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| {{Infobox_Disease | | | __NOTOC__ |
| Name = {{PAGENAME}} |
| | {{Infobox disease |
| Image = Epsilon2.jpg |
| | | Name = Arrhythmogenic right ventricular dysplasia |
| Caption = |
| | | Image = Arvd_histo.jpg |
| DiseasesDB = 29750 |
| | | Caption = Photomicrograph of an ARVC heart. |
| ICD10 = {{ICD10|I|42|8|i|30}} |
| | | DiseasesDB = 29750 |
| ICD9 = |
| | | ICD10 = {{ICD10|I|42|8|i|30}} |
| ICDO = |
| | | ICD9 = |
| OMIM = 107970 |
| | | ICDO = |
| MedlinePlus = | | | | OMIM = 107970 |
| eMedicineSubj = |
| | | MedlinePlus = |
| eMedicineTopic = |
| | | eMedicineSubj = |
| MeshID = D019571 |
| | | eMedicineTopic = |
| | | MeshID = D019571 |
| }} | | }} |
| {{SI}} | | {{Arrhythmogenic right ventricular dysplasia}} |
| {{CMG}} | | {{CMG}} |
| __NOTOC__
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| '''Associate Editor-In-Chief:''' {{CZ}}
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| '''''Synonyms and Keywords:''''' ARVD, arrhythmogenic right ventricular cardiomyopathy or ARVC
| | {{SK}} Arrhythmogenic right ventricular cardiomyopathy; arrhythmogenic right ventricular cardiomyopathy-dysplasia; arrhythmogenic RV dysplasia; ARVC; ARVC/D; ARVD; ARVD/C |
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| ==Overview== | | ==[[Arrhythmogenic right ventricular dysplasia overview|Overview]]== |
| Arrhythmogenic right ventricular dysplasia 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 [[arrhythmia]]s originating in the right ventricle. | |
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| ==Epidemiology and Demographics== | | ==[[Arrhythmogenic right ventricular dysplasia historical perspective|Historical Perspective]]== |
| ARVD is an important cause of [[ventricular arrhythmia]]s in children and young adults. It is seen predominantly in males, and 30-50% of cases have a familial distribution.
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| ==Genetics== | | ==[[Arrhythmogenic right ventricular dysplasia classification|Classification]]== |
| It is usually inherited in an [[autosomal dominant]] pattern, with variable expression. The [[penetrance]] is 20-35% in general, but significantly higher in Italy. Seven gene loci have been implicated in ARVD. However, about 50% of families that express ARVD that undergo genetic screening do not show linkage with any of the known [[chromosome | chromosomal]] [[allele | loci]]. It is unclear whether the pathogenesis varies with the different loci involved. A standard genetic screening test is not available.
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| ==Differential Diagnosis== | | ==[[Arrhythmogenic right ventricular dysplasia pathophysiology|Pathophysiology]]== |
| The differential diagnosis for the ventricular tachycardia due to ARVD include:
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| * [[Congenital heart disease]]
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| ** Repaired [[tetralogy of Fallot]]
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| ** [[Ebstein's anomaly]]
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| ** [[Uhl anomaly]]
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| ** [[Atrial septal defect]]
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| ** Partial anomalous venous return
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| * Acquired heart disease
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| ** [[Tricuspid valve disease]]
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| ** [[Pulmonary hypertension]]
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| ** [[Right ventricular infarction]]
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| ** Bundle-branch re-entrant tachycardia
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| * Miscellaneous
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| ** Pre-excited AV re-entry tachycardia
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| ** Idiopathic RVOT tachycardia
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| == Naxos Disease == | | ==[[Arrhythmogenic right ventricular dysplasia causes|Causes]]== |
| [[Image:Naxos disease.jpg|thumb|left|300px|Cutaneous [[phenotype]] of Naxos disease: woolly hair (A), palmar (B) and plantar (C) keratoses.]] | |
| '''Naxos disease''' is an [[autosomal recessive]] variant of ARVD, described initially on the Greek island of Naxos. There, the [[penetrance]] is >90%.
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| It involves the gene that codes for plakoglobin (a protein that is involved in cellular adhesion), on [[chromosome]] 17p.
| | ==[[Arrhythmogenic right ventricular dysplasia differential diagnosis|Differentiating Arrhythmogenic Right Ventricular Dysplasia from Other Diseases]]== |
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| Naxos disease is described as a triad of ARVD, palmoplantar [[keratosis]], and wooly hair.
| | ==[[Arrhythmogenic right ventricular dysplasia epidemiology and demographics|Epidemiology and Demographics]]== |
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| The signs of Naxos disease are more severe than with autosomal dominant ARVD.
| | ==[[Arrhythmogenic right ventricular dysplasia risk factors|Risk Factors]]== |
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| == Presentation == | | ==[[Arrhythmogenic right ventricular dysplasia screening|Screening]]== |
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| Up to 80% of individuals with ARVD present with [[syncope]] or sudden cardiac death. The remainder frequently present with palpitations or other symptoms due to right ventricular outflow tract (RVOT) tachycardia (a type of [[monomorphic ventricular tachycardia]]).
| | ==[[Arrhythmogenic right ventricular dysplasia natural history|Natural History, Complications and Prognosis]]== |
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| Symptoms are usually exercise-related. In populations where [[hypertrophic cardiomyopathy]] is screened out prior to involvement in competitive athletics, it is a common cause of sudden cardiac death. | | ==Diagnosis== |
| | :[[Arrhythmogenic right ventricular dysplasia diagnostic criteria|Diagnostic Criteria]] | [[Arrhythmogenic right ventricular dysplasia history and symptoms|History and Symptoms]] | [[Arrhythmogenic right ventricular dysplasia physical examination|Physical Examination]] | [[Arrhythmogenic right ventricular dysplasia laboratory tests|Laboratory Tests]] | [[Arrhythmogenic right ventricular dysplasia x ray|X-ray]] | [[Arrhythmogenic right ventricular dysplasia ECG|ECG]] | [[Arrhythmogenic right ventricular dysplasia cardiac MRI|Cardiac MRI]] | [[Arrhythmogenic right ventricular dysplasia echocardiogram|Echocardiogram]] | [[Arrhythmogenic right ventricular dysplasia other imaging findings|Other Imaging Findings]] | [[Arrhythmogenic right ventricular dysplasia endomyocardial biopsy|Endomyocardial biopsy]] | [[Arrhythmogenic right ventricular dysplasia autopsy|Autopsy]] |
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| The first clinical signs of ARVD are usually during [[adolescence]]. However, signs of ARVD have been demonstrated in infants.
| | ==Treatment== |
| | :[[Arrhythmogenic right ventricular dysplasia medical therapy|Medical Therapy]] | [[Arrhythmogenic right ventricular dysplasia surgery|Surgery]] | [[Arrhythmogenic right ventricular dysplasia primary prevention|Primary Prevention]] | [[Arrhythmogenic right ventricular dysplasia secondary prevention|Secondary Prevention]] | [[Arrhythmogenic right ventricular dysplasia future or investigational therapies|Future or Investigational Therapies]] |
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| == Pathogenesis ==
| | [[fr:Maladie de Naxos]] |
| | | [[pl:Arytmogenna kardiomiopatia prawej komory]] |
| The pathogenesis of ARVD is largely unknown. [[Apoptosis]] (programmed cell death) appears to play a large role. It is unclear why only the right ventricle is involved. The disease process starts in the subepicardial region and works its way towards the endocardial surface, leading to transmural involvement (possibly accounting for the aneurysmal dilatation of the RV). Residual myocardium is confined to the subendocardial region and the trabeculae of the RV. These trabeculae may become hypertrophied.
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| Aneurysmal dilatation is seen in 50% of cases at autopsy. It usually occurs in the diaphragmatic, apical, and infundibular regions (known as the triangle of dysplasia). The left ventricle is involved in 50-67% of individuals. If the left ventricle is involved, it is usually late in the course of disease, and confers a poor prognosis.
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| There are two pathological patterns seen in ARVD, Fatty infiltration and fibro-fatty infiltration.
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| === Fatty infiltration ===
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| The first, fatty infiltration, is confined to the right ventricle. This involves a partial or near-complete substitution of myocardium with fatty tissue ''without'' wall thinning. It involves predominantly the apical and infundibular regions of the RV. The left ventricle and ventricular septum are usually spared. No inflammatory infiltrates are seen in fatty infiltration. There is evidence of [[myocyte]] (myocardial cell) degeneration and death seen in 50% of cases of fatty infiltration.
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| === Fibro-fatty infiltration ===
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| The second, fibro-fatty infiltration, involves replacement of myocytes with fibrofatty tissue. A patchy myocarditis is involved in up to 2/3 of cases, with inflammatory infiltrates (mostly [[T cell]]s) seen on microscopy. Myocardial atrophy is due to injury and [[apoptosis]]. This leads to thinning of the RV free wall (to < 3 mm thickness) Myocytes are replaced with fibrofatty tissue. The regions preferentially involved include the RV inflow tract, the RV outflow tract, and the RV apex. However, the LV free wall may be involved in some cases. Involvement of the ventricular septum is rare. The areas involved are prone to aneurysm formation.
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| == Ventricular arrhythmias ==
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| {| border="1" cellpadding="2" cellspacing="0" width="300px" align="left"
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| |-----
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| | bgcolor="#ffffff" align="center" |
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| [[Image:RVOT_Tachycardia.png|center|550px|Monomorphic ventricular tachycardia]]
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| <small>Right ventricular outflow tract tachycardia</small>
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| | align="center" style="border-bottom:3px solid gray;" | <font size="-1">''Monomorphic ventricular tachycardia originating from the right ventricular outflow tract.''</font>
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| |}
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| [[Ventricular arrhythmias]] due to ARVD typically arise from the diseased right ventricle. The type of arrhythmia ranges from frequent [[premature ventricular contraction | premature ventricular complexes]] (PVCs) to [[ventricular tachycardia]] (VT) to [[ventricular fibrillation]] (VF).
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| While the initiating factor of the ventricular arrhythmias is unclear, it may be due to triggered activity or reentry.
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| Ventricular arrhythmias are usually exercise-related, suggesting that they are sensitive to catecholamines. The ventricular beats typically have a right axis deviation. Multiple morphologies of ventricular tachycardia may be present in the same individual, suggesting multiple [[arrhythmogenic]] foci or pathways.
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| Right ventricular outflow tract (RVOT) tachycardia is the most common VT seen in individuals with ARVD. In this case, the EKG shows a [[left bundle branch block]] (LBBB) morphology with an inferior axis.
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| == Diagnosis ==
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| === Clinical testing ===
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| In order to make the diagnosis of ARVD, a number of clinical tests are employed, including the [[electrocardiogram]] (EKG), [[echocardiography]], right ventricular angiography, and cardiac MRI.
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| Researchers at Beth Israel Deaconess Medical Center in Boston have demonstrated an immunohistochemical test that reliably diagnoses ARVD. Doctors were able to correctly diagnose 10 of 11 subjects with definite ARVD and correctly ruled it out in another 10 of 11 subjects, according to results published in the ''New England Journal of Medicine''<ref name="pmid19279339">{{cite journal |author=Asimaki A, Tandri H, Huang H, ''et al'' |title=A new diagnostic test for arrhythmogenic right ventricular cardiomyopathy |journal=N. Engl. J. Med. |volume=360 |issue=11 |pages=1075–84 |year=2009 |month=March |pmid=19279339 |doi=10.1056/NEJMoa0808138 |url=}}</ref>. The authors conclude more research is needed but the results hold promise for identifying the condition when it can still be treated. "This is a disease in many cases where the first and only symptom is sudden death. And then it's discovered in autopsy," senior author Dr. Jeffrey Saffitz told Wiki''Doc''. "The next step will be testing whether a skin test could detect (ARVC)," Saffitz says, which would eliminate the need for the heart biopsy Saffitz' team used in conducting their ARVC test.
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| ==== Electrocardiogram ====
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| 90% of individuals with ARVD have some EKG abnormality. The most common EKG abnormality seen in ARVD is T wave inversion in leads V<sub>1</sub> to V<sub>3</sub>. However, this is a non-specific finding, and may be considered a normal variant in [[right bundle branch block]] (RBBB), women, and children under 12 years old.
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| [[RBBB]] itself is seen frequently in individuals with ARVD. This may be due to delayed activation of the [[right ventricle]], rather than any intrinsic abnormality in the right bundle branch.
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| [[Image:ARVD-Epsilon_wave.png|center|300px|EKG lead demonstrating the epsilon wave]]
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| <small>The epsilon wave</small>
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| | align="center" colspan="2" style="border-bottom:3px solid gray;" | <font size="-1">''The epsilon wave (red triangle), seen in ARVD.''</font>
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| |}
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| The '''epsilon wave''' is found in about 50% of those with ARVD. This is described as a terminal notch in the [[QRS complex]]. It is due to slowed intraventricular conduction. The epsilon wave may be seen on a surface EKG; however, it is more commonly seen on signal averaged EKGs.
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| Ventricular [[Cardiac ectopy|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. The origin of the ectopic beats is usually from one of the three regions of fatty degeneration (the "triangle of dysplasia"): the RV outflow tract, the RV inflow tract, and the RV apex.
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| [[Image:Epsilon2.jpg|350px|left|thumb|EKG of a patient with ARVD. The Epsilon wave is marked with an arrow.]]
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| ===Summary of EKG Characteristics===
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| *Incomplete or complete [[RBBB]]
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| *Inverted T waves in the anterior precordial leads
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| *Localized prolongation of the QRS complex in leads V1 and V2
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| *Epsilon waves visible as sharp discrete deflections at the terminal portion of the QRS complex in the anterior precordial leads
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| *Use QRS width in Lead I which is always <120ms
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| *Lead III R>S
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| *S wave upstroke in V1 - V3 >55ms was found in 95 percent of ARVD
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| ==== Signal averaged ECG ====
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| Signal averaged ECG (SAECG) is used to detect late potentials and epsilon waves in individuals with ARVD.
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| ==== Echocardiography ====
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| Echocardiography may reveal an enlarged, hypokinetic right ventricle with a paper-thin RV free wall. The dilatation of the RV will cause dilatation of the tricuspid valve annulus, with subsequent [[tricuspid regurgitation]].Paradoxical septal motion may also be present.
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| * Right Ventricular Dysplasia 1
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| <googlevideo>-8417307416816520415&hl=en</googlevideo>
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| * Right Ventricular Dysplasia 2
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| <googlevideo>-8312017427182612822&hl=en</googlevideo>
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| ==== Cardiac MRI ====
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| Fatty infiltration of the RV free wall can be visible on cardiac MRI. Fat has increased intensity in T1-weighted images. However, it may be difficult to differentiate intramyocardial fat and the epicardial fat that is commonly seen adjacent to the normal heart. Also, the sub-tricuspid region may be difficult to distinguish from the atrioventricular sulcus, which is rich in fat.
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| Cardiac MRI can visualize the extreme thinning and akinesis of the RV free wall. However, the normal RV free wall may be about 3 mm thick, making the test less sensitive.
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| *The typical criteria that can be demonstrated with MR imaging are
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| **Fatty infiltration of the right ventricular myocardium with high signal intensity on T1-weighted images(major criterion)
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| **Fibrofatty replacement, which leads to diffuse thinning of the right ventricular myocardium (major criterion)
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| **Aneurysms of the right ventricle and right ventricular outflow tract (major criterion)
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| **Dilatation of the right ventricle and right ventricular outflow tract (when severe, major criterion; when mild, minor criterion)
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| **Regional contraction abnormalities (minor criterion)
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| **Global systolic dysfunction (major criterion) and global diastolic dysfunction (minor criterion).
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| [[Image:Cardiac magnetic resonance Arrhythmogenic right ventricular dysplasia.gif|left|thumb|400px|Cardiac MRI: Arrhythmogenic right ventricular dysplasia. A short axis view of the heart. In this case, the scan demonstrates features of Arrhythmogenic right ventricular dysplasia with fatty infiltration of the left and right ventricles.]] | |
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| [[Image:Arvdf.jpg|left|thumb|400px|In vitro MRI (c) and corresponding cross section of the heart (d) show RV dilatation with anterior and posterior aneurysms.]] | |
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| ==== Right ventricular angiography ====
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| Right ventricular angiography is considered the [[gold standard (test)|gold standard]] for the diagnosis of ARVD. Findings consistent with ARVD are an akinetic or dyskinetic bulging localized to the infundibular, apical, and subtricuspid regions of the RV. The specificity is 90%; however, the test is observer dependent.
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| ==== Right ventricular biopsy ====
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| Transvenous biopsy of the right ventricle can be highly specific for ARVD, but it has low sensitivity. False positives include other conditions with fatty infiltration of the ventricle, such as chronic alcohol abuse and Duchenne/Becker muscular dystrophy.
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| False negatives are common, however, because the disease progresses typically from the epicardium to the endocardium (with the biopsy sample coming from the endocardium), and the segmental nature of the disease. Also, due to the paper-thin right ventricular free wall that is common in this disease process, most biopsy samples are taken from the ventricular septum, which is commonly ''not'' involved in the disease process.
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| A biopsy sample that is consistent with ARVD would have > 3% fat, >40% fibrous tissue, and <45% myocytes.
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| ====Histopathological Findings====
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| [[Image:Arvd_histo.jpg|thumb|left|300px|Micrograph of an ARVC heart. Typical histologic features of ARVC/D. Ongoing myocyte death (a) with early fibrosis and adipocytes infiltration (b).]]
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| [[Image:Arvde.jpg|left|thumb|350px|A 17 year old asymptomatic male athlete who died suddenly during a soccer game. Note the biventricular involvement at long axis in vitro MRI (a), with transmural fibro-fatty replacement in the RV free wall (b) and focal subepicardial in the LV free wall (c).]]
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| [[Image:Arvd SEM.jpg|left|thumb|350px|Transmission electron microscopy of the intercellular junction between two adjacent myocytes in ARVC/D. Note the presence of abnormal desmosomes, either long (arrows) or short-repeated structures (insert)]]
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| ==== Autopsy ====
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| A post mortem histological demonstration of full thickness substitution of the RV myocardium by fatty or fibro-fatty tissue is consistent with ARVD.
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| == Diagnostic Criteria ==
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| There is no pathognomonic feature of ARVD. The diagnosis of ARVD is based on a combination of major and minor criteria. To make a diagnosis of ARVD requires either 2 major criteria ''or'' 1 major and 2 minor criteria ''or'' 4 minor criteria.
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| ===Major Criteria===
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| * Right ventricular dysfunction
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| ** Severe dilatation and reduction of RV [[ejection fraction]] with little or no LV impairment
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| ** Localized RV aneurysms
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| ** Severe segmental dilatation of the RV
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| * Tissue characterization
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| ** Fibrofatty replacement of myocardium on endomyocardial biopsy
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| * Conduction abnormalities
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| ** Epsilon waves in V<sub>1</sub> - V<sub>3</sub>.
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| ** Localized prolongation (>110 ms) of QRS in V<sub>1</sub> - V<sub>3</sub>
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| * Family history
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| ** Familial disease confirmed on autopsy or surgery
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| ===Minor Criteria===
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| * Right ventricular dysfunction
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| ** Mild global RV dilatation and/or reduced ejection fraction with normal LV.
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| ** Mild segmental dilatation of the RV
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| ** Regional RV hypokinesis
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| * Tissue characterization
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| * Conduction abnormalities
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| ** Inverted T waves in V<sub>2</sub> and V<sub>3</sub> in an individual over 12 years old, in the absence of a [[right bundle branch block]] (RBBB)
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| ** Late potentials on signal averaged EKG.
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| ** Ventricular tachycardia with a [[left bundle branch block]] (LBBB) morphology
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| ** Frequent PVCs (> 1000 PVCs / 24 hours)
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| * Family history
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| ** Family history of sudden cardiac death before age 35
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| ** Family history of ARVD
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| == Natural History ==
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| There is a long asymptomatic lead-time in individuals with ARVD. While this is a genetically transmitted disease, individuals in their teens may not have any characteristics of ARVD on screening tests.
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| Many individuals have symptoms associated with ventricular tachycardia, such as palpitations, light-headedness, or syncope. Others may have symptoms and signs related to right ventricular failure, such as lower extremity edema, liver congestion with elevated hepatic enzymes. Unfortunately, sudden death may be the first manifestation of disease.
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| ARVD is a progressive disease. Over time, the right ventricle becomes more involved, leading to right ventricular failure. The right ventricle will fail before there is left ventricular dysfunction. However, by the time the individual has signs of overt right ventricular failure, there will be histological involvement of the left ventricle. Eventually, the left ventricle will also become involved, leading to bi-ventricular failure. Signs and symptoms of left ventricular failure may become evident, including congestive heart failure, atrial fibrillation, and an increased incidence of thromboembolic events.
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| == Management ==
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| The goal of management of ARVD is to decrease the incidence of sudden cardiac death. This raises a clinical dilemma: How to prophylactically treat the asymptomatic patient who was diagnosed during family screening.
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| A certain subgroup of individuals with ARVD are considered at high risk for sudden cardiac death. Characteristics associated with high risk of sudden cardiac death include:
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| * Young age
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| * Competitive sports activity
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| * Malignant familial history
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| * Extensive RV disease with decreased right ventricular ejection fraction.
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| * Left ventricular involvement
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| * Syncope
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| * Episode of ventricular arrhythmia
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| Management options include pharmacological, surgical, catheter ablation, and placement of an [[implantable cardioverter-defibrillator]].
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| Prior to the decision of the treatment option, [[programmed electrical stimulation]] in the [[cardiac electrophysiology | electrophysiology]] laboratory may be performed for additional prognostic information. Goals of programmed stimulation include:
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| *Assessment of the disease's arrhythmogenic potential
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| *Evaluate the hemodynamic consequences of sustained VT
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| *Determine whether the VT can be interrupted via antitachycardia pacing.
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| Regardless of the management option chosen, the individual is typically suggested to undergo lifestyle modification, including avoidance of strenuous exercise, cardiac stimulants (ie: caffeine, nicotine, pseudoephedrine) and alcohol. If the individual wishes to begin an exercise regimen, an exercise stress test may have added benefit.
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| === Pharmacologic management ===
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| Pharmacologic management of ARVD involves arrhythmia suppression and prevention of thrombus formation.
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| [[Sotalol]], a [[beta blocker]] and a class III [[antiarrhythmic agent]], is the most effective antiarrhythmic agent in ARVD. Other antiarrhythmic agents used include [[amiodarone]] and conventional beta blockers (ie: metoprolol). If antiarrhythmic agents are used, their efficacy should be guided by series ambulatory holter monitoring, to show a reduction in arrhythmic events.
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| While [[angiotensin converting enzyme inhibitor]]s (ACE Inhibitors) are well known for slowing progression in other cardiomyopathies, they have not been proven to be helpful in ARVD.
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| Individuals will decreased RV ejection fraction with dyskinetic portions of the right ventricle may benefit from long term anticoagulation with [[warfarin]] to prevent thrombus formation and subsequent [[pulmonary embolism]].
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| === Catheter ablation ===
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| Catheter ablation may be used to treat intractable ventricular tachycardia. It has a 60-90% success rate.<ref name="Fontaine-2000">{{cite journal | author=Fontaine G, Tonet J, Gallais Y, Lascault G, Hidden-Lucet F, Aouate P, Halimi F, Poulain F, Johnson N, Charfeddine H, Frank R. | title=Ventricular tachycardia catheter ablation in arrhythmogenic right ventricular dysplasia: a 16-year experience. | journal=Curr Cardiol Rep | year=2000 | volume=2 | issue=6 | pages=498-506 | id=PMID 11203287}}</ref> Unfortunately, due to the progressive nature of the disease, recurrence is common (60% recurrence rate), with the creation of new arrhythmogenic foci. Indications for catheter ablation include drug-refractory VT and frequent recurrence of VT after [[implantable cardioverter-defibrillator | ICD]] placement, causing frequent discharges of the ICD.
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| === Implantable cardioverter-defibrillator ===
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| An [[implantable cardioverter-defibrillator|ICD]] is the most effective prevention against sudden cardiac death. Due to the prohibitive cost of ICDs, they are not routinely placed in all individuals with ARVD.
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| Indications for ICD placement in the setting of ARVD include:
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| *Cardiac arrest due to VT or VF
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| *Symptomatic VT that is not inducible during [[programmed stimulation]]
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| *Failed programmed stimulation-guided drug therapy
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| *Severe RV involvement with poor tolerance of VT
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| *Sudden death of immediate family member
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| Since ICDs are typically placed via a transvenous approach into the right ventricle, there are complications associated with ICD placement and follow-up.
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| Due to the extreme thinning of the RV free wall, it is possible to perforation the RV during implantation, potentially causing [[cardiac tamponade | pericardial tamponade]]. Because of this, every attempt is made at placing the defibrillator lead on the ventricular septum.
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| After a successful implantation, the progressive nature of the disease may lead to fibro-fatty replacement of the myocardium at the site of lead placement. This may lead to undersensing of the individual's electrical activity (potentially causing inability to sense VT or VF), and inability to pace the ventricle.
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| === Cardiac transplant surgery ===
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| Cardiac transplant surgery is rarely performed in ARVD. It may be indicated if the arrhythmias associated with the disease are uncontrollable or if there is severe bi-ventricular heart failure that is not manageable with pharmacological therapy.
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| === Family screening ===
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| All first degree family members of the affected individual should be screened for [[ARVD]]. This is used to establish the pattern of inheritance. Screening should begin during the teenage years unless otherwise indicated. Screening tests include:
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| *Echocardiogram
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| *EKG
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| *Signal averaged EKG
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| *Holter monitoring
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| *Cardiac MRI
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| *Exercise stress test
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| ==References==
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| {{reflist|2}}
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| ==Related literature==
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| {{refbegin|2}}
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| #Fontaine G, Gallais Y, Fornes P, Hebert JL, Frank R. Arrhythmogenic right ventricular dysplasia/cardiomyopathy. Anesthesiology. 2001 Jul;95(1):250-4. ([http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=11465564 Medline abstract])
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| #Corrado D, Basso C, Thiene G. Arrhythmogenic right ventricular cardiomyopathy: diagnosis, prognosis, and treatment. Heart. 2000 May;83(5):588-95. ([http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=10768917 Medline abstract])
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| #McRae AT 3rd, Chung MK, Asher CR. Arrhythmogenic right ventricular cardiomyopathy: a cause of sudden death in young people. Cleve Clin J Med. 2001 May;68(5):459-67. ([http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=11352326 Medline abstract])
| |
| {{refend}}
| |
| | |
| ==Additional Resources==
| |
| {{refbegin|2}}
| |
| # Ahmad F, Li D, Karibe A, et al. Localization of a gene responsible for arrhythmogenic right ventricular dysplasia to chromosome 3p23. Circulation. Dec 22-29 1998;98(25):2791-5.
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| # Basso C, Thiene G, Corrado D, et al. Arrhythmogenic right ventricular cardiomyopathy. Dysplasia, dystrophy, or myocarditis?. Circulation. Sep 1 1996;94(5):983-91.
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| # Blake LM, Scheinman MM, Higgins CB. MR features of arrhythmogenic right ventricular dysplasia. AJR Am J Roentgenol. Apr 1994;162(4):809-12.
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| # Bomma C, Dalal D, Tandri H, Prakasa K, Nasir K, Roguin A. Evolving role of multidetector computed tomography in evaluation of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Am J Cardiol. Jul 1 2007;100(1):99-105.
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| # Burke AP, Farb A, Tashko G, Virmani R. Arrhythmogenic right ventricular cardiomyopathy and fatty replacement of the right ventricular myocardium: are they different diseases?. Circulation. Apr 28 1998;97(16):1571-80.
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| # Carlson MD, White RD, Trohman RG, et al. Right ventricular outflow tract ventricular tachycardia: detection of previously unrecognized anatomic abnormalities using cine magnetic resonance imaging. J Am Coll Cardiol. Sep 1994;24(3):720-7.
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| # Celbis O, Aydin NE, Mizrak B, Ozdemir B. Arrhythmogenic right ventricular dysplasia cases in forensic autopsies. Am J Forensic Med Pathol. Sep 2007;28(3):235-7.
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| # Corrado D, Basso C, Thiene G, et al. Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study. J Am Coll Cardiol. Nov 15 1997;30(6):1512-20.
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| # Daliento L, Rizzoli G, Thiene G, et al. Diagnostic accuracy of right ventriculography in arrhythmogenic right ventricular cardiomyopathy. Am J Cardiol. Sep 15 1990;66(7):741-5.
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| # El Masry HZ, Yadav AV. Arrhythmogenic right ventricular dysplasia/cardiomyopathy. Expert Rev Cardiovasc Ther. Feb 2008;6(2):249-60.
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| # Fontaine G, Fontaliran F, Hebert JL, et al. Arrhythmogenic right ventricular dysplasia. Annu Rev Med. 1999;50:17-35.
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| # Fontaine G, Gallais Y, Fornes P, Hébert JL, Frank R. Arrhythmogenic right ventricular dysplasia/cardiomyopathy. Anesthesiology. Jul 2001;95(1):250-4.
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| # Fontaine G, Tonet J, Gallais Y, et al. Ventricular tachycardia catheter ablation in arrhythmogenic right ventricular dysplasia: a 16-year experience. Curr Cardiol Rep. Nov 2000;2(6):498-506.
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| # Kantarci M, Bayraktutan U, Sevimli S, Bayram E, Durur I. Multidetector computed tomography findings of arrhythmogenic right ventricular dysplasia: a case report. Heart Surg Forum. 2007;10(6):E431-3.
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| # Kinsara AJ, Zaman L, Gorgels A. Arrhythmogenic right ventricular dysplasia. Am J Emerg Med. Jan 2001;19(1):67-70.
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| # Li D, Ahmad F, Gardner MJ, et al. The locus of a novel gene responsible for arrhythmogenic right- ventricular dysplasia characterized by early onset and high penetrance maps to chromosome 10p12-p14. Am J Hum Genet. Jan 2000;66(1):148-56.
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| # Midiri M, Finazzo M, Brancato M, et al. Arrhythmogenic right ventricular dysplasia: MR features. Eur Radiol. 1997;7(3):307-12.
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| # Midiri M, Finazzo M. MR imaging of arrhythmogenic right ventricular dysplasia. Int J Cardiovasc Imaging. Aug 2001;17(4):297-304.
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| # Moric-Janiszewska E, Markiewicz-Loskot G. Review on the genetics of arrhythmogenic right ventricular dysplasia. Europace. May 2007;9(5):259-66.
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| # Peters S. Left ventricular impairment in arrhythmogenic right ventricular dysplasia: what we can learn from angiography. Cardiology. 1995;86(6):473-6.
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| # Rampazzo A, Nava A, Danieli GA, et al. The gene for arrhythmogenic right ventricular cardiomyopathy maps to chromosome 14q23-q24. Hum Mol Genet. Jun 1994;3(6):959-62.
| |
| # Rampazzo A, Nava A, Miorin M, et al. ARVD4, a new locus for arrhythmogenic right ventricular cardiomyopathy, maps to chromosome 2 long arm. Genomics. Oct 15 1997;45(2):259-63.
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| # Ricci C, Longo R, Pagnan L, et al. Magnetic resonance imaging in right ventricular dysplasia. Am J Cardiol. Dec 15 1992;70(20):1589-95.
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| # Segal HN. Parchment heart. Am Heart J. 1950;40:948.
| |
| # Severini GM, Krajinovic M, Pinamonti B, et al. A new locus for arrhythmogenic right ventricular dysplasia on the long arm of chromosome 14. Genomics. Jan 15 1996;31(2):193-200.
| |
| # Tandri H, Macedo R, Calkins H, Marcus F, Cannom D, Scheinman M. Role of magnetic resonance imaging in arrhythmogenic right ventricular dysplasia: insights from the North American arrhythmogenic right ventricular dysplasia (ARVD/C) study. Am Heart J. Jan 2008;155(1):147-53.
| |
| # Van der Wall EE, Kayser HW, Bootsma MM, et al. Arrhythmogenic right ventricular dysplasia: MRI findings. Herz. 25(4):356-64.
| |
| # White RD, Trohman RG, Flamm SD, et al. Right ventricular arrhythmia in the absence of arrhythmogenic dysplasia: MR imaging of myocardial abnormalities. Radiology. Jun 1998;207(3):743-51.
| |
| # Wichter T, Hindricks G, Lerch H, et al. Regional myocardial sympathetic dysinnervation in arrhythmogenic right ventricular cardiomyopathy. An analysis using 123I-meta-iodobenzylguanidine scintigraphy. Circulation. Feb 1994;89(2):667-83
| |
| # Willerson JT, Cohn JN. Cardiovascular Medicine. 2nd ed. New York, NY: Churchill Livingstone; 2000: 1665-76.
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| {{refend}}
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| == External links ==
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| *http://www.arvd.com
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| *http://www.arvd.org
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| *http://www.arvd-arvc-info.com
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| *http://ourworld.compuserve.com/homepages/drmarknorman/
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| *http://telethon.bio.unipd.it/ARVDnet/
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| *http://www.cardiomyopathy.org/html/which_card_arvc.htm
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