Complete heart block in a patient with systemic sclerosis - Springer Link

1 downloads 0 Views 55KB Size Report
ventricular (AV) heart block associated with bifascicular intraventricular block [left posterior bundle branch block. (LBBB) and right bundle branch block (RBBB)].
Clin Rheumatol (2006) 25: 551–552 DOI 10.1007/s10067-005-0068-2

BRIEF REPORT

I. Moyssakis . D. P. Papadopoulos . A. G. Tzioufas . V. Votteas

Complete heart block in a patient with systemic sclerosis

Received: 10 May 2005 / Revised: 19 May 2005 / Accepted: 24 May 2005 / Published online: 1 November 2005 # Clinical Rheumatology 2005

Abstract We describe a patient with diffuse systemic sclerosis and presyncopal episodes where the electrocardiogram revealed complete atrioventricular heart block associated with left posterior and right heart bundle block. The patient underwent implantation of a permanent pacemaker. Keywords Complete heart block . Systemic sclerosis

Introduction We describe a female patient with diffuse systemic sclerosis (SSc) and electrocardiographic findings of complete atrioventricular (AV) heart block associated with bifascicular intraventricular block [left posterior bundle branch block (LBBB) and right bundle branch block (RBBB)].

Case report The patient, a 68-year-old woman, was admitted to the cardiology clinic of our hospital due to generalized weakness, presyncopal episodes, and deterioration of dyspnea class IV according to New York Heart Association (NYHA). Her previous medical history reveals the presence of diffuse SSc which was diagnosed 18 years ago when she presented Raynaud's phenomenon, arthralgias, subcutaneous edema, sclerodactyly, fever, and malaise. At the time of final admission, 7.5 mg prednisone per day and 20 mg

nifedipine twice a day were administered, whereas previous medications included methotrexate and cyclophosphamide. Until the appearance of her recent symptoms, the patient was in dyspnea on exertion class II according to NYHA, whereas in previous electrocardiogram (ECG), RBBB was shown. On admission, the physical examination revealed jugular vein congestion, ascots, and peripheral edema. The liver was palpable 3 cm below the right costal margin. Her heart rate ranged from 35 to 48 beats per minute, and the blood pressure was 105/60 mmHg. On auscultation, there was a grade II/VI systolic murmur at the lower left parasternal border. Blood tests were normal besides a normochromic, normocytic anemia with 34% hematocrit and 53 mm/h erythrocyte sedimentation rate. Antinuclear antibodies were present in a titer 1:640, and anti Scl-70 antibodies were also present. Pulmonary function tests also disclosed extensive pulmonary fibrosis. The ECG revealed complete AV block associated with bifascicular intraventricular block [left posterior heart block (LPHB) and RBBB]. The echocardiogram showed a borderline impaired left ventricular (LV) systolic function with an LV ejection fraction of about 50% and mild right ventricular hypertrophy and dilatation. There was a moderate degree tricuspid regurgitation with a maximal pressure gradient of 58 mmHg. Treatment was initiated with diuretics, angiotensin-converting enzyme inhibitors, and supplemental oxygen on which the patient improved both clinically and hemodynamically. During hospitalization, the patient underwent a permanent dual chamber pacemaker implantation.

Discussion I. Moyssakis (*) . D. P. Papadopoulos . V. Votteas Department of Cardiology, Laiko Hospital of Athens, 17 Agiou Thomas St. Goudi, 15727, Athens, Greece e-mail: [email protected] A. G. Tzioufas Pathophysiology Department, School of Medicine, University of Athens, Athens, Greece

The heart has been generally recognized as a target organ in SSc, mainly affecting pericardium, myocardium, and conduction system [1]. Evaluation of the severity of cardiac lesions in SSc is the key to the prognosis and have also pointed out that electrocardiographic abnormalities have the greatest influence on the survival in such patients [2, 3]. SSc is connected with various atrial or ventricular arrhythmias and also conduction system abnormalities con-

552

cerning sinus node, AV node, and His bundle [4–6]. Specifically, the conduction system lesions include firstdegree heart block, RBBB, LBBB, and high-grade heart block [6]. However, complete heart block has been reported in a few cases [7–9]. In our case, the complete AV block was associated with bifascicular intraventricular block (LPHB and RBBB). The most probable explanation of the AV and intraventricular conduction abnormalities might be connected with the progressive fibrotic replacement of the myocardium. Especially the LBBB and RBBB possibly are related to the progressive fibroelastic replacement and more recent degenerative changes in the left and right bundle branch and, finally, progressive destruction to both of them [10]. The electrocardiographic changes of left bundle branch appeared later compared to right branch because of its wide distribution of this bundle, despite the similar fibrotic and degenerative changes in both branches [10]. It should be noted also that in case of complete heart block in SSc, the postmortem histological examination revealed that the sinoatrial node and AV node as well as its initial portion were without changes, whereas changes in the distal portion of the AV node and bundle of His are of lesser magnitude than those in the bundle branches [4]. Conversely, another case report is referred about a patient with anterior myocardial infarction and an ECG evolving from the infarctual pattern associated with RBBB to RBBB associated with LPHB. His bundle electrogram documented a markedly prolonged His ventricular interval, confirming an advanced impairment of distal conduction system. This case supports the suggestion that intraventricular conduction disorders in SSc are not always related to diffuse myocardial involvement [11]. Others have described a relative sparing of the conduction system and attributed these faults to necrosis and disturbance of impulse conduction through the underlying myocardium [4]. Cardiac conducting tissue antibodies were detected in patients with SSc, and conduction abnormalities were

present in 59% of patients. However, whether the above antibodies should be mainly regarded as an expression of the immunological derangement underlying these pathological conditions or whether they are secondary to myocardial tissue damage must still be clarified [12].

References 1. Deswal A, Follansbee WP (1996) Cardiac involvement in scleroderma. Rheum Dis Clin North Am 22:841–861 2. Mizuno R, Fujimoto S, Nakano H, Nakajima T, Kimura A, Nakagawa Y, Dohl K (1997) Atrial conduction abnormalities in patients with systemic progressive sclerosis. Eur Heart J 18:1995–2001 3. Bennet R, Bluestone R, Holt PJL, Bywaters EGL (1971) Survival in scleroderma. Ann Rheum Dis 30:581–588 4. Ridolfi RL, Bulkley BH, Hutchins GM (1976) The cardiac conduction system in progressive systemic sclerosis. Clinical and pathologic features of 35 patients. Am J Med 61:361–366 5. Roberts NK, Cabeen WR, Moss J, Clements PJ, Furst DE (1981) The prevalence of conduction defects and cardiac arrhythmias in progressive systemic sclerosis. Ann Intern Med 4:38–40 6. Follansbee W, Curtiss E, Rahko P, Medsger T, Lavine S, Owens G, Steen V (1985) The electrocardiogram in systemic sclerosis (Scleroderma). Am J Med 9:183–192 7. Raynaud R, Benatre A, Brochier M, Morand P, Raynaud P (1967) Scleroderma heart and complete auriculo-ventricular block. Arch Mal Coeur Vaiss 60:1865–1870 8. Anuar M, Singham KT (1983) Systemic scleroderma with complete heart block. Med J Malaysia 38:65–67 9. Mory B, Luquel L, Catanese V, Offenstadt G (1988) Complete atrioventricular dissociation in a patient with systemic scleroderma. Presse Med 17:590–592 10. Lev M, Landowne M, Matchar J, Wagner J (1966) Systemic scleroderma with complete heart block. Am Heart J 72:13–24 11. Loperfido F, Fiorilli R, Santarelli P, Bellocci F, Zecchi P (1982) Severe involvement of the conduction system in a patient with sclerodermal heart disease. An electrophysiological study. Acta Cardiol 37:31–38 12. Volta U, Villecco AS, Bianchi FB, Varotti C, Cassani F, Lenzi M, Veronesi S, Tosti A, Pisi E (1981) Antibodies to cardiac conducting tissue in progressive systemic sclerosis. Clin Exp Immunol 43:478–485