Arrhythmogenic right ventricular dysplasia and rheumatoid arthritis/Aritmojenik sag ventrikul displazisi ve romatoid artrit.
A 60-year-old woman with rheumatoid arthritis (RA) presents to the emergency room with palpitation and near syncope. Her hemodynamic was unstable and electrocardiogram (ECG) showed a wide QRS tachycardia. A sinus rhythm was obtained after electrical cardioversion (DC). The patient's history revealed infrequent palpitation with presyncope symptoms. She was on methotrexate and cyclosporine. She had no family history of sudden death and similar disease. On physical examination, her blood pressure after DC was 130/80 mmHg, pulse rate was 80/min. Upper extremity examination reve aled radial deviation of both wrist with ulnar deviation of the finger ("Z" deformation) and swan-neck deformities of the fingers of both hands (Fig. 1). After DC, control ECG showed right bundle branch block with epsilon wave in V1 and ST segment depression in precordial leads (V2-V4) (Fig. 2). An echocardiography revealed normal left ventricular systolic function without wall motion abnormalities and sever dilatation of right ventricle (48 mm) (Fig. 3). Magnetic resonance imaging showed the muscular tissue in free wall of right ventricle was replaced in heterogeneous manner by fatty tissue with dilatation of both right ventricle and right atrium (Fig. 4).
An electrophysiological study demonstrated normal conduction system with induction sustains ventricular tachycardia that result in deterioration of the hemodynamics of the patient. Therefore, the patient received an implantable defibrillator (ICD) without complication and discharged with medical advices.
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Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is genetically transmitted disease affecting the muscle of the heart. The combination of ARVD with rheumatoid arthritis was the characteristic feature of our patient. To our knowledge, it is the first time in literature to report such presentation. Cardiac disease in RA can take many forms related to granulomatous proliferation or vasculitis. Involvement can be classified as follows: (1) pericarditis, (2) myocarditis, (3) endocardial (valve) involvement, (4) conduction defects, (5) coronary arteritis, and (6) granulomatous aortitis (1). Clinical recognition of cardiac disease occurs in approximately one third of patients with chronic RA, but is more common in autopsy series (2). Direct involvement of right ventricle with development right ventricular failure in patients with RA is not well known. The presence both of these diseases in this patient bring to our mind the role of the inflammation or the presence of shared genetic predisposition. The role of infection or inflammation in the etiology of ARVD was proposed and thought is the remnant of myocarditis. Fontaine et al. (1) in examining 27 patients, they found inflammatory infiltrations in eight of them. In other site of view, the major genetic risk factor for RA is class II histocompatible complex alel HLA-DR4. Meanwhile ARVD shows different type of family genetic transmission in which the plakophilin (PKP2) mutation has been found as a major cause of ARVC/D with prevalence of mutations among unrelated index cases as high as 43% (3, 4).
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It is unexplained whether unknown genetic mutation or the effect of inflammation played a main role in the etiology of our case. However, it is logical to keep in our mind the diagnosis of ARVD in-patient with rheumatoid arthritis when presented to us with malignant ventricular arrhythmias. In these conditions clinical evaluation with meticulous echocardiography examination, cardiac MRI and right ventriculography should be done and if it is possible to be supported by genetic study.
(1.) Fontaine G, Fontaliran F, Lascault G, Frank R, Tonet J, Chomette G, et al. Congenital and acquired right ventricular dysplasia. Arch Mal Coeur Vaiss 1990; 83: 915-20.
(2.) Harris: Kelley's Textbook of Rheumatology, 7th ed, 696-97.
(3.) Dalal D, Molin LH, Piccini J, Tichnell C, James C, Bomma C, et al. Clinical features of arrhythmogenic right ventricular dysplasia/cardiomyopathy associated with mutations in plakophilin-2. Circulation 2006; 113: 1641-9. [CrossRef]
(4.) van Tintelen JP Entius MM, Bhuiyan ZA, Jongbloed R, Wiesfeld AC, Wilde AA, et al. Plakophilin-2 mutations are the major determinant of familial arrhythmogenic right ventricular dysplasia/cardiomyopathy. Circulation 2006; 113: 1650-8. [CrossRef]
Diyar Koprulu, Halit Zengin , Zeydin Acar , Sabri Demircan 
Clinic of Cardiology, Ordu State Hospital, Ordu
 Department of Cardiology, Faculty of Medicine, Ondokuz Mayis University, Samsun
 Clinic of Cardiology, Ahi Evren State Hospital, Trabzon-Turkey
Address for Correspondence/Yazisma Adresi: Dr. Diyar Koprulu, Ordu Devlet Hastanesi, Kardiyoloji Klinigi, Ordu-Turkiye Phone: +90 452 234 95 44 E-mail: email@example.com
Available Online Date/Cevrimici Yayin Tarihi: 07.02.2012
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|Title Annotation:||Letters to Editor/Editore Mektuplar|
|Author:||Koprulu, Diyar; Zengin, Halit; Acar, Zeydin; Demircan, Sabri|
|Publication:||The Anatolian Journal of Cardiology (Anadolu Kardiyoloji Dergisi)|
|Date:||Mar 1, 2012|
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