle cause of congestive heart failure. It may lead to temporary incapacitation with catastrophic consequences.

[Arrhythmogenic right ventricular dysplasia and cardiomyopathy. Clinical and anatomic-pathologic aspects, nosologic approach].

Arrhythmogenic right ventricular dysplasia is a polymorphous clinical entity. Its diagnosis is difficult in incomplete forms or at the onset of the disease. The diagnosis is based on the association of clinical, electrocardiographic and electrophysiologic signs which are the result of a specific pathological structure, consisting of fibromuscular bundles isolated from each other by fatty tissue resulting from apoptosis and/or the basic dysplastic phenomenon.

[Arrhythmogenic right ventricular dysplasia, torsades de pointes and sudden death. New concepts].

M cells as well as vortex like reentrant tachycardia could explain the torsade de pointes pattern leading to sudden death at night in a patient with arrhythmogenic right ventricular dysplasia and saddle-back ST segment elevation in lead V2. The mechanism of the torsade is explained by the two-dimensional structure of the right ventricular free wall reconstructed from paraffin blocks. This case may represent a particular form of Brugada's syndrome and cases of sudden death in young males in South East Asia.

Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study.

Objectives: The aim of the present investigation was to redefine the clinicopathologic profile of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC), with special reference to disease progression and left ventricular (LV) involvement.

Background: Long-term follow-up data from clinical studies indicate that ARVC is a progressive heart muscle disease that with time may lead to more diffuse right ventricular (RV) involvement and LV abnormalities and culminate in heart failure.

Methods: Forty-two patients (27 male, 15 female; 9 to 65 years old, mean [+/-SD] age 29.

Catastrophic global heart failure in a patient with non-arrhythmogenic right ventricular dysplasia.

A young patient with congestive heart failure had pathological findings of myocarditis superimposed on the substrate of a non-arrhythmogenic form of right ventricular dysplasia. The only clinical findings suggestive of right ventricular dysplasia were T-wave inversions on the right precordial leads.

Evidence of apoptosis in arrhythmogenic right ventricular dysplasia.

Background: Arrhythmogenic right ventricular dysplasia, a disorder that may lead to severe ventricular arrhythmias and sudden death, is characterized by the progressive replacement of myocardial cells by fat and fibrous tissue. We examined whether the loss of myocardial cells in this disease could result from cell death by apoptosis (programmed cell death).

Methods: Specimens obtained at autopsy from the right ventricular myocardium of eight patients with arrhythmogenic right ventricular dysplasia and four age-matched normal subjects were analyzed.

[Right precordial leads and sudden death. Relation with arrhythmogenic right ventricular dysplasia].

Non-coronary ST-segment elevation during right sided chest pain has been described in subjects with episodes of ventricular fibrillation at rest. This syndrome has been attributed to functional phenomena or to structural myocardial changes. A personal case has features belonging to two categories: ST-segment elevation observed before, during and after episodes of arrhythmia was compared to 11 previously recorded ECG recordings.

Progressive latissimus dorsi muscle denervation for free-flap dynamic cardiomyoplasty.

Background: The creation of free muscle grafts for surgical myoplasty is limited by the dependence of muscle on its original nerve supply. The aim of this study was to develop a model of gradual denervation of a large skeletal muscle (latissimus dorsi) and evaluate the possibility that atrophic degeneration and loss of function would be reduced using progressive nerve compression instead of surgical division of the nerve. The effects of chronic stimulation prior to, and after, denervation were also evaluated.

Liver preservation below 0 degrees C with UW solution and 2,3-butanediol.

Long-term preservation of the liver is needed to transform liver transplantation from an emergency operation to an elective procedure and therefore to improve the results of liver transplantation. We explored the possibility of extending the cold ischemia time of the rat liver by using a preservation temperature below 0 degrees C together with the addition of a cryoprotective agent (2,3-butanediol) at a low concentration in the preservation solution. Rat livers were preserved for 72 h either with UW solution at +4 degrees C (group 1) or with a UW solution, to which 2,3-butanediol at 8% (at +4 degrees C (group 2) or at -4 degrees C (group 3, experimental group)) was added.

[Arrhythmogenic right ventricle: dysplasia or cardiomyopathy? Value of left ventricle ejection fraction].

The left ventricular ejection fraction (LVEF) of 76 patients suffering from arrhythmogenic dysplasia or cardiomyopathy of the right ventricle (ventricular tachycardia associated with structural abnormalities of the right ventricle) demonstrated two subgroups situated above and below 45%. Values of LVEF less than 45% were similar to those of a control population of 6 cases of idiopathic dilated cardiomyopathy with ventricular tachycardia of left ventricular origin (p = 0.2).

[Outcome of arrhythmogenic right ventricular dysplasia. Apropos of 4 cases].

The authors propose a classification of the outcome of arrhythmogenic right ventricular dysplasia with reference to 4 selected cases with a follow-up period of over 9 years. In type I, the left ventricular ejection fraction is normal (EF > 50%) and the risk, exclusively arrhythmic, can be controlled by appropriate antiarrhythmic therapy. This is the commonest form of arrhythmogenic right ventricular dysplasia with different varieties according to the degree of dilatation of the right ventricle.