Thromboembolic complications in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy.

AIMS Incidence and clinical presentation of thromboembolic complications in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) were analysed. In reports on ARVD/C, thromboembolism is rarely mentioned. The possible risk factors are: right ventricle (RV) dilatation, aneurysms, and wall motion abnormalities. METHODS AND RESULTS A group of 126 patients (89 male, 37 female, aged 43.6+/-14.3) with ARVD/C was retrospectively analysed for the presence of thromboembolic complications. The mean follow-up period was 99+/-64 months. Thromboembolic complications, i.e. pulmonary embolism (n=2), RV outflow tract thrombosis with severe RV failure (n=1), and cerebrovascular accident associated with atrial fibrillation (n=2) were observed in 4% of the patients. Spontaneous echogenic contrast was observed in seven patients with severe damage to RV. In four of them supraventricular arrhythmias resulting in heart failure were reported. Annual incidence of thromboembolic complications was 0.5/100 patients. CONCLUSIONS (i) ARVD/C may be complicated by thrombosis. Annual incidence of such complications is significantly lower than reported for left ventricle failure. (ii) Anticoagulation should be used in ARVD/C patients with large, hypokinetic RV and slow blood flow. (iii) Patients with severe forms of ARVD/C, thrombus formation in the RV and/or spontaneous echocardiographic contrast are at higher risk of a poor outcome.

[1]  F. Marcus,et al.  Arrhythmogenic Right Ventricular Dysplasia Masquerading as Peripartum Cardiomyopathy with Atrial Flutter, Advanced Atrioventricular Block and Embolic Stroke , 2002, Cardiology.

[2]  E. Oechslin,et al.  Extensive Thrombus Formation in the Right Ventricle due to a Rare Combination of Arrhythmogenic Right Ventricular Cardiomyopathy and Heterozygous Prothrombin Gene Mutation G20210 A , 2000, Cardiology.

[3]  L. Goldberg Right ventricular thrombosis in arrhythmogenic right ventricular dysplasia. , 2000, Cardiovascular journal of South Africa : official journal for Southern Africa Cardiac Society [and] South African Society of Cardiac Practitioners.

[4]  G. Nicolosi,et al.  [Right ventricular thrombosis in arrhythmogenic cardiomyopathy. A case report]. , 2000, Italian heart journal. Supplement : official journal of the Italian Federation of Cardiology.

[5]  B. Eryonucu,et al.  A case of arrhythmogenic right ventricular cardiomyopathy in sinus rhythm associated with thrombus in the right atrium. , 2000, Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography.

[6]  Z. Kornacewicz-Jach,et al.  Atrial Epicardial Pacing with Long Stimulus to P Wave Interval in a Patient with Arrhythmogenic Right Ventricular Dysplasia Complicated by Right Atrial Thrombosis , 1999, Pacing and clinical electrophysiology : PACE.

[7]  G. Deák,et al.  [Arrhythmogenic right ventricular cardiomyopathy associated with multiple right atrial thrombi]. , 1997, Orvosi hetilap.

[8]  I. B. Kristensen,et al.  Arrhythmogenic right ventricular dysplasia as a cause of sudden death. , 1997, The American journal of forensic medicine and pathology.

[9]  M. Davies,et al.  Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study. , 1997, Journal of the American College of Cardiology.

[10]  A. Angelini,et al.  Arrhythmogenic right ventricular cardiomyopathy. Dysplasia, dystrophy, or myocarditis? , 1996, Circulation.

[11]  D. Baker,et al.  Management of Heart Failure: IV. Anticoagulation for Patients With Heart Failure Due to Left Ventricular Systolic Dysfunction , 1994 .

[12]  G. Thiene,et al.  Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Task Force of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology. , 1994, British heart journal.

[13]  J. A. Vázquez de Prada,et al.  Resolution of relapsing thrombus by heparin therapy in right ventricular dysplasia. , 1990, International journal of cardiology.

[14]  D. Wright,et al.  Management of Heart Failure , 1999, Journal of the Royal College of Physicians of London.