Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C)

Marcus, Frank I.; Towbin, Jeffrey A.; Zaręba, Wojciech; Moss, Arthur J.; Calkins, Hugh; Brown, Mary W.; Gear, Kathleen

doi:10.1161/01.cir.0000071380.43086.29

Cited by 112 publications

(67 citation statements)

References 16 publications

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“…It is likely that the results from the North American ARVD Registry and the international ARVD registry may provide important information in the future. 40,41 …”

Section: Resultsmentioning

confidence: 99%

Arrhythmogenic Right Ventricular Dysplasia

et al. 2005

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Background-Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited cardiomyopathy characterized by right ventricular dysfunction and ventricular arrhythmias. The purpose of our study was to describe the presentation, clinical features, survival, and natural history of ARVD in a large cohort of patients from the United States. Methods and Results-The patient population included 100 ARVD patients (51 male; median age at presentation, 26[interquartile range {IQR}, 18 to 38; range, 2 to 70] years). A familial pattern was observed in 32 patients. The most common presenting symptoms were palpitations, syncope, and sudden cardiac death (SCD) in 27%, 26%, and 23% of patients, respectively. Among those who were diagnosed while living (nϭ69), the median time between first presentation and diagnosis was 1 (range, 0 to 37) year. During a median follow-up of 6 (IQR, 2 to 13; range, 0 to 37) years, implantable cardioverter/defibrillators (ICD) were implanted in 47 patients, 29 of whom received an appropriate ICD discharge, including 3 patients who received the ICD for primary prevention. At follow-up, 66 patients were alive, of whom 44 had an ICD in place, 5 developed signs of heart failure, 2 had a heart transplant, and 18 were on drug therapy. Thirty-four patients died either at presentation (nϭ23: 21 SCD, 2 noncardiac deaths) or during follow-up (nϭ11: 10 SCD, 1 of biventricular heart failure), of whom only 3 were diagnosed while living and 1 had an ICD implanted. On Kaplan-Meier analysis, the median survival in the entire population was 60 years. Conclusions-ARVD patients present between the second and fifth decades of life either with symptoms of palpitations and syncope associated with ventricular tachycardia or with SCD. Diagnosis is often delayed. Once diagnosed and treated with an ICD, mortality is low. There is a wide variation in presentation and course of ARVD patients, which can likely be explained by the genetic heterogeneity of the disease. (Circulation. 2005;112:3823-3832.)

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“…It is likely that the results from the North American ARVD Registry and the international ARVD registry may provide important information in the future. 40,41 …”

Section: Resultsmentioning

confidence: 99%

Arrhythmogenic Right Ventricular Dysplasia

et al. 2005

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“…The excellent image resolution obtained with current techniques allows for the accurate quantification of chamber volumes, LV mass, and ventricular function (166 -168). This is of particular value to patients with suspected arrhythmogenic RV cardiomyopathy (ARVC), in whom MRI provides excellent assessment of RV size, function, and regional wall motion and, importantly, may allow the detection of fatty infiltration within the RV myocardium (169,170). RV angiography may also be useful.…”

Section: Cardiac Magnetic Resonance Imagingmentioning

confidence: 99%

ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death

Zipes¹,

Camm²,

Borggrefe³

et al. 2006

Circulation

1,200

671

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“…[6][7][8][9][10][11] Our first patient had his genetic screening for mutations of desmosomal proteins, also his father had the same diagnosis, and the third patient had a family history of ARVD/C, and his elder brother managed by medical therapy.…”

Section: Discussionmentioning

confidence: 98%

“…The second phase symptomatic arrhythmias of RV origin are common and more prominent; the third phase the isolated RV failure and concomitant dysplastic process becomes diffuse with greater dilatation and dysfunction of RV and finally the fourth phase is left ventricular involvement and biventricular failure. [7][8][9][10][11] All three patients had admitted our hospital with either third phase and fourth phase. The presence of biventricular heart failure and intracardiac thrombus formation were identified as high risk group for our patients.…”

Section: Discussionmentioning

confidence: 99%

Arrhytmogenic Right Ventricular Dysplasia/ Cardiomyopathy (ARVD/C) and Heart Transplantation: Case Series

Demirozu¹,

Küçükaksu²

2015

Turkiye Klinikleri J Cardiovasc Sci

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36rrhytmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a heart muscle disease of unknown etiology, characterized by peculiar right ventricular (RV) involvement, has a prevalence of at least 1 in 1000 and ventricular arrhythmias, sudden death may be observed. 1Arrhytmogenic Right Ventricular Dysplasia/ Cardiomyopathy (ARVD/C) and Heart Transplantation: Case Series A AB BS ST TR RA AC CT T Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) usually originates from right ventricle, has a prevalence 1 in 1000 and leading cause of death in people aged less than 35 years of age, also desmosome mutations and family history can be seen in this disease. We reviewed the literature and described three patients with arrhythmogenic right ventricular dysplasia/ cardiomyopathy (ARVD/C). They had T wave inversion in chest leads V1 to V4 and had premature ventricular complexes of left bundle branch block and left axis deviation, or right bundle branch block (RBB) had implantable cardioverter defibrillator (ICD) implantation and fulfilled the task force criteria for diagnosis of ARVD/C. One patient had familial erythrocytosis and thrombus formation in the right atrium and the right ventricle. He was listed as status 1A and had 2 times phlebotomy during the hospitalization. The other patient had warfarin intoxication, and had hepatic congestion due to end-stage right heart failure. The third patient had a history of cerebrovascular event and had a family history of ARVD/C. All of our patients had the medical regimen for the management of ARVD/C. All of the patients were in NYHA Class III-IV while they admitted to our clinic and they had their final therapeutic option as an orthotopic heart transplantation and have a good quality of life.K Ke ey y W Wo or rd ds s: : Arrhythmogenic right ventricular dysplasia; heart transplantation Ö ÖZ ZE ET T Aritmojenik sağ ventriküler displazisi/kardiyomiyopati (ARVD/C), sağ ventrikülden orijin alır, 1000'de 1 görülme sıklığı vardır, 35 yaşından genç insanlarda en fazla ölüm sebeplerindendir. Bu hastalıkta desmozom mutasyonları ve aile hikayesi görülebilir. Biz detaylı olarak literatürü taradıktan sonra, aritmojenik sağ ventrikül displazisi/kardiyomiyopati (ARVD/C) olan 3 hastamızı sunuyoruz.V1'den V4'e kadar tüm göğüs derivasyonlarında ve sol dal bloğu ile prematür ventrikül atımlar ve sol aks deviasyonu ve sağ dal bloğu tespit edilmesi üzerine hastalara kardiyoverter implantable defibrilatör (ICD) implante edildi. Hastalarımızdan biri 1A statüsünde idi ve hastanede bulunduğu sürede 2 kez filebotomi yapıldı. Diğer hastanın warfarin intoksikasyonu ve sağ kalp yetmezliğine bağlı karaciğer konjesyonu mevcuttu. Üçüncü hastamızın da daha önce geçirilmiş serebrovasküler olayı ve ARVD/C aile hikayesi vardı. Tüm hastalarımız ARVD/C yönelik maksimum medikal tedavilerini almışlardı ve kliniğimize başvurduklarında New York Heart Association (NYHA) Klass III-IV idiler ve onlar için en iyi tedavi kalp nakli olmaları idi. Ortotopik kalp naklini olduktan sonra daha iyi bir yaşam ...

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Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C)

Cited by 112 publications

References 16 publications

Arrhythmogenic Right Ventricular Dysplasia

Arrhythmogenic Right Ventricular Dysplasia

ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death

Arrhytmogenic Right Ventricular Dysplasia/ Cardiomyopathy (ARVD/C) and Heart Transplantation: Case Series

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