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Topic: Arrhythmogenic right ventricular dysplasia


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In the News (Thu 24 Dec 09)

  
  Arrhythmogenic right ventricular dysplasia - Wikipedia, the free encyclopedia
Arrhythmogenic right ventricular dysplasia (ARVD, also known as arrhythmogenic right ventricular cardiomyopathy or ARVC) is a type of nonischemic cardiomyopathy that involves primarily the right ventricle.
It is characterized by hypokinetic areas involving the free wall of the right ventricle, with fibrofatty replacement of the right ventricular myocardium, with associated arrhythmias originating in the right ventricle.
Ventricular ectopy seen on a surface EKG in the setting of ARVD is typically of left bundle branch block (LBBB) morphology, with a QRS axis of -90 to +110 degrees.
en.wikipedia.org /wiki/Arrhythmogenic_right_ventricular_dysplasia   (2306 words)

  
 Multidisciplinary Study of Right Ventricular Dysplasia
he Multidisciplinary Study of Right Ventricular Dysplasia is a multidisciplinary, multicenter, collaborative study to investigate the cardiac, clinical, and genetic aspects of arrhythmogenic right ventricular dysplasia.
ARVD is a disorder that predominantly affects the right side of the heart and causes ventricular arrhythmias.
ARVD may account for as many as 5% of unexpected sudden deaths under the age of 65 and 3-4% of sudden death during sports.
www.arvd.org   (328 words)

  
 [No title]
Arrhythmogenic right ventricular dysplasia (ARVD) is a poorly understood and often underdiagnosed cardiomyopathy.
ARVD should be considered as a cause of ventricular tachycardia of left bundle branch block configuration and/or sudden death, particularly during exercise in young subjects.
Echocardiographic studies focusing on the size and wall-motion abnormalities of the right ventricle are useful in confirming the diagnosis.
telethon.bio.unipd.it /ARVDnet/arvd_clinical.html   (160 words)

  
 ARVD   (Site not responding. Last check: 2007-10-19)
Ventricular arrhythmias ranging from symptomatic or asymptomatic isolated extrasystoles to sustained poorly tolerated ventricular tachycardia of right ventricular origin with a left bundle-branch block pattern, are the most common form of presentation (4,11).
Arrhythmogenic right ventricular cardiomyopathy: dysplasia, dystrophy, or myocarditis?.
Right ventricular arrhythmia in the absence of arrhythmogenic dysplasia: MR imaging of myocardial abnormalities.
heartmri.com /applications/arvd.htm   (1896 words)

  
 Children's Cardiomyopathy Foundation - Description Of Disorder   (Site not responding. Last check: 2007-10-19)
Other forms of hypertrophy are symmetric ventricular hypertrophy (thickening is evenly distributed throughout the ventricle including the septum and wall) and apical hypertrophy (thickening is localized at the bottom portion of the heart).
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a very rare condition, and almost unheard of in children.
In the early stages of the disease, the right side of the heart may appear to be thickened in certain patches but later becomes dilated with a thinner wall.
www.childrenscardiomyopathy.org /main/description.htm   (1928 words)

  
 Electrophysiological characteristics and outcome in patients with idiopathic right ventricular arrhythmia compared with ...
The induction of polymorphic ventricular tachycardia or ventricular
In right ventricular dysplasia, the intracavitary recording shows an isolated and fragmented diastolic potential (-158 ms with respect to QRS onset), while the earliest presystolic activity for idiopathic premature beat is recorded at the ablation site 20 ms before the QRS.
Ventricular dimensions and wall motion assessed by echocardiography in patients with arrhythmogenic right ventricular dysplasia.
heart.bmjjournals.com /cgi/content/full/87/1/41   (4415 words)

  
 CHEST: Treatment of presumed arrhythmogenic right ventricular dysplasia in an adolescent   (Site not responding. Last check: 2007-10-19)
Familial arrhythmogenic right ventricular dysplasia is a rare cardiomyopathy that is usually diagnosed on postmortem examination or on presentation with progressive congestive heart failure.
Right ventricular cardiomyopathy or Fontaine's ARVD has been documented as a rare cause of sudden death.[1,2,3,8] It has been shown to have a genetic pattern that is autosomal dominant with variable penetrance and variable expression.[8]
Fatty infiltration is characterized by accumulation of lipids in the interstitium of the myocardium, particularly within the right ventricle.[14] Fatty infiltration may be distinguished from ARVD by several features, including the interstitial location of the fatty tissue, and the absence of inflammation, myofiber degeneration, or fibrosis.
www.findarticles.com /p/articles/mi_m0984/is_n1_v104/ai_14105741   (1113 words)

  
 Clinical relevance of right ventricular dysplasia/cardiomyopathy -- PINAMONTI et al. 83 (1): 9 -- Heart
Its pathological hallmark is the atrophy of myocytes with fatty or fibro-fatty infiltration of the right ventricle.
The spectrum of clinico-pathologic manifestations of right ventricular cardiomyopathy/dysplasia.
Right ventricular dysplasia: a clinical and pathological study of two families with left ventricular involvement.
www.heartjnl.com /cgi/content/full/83/1/9   (1541 words)

  
 ARVD1 - arrhythmogenic right ventricular dysplasia 1
Regional abnormalities of the right ventricular wall also were more pronounced in patients with ARVD 1 than in patients with ARVD 2.
Signal-intensity increases corresponding to fatty replacement shown by biopsy were seen in 33% of patients with ARVD 1 and in 11% of patients with ARVD 2.
In humans, TAIL1 gene is located on chromosome 14q24.3 within ARVD1 (arrhythmogenic right ventricular dysplasia/cardiomyopathy, type 1) critical region; preliminary evidence suggests that it is expressed in several tissues, showing multiple alternative splicing.
www.ihop-net.org /UniPub/iHOP/gg/86548.html   (739 words)

  
 ARVD3 - arrhythmogenic right ventricular dysplasia 3   (Site not responding. Last check: 2007-10-19)
Right ventricular ejection fraction was significantly lower in patients with ARVD 1 than in patients with ARVD 2 or in control subjects.
In addition, RyR2 mutations were identified in patients affected with a variant form of arrhythmogenic right ventricular dysplasia (ARVD2), a phenotypically distinct disease entity.
Arrhythmogenic right ventricular dysplasia/cardiomyopathy type 2 (ARVD2, OMIM 600996) and stress-induced polymorphic ventricular tachycardia (VTSIP, OMIM 604772) are two cardiac diseases causing juvenile sudden death, both associated with mutations in the RyR2 calcium channel.
www.pdg.cnb.uam.es /UniPub/iHOP/gg/86550.html   (492 words)

  
 Arrhythmogenic right ventricular dysplasia. An illustrated review highlighting developments in the diagnosis and ...
ARVD is characterised by the deposition of adipose tissue and the development of fibrosis within the right ventricular myocardium
Electrophysiologic characteristics in arrhythmogenic right ventricular dysplasia and dilated cardiomyopathies.
Noninvasive recognition of the parchment right ventricle (Uhl's anomaly arrhythmogenic right ventricular dysplasia) syndrome.
www.postgradmedj.com /cgi/content/full/76/897/395   (2017 words)

  
 ARVD3 - arrhythmogenic right ventricular dysplasia 3   (Site not responding. Last check: 2007-10-19)
A new locus for arrhythmogenic right ventricular cardiomyopathy (ARVD2) maps to chromosome 1q42-q43.
Identification of mutations in the cardiac ryanodine receptor gene in families affected with arrhythmogenic right ventricular cardiomyopathy type 2 (ARVD2).
Arrhythmogenic right ventricular dysplasia type 2 (ARVD2, OMIM 600996) is an autosomal dominant cardiomyopathy, characterized by partial degeneration of the myocardium of the right ventricle, electrical instability and sudden death.
www.pdg.cnb.uam.es /UniPub/iHOP/gi/86550.html   (84 words)

  
 Diagnosis of Arrhythmogenic Right Ventricular Dysplasia: A Review -- Kayser et al. 22 (3): 639 -- RadioGraphics
are (a) fatty infiltration of the right ventricular myocardium
Strain J. Adipose dysplasia of the right ventricle: is endomyocardial biopsy useful?.
Right ventricular outflow tract ventricular tachycardia: detection of previously unrecognized anatomic abnormalities using cine magnetic resonance imaging.
radiographics.rsnajnls.org /cgi/content/full/22/3/639   (3624 words)

  
 Indian Pacing and Electrophysiology Journal - Indik   (Site not responding. Last check: 2007-10-19)
Right ventricular late potentials in the form of epsilon waves may be found on the routine 12 lead ECG.
Evidence of right ventricular parietal block is a QRS duration that is longer in the right (leads V1, V2 and V3) than in the left (V4, V5, V6) precordial leads.
Right ventricular outflow tract tachycardia (RVOT), must be excluded as this diagnosis carries a benign prognosis and is not hereditary5.
www.ipej.org /0303/indik.htm   (3415 words)

  
 ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA (ARVD) IN CHILDHOOD: CASE REPORT WITH A REVIEW OF THE LITERATURE
The diagnosis of ARVD in the past was based on the presence of ventricular arrhythmia with left bundle branch block configuration and morphologic changes or motion abnormalities in the free wall of the right ventricle.
The two-dimensional echo-cardiography demonstrated huge dilation of the right atrium, congestion of the inferior vena cava and the hepatic vein, and moderate dilation of the right ventricle.
It remains unclear whether this genetic background predisposes to a degenerative disease with atrophy and fibrofatty replacement of the right ventricular myocardium, or whether the inflammatory cells seen in approximately 25% of cases indicate an infectious or possibly genetically determined immune pathogenesis.
www.kfshrc.edu.sa /annals/173/96-199.html   (1895 words)

  
 Chest: Treatment of presumed arrhythmogenic right ventricular dysplasia in an adolescent.@ HighBeam Research   (Site not responding. Last check: 2007-10-19)
Treatment of presumed arrhythmogenic right ventricular dysplasia in an adolescent.
Chest; 7/1/1993; Gold, Jeffrey P. Familial arrhythmogenic right ventricular dysplasia is a rare cardiomyopathy that is usually diagnosed on postmortem examination or on presentation with progressive congestive heart failure.
Right ventricular cardiomyopathy or arrhythmogenic right ventricular dysplasia (ARVD) is a known, albeit rare, cause of sudden death in young people.
highbeam.com /library/doc0.asp?docid=1G1:14105741&refid=ink_tptd_mag   (181 words)

  
 Penn State Faculty Research Expertise Database (FRED)   (Site not responding. Last check: 2007-10-19)
Arrhythmogenic right ventricular dysplasia: cardiomyopathy current opinions on diagnostic and therapeutic aspects.
Right Ventricular Dysplasia constitutes a genetic cardiomyopathy characterized by fibrous-adipose substitution of the right and rarely of the left ventricular myocardium.
This disorder is associated with ventricular arrhythmias ranging from frequent ventricular ectopic beats, nonsustained and sustained ventricular tachycardia of left bundle branch morphology and sudden death.
fred.hmc.psu.edu /ds/retrieve/fred/publication/11124713   (168 words)

  
 ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA/CARDIOMYOPATHY   (Site not responding. Last check: 2007-10-19)
The current concepts with respect to one of the most prevailing origins of development of the paroxysmal and sustained right-ventricular tachycardias, namely: arrhythmogenic dysplasia/cariomyopathy of the right ventricle are considered in the paper.
A peculiar attention is paid for the criteria of the arrhythmogenic dysplasia diagnosis as well as the differential diagnosis of various non-coronarogenic ventricular tachycardias.
The authors give their own data when discussing the problem of diagnosis (differential diagnosis) and treatment of ventricular tachyarrhythmias in the patients with the right ventricle arrhythmogenic dysplasia.
www.incart.spb.ru /vestnic/n20/rez03eng.htm   (117 words)

  
 Cardiomyopathy Association - Arrhythmogenic Right Ventricular Cardiomyopathy
Arrhythmogenic is an electrical disturbance of the heart which tends to affect the Right Ventricle Figure 8 more than the left ventricle.
If the heart is examined, the right side of the heart may appear to be thickened early in the disease, but later it may become dilated (enlarged) with a thinner wall.
Because the disease affects the right side of the heart more than the left, features of 'right heart failure' may be more common and include prominent visible veins in the neck, distension of the liver, which may lead to a sensation of fullness or discomfort in the upper abdomen, and swollen legs or ankles.
www.cardiomyopathy.org /html/which_card_arvc.htm   (5148 words)

  
 Ventricle (heart)
In the heart, a ventricle is a chamber which collects blood from an atrium (another heart chamber) and pumps it out of the heart.
In a four-chambered heart, such as that in humans, there are two ventricles: the right ventricle pumps blood into the pulmonary circulation for the lungs, and the left ventricle pumps blood into the systemic circulation for the rest of the body.
Ventricular assist device (VAD), a temporary 'blood-pumping' device for patient use while the heart is 'healing' or 'being fixed'
www.brainyencyclopedia.com /encyclopedia/v/ve/ventricle__heart_.html   (160 words)

  
 ARVD - Page 1 - HeartCenterOnline:
Studies have shown that ARVD is a significant cause of sudden cardiac death among young athletes.
In the meantime, treatment for ARVD is aimed at controlling the arrhythmias that could lead to a life–threatening cardiac event.
Patients are encouraged to contact the ARVD Registry of North America for further information about the disease and about contacting others with the same condition.
heart.healthcentersonline.com /cardiomyopathy/arvd.cfm   (420 words)

  
 Arrhythmogenic Right Ventricular Dysplasia   (Site not responding. Last check: 2007-10-19)
Arrhythmogenic right ventricular dysplasia (ARVD) is a type of cardiomyopathy which is diagnosed on the basis of number of criteria which include structural, functional, electrocardiographic, arrhythmic, and genetic factors.
ARVD is recognized as a major cause of sudden death in adolescents, with one series reporting that it accounted for 20 percent of sudden deaths in all individuals younger than 35 years and 22 percent of sudden deaths in young atheletes.
The diagnosis of ARVD may have important consequences for direct relatives, because they have an increased change of having the disease, with an increased risk of sudden death.
www.cvimaging.northwestern.edu /physician/mriind/dysplas.cfm   (514 words)

  
 NEJM -- Right ventricular cardiomyopathy and sudden death in young people
Two main histologic patterns were identified--a lipomatous transformation or a fibrolipomatous transformation of the right ventricular free wall (6 cases each); in all cases, the left ventricle was substantially spared.
These findings indicate that right ventricular cardiomyopathy, the cause of which is still unknown, may be more frequent than previously thought.
Signal-averaged electrocardiogram in patients with arrhythmogenic right ventricular cardiomyopathy and ventricular arrhythmias.
content.nejm.org /cgi/content/abstract/318/3/129   (2378 words)

  
 Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy -- Gear and Marcus 107 (4): 31 -- Circulation   (Site not responding. Last check: 2007-10-19)
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a cardiomyopathy that primarily affects the heart muscle
An illustration of fatty replacement of cardiac muscle within the right ventricular wall.
The Multidisciplinary Study of Right Ventricular Dysplasia is
circ.ahajournals.org /cgi/content/full/107/4/e31   (1134 words)

  
 Glossary of Cardiovascular Terms - Texas Heart Institute Heart Information Center
- ARVD is a type of cardiomyopathy with no known cause.
An RVAD does not replace the heart—it "assists" or "helps" it pump oxygen-poor blood from the right ventricle to the lungs.
The node is a group of specialized cells in the top of the right atrium which produces the electrical impulses that travel down to eventually reach the ventricular muscle, causing the heart to contract.
www.tmc.edu /thi/glossary.html   (6155 words)

  
 Energy Citations Database (ECD) - Energy and Energy-Related Bibliographic Citations   (Site not responding. Last check: 2007-10-19)
A new locus for arrhythmogenic right ventricular dysplasia on the long arm of chromosome 14
Familial arrhythmogenic right ventricular cardiomyopathy or dysplasia (ARVD) is an idiopathic heart muscle disease with an autosomal-dominant pattern of transmission, characterized by fibro-fatty replacement of the right ventricular myocardium and ventricular arrhythmias.
In the present study, three unrelated families with ARVD were investigated.
www.osti.gov /energycitations/product.biblio.jsp?osti_id=501816   (286 words)

  
 Molecular Mechanisms of Inherited Cardiomyopathies -- Fatkin and Graham 82 (4): 945 -- Physiological Reviews
The development of left ventricular hypertrophy has been generally considered to be an adaptive response to biomechanical
Left ventricular hypertrophy may be "physiological" in elite athletes or occur in pathological states, such as hypertension,
mice, left ventricular hypertrophy is observed in 100% of mice
physrev.physiology.org /cgi/content/full/82/4/945   (9059 words)

  
 Pathology of arrhythmogenic right ventricular cardiomyopathy/dysplasia—An autopsy study of 20 forensic cases
Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC) is characterized histologically by massive infiltration of the right ventricular wall by fat tissue, with surviving strands of cardiomyocytes bordered by or embedded in fibrosis.
ARVC has been recognized as a cause of sudden death, especially in the young.
Author Fornes P, Ratel S, Lecomte D Title Pathology of arrhythmogenic right ventricular cardiomyopathy/dysplasia—An autopsy study of 20 forensic cases Symposium, Committee on
www.astm.org /JOURNALS/FORENSIC/PAGES/2847.htm   (325 words)

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