Syncope Due to Paroxysmal Atrioventricular Block in a Patient With Systemic Sclerosis: A Case Report

Abstract

A 79-year-old woman with systemic sclerosis was admitted to our hospital because of syncope. On admission, electrocardiogram showed progression of intraventricular conduction defect. Chest radiograph showed marked cardiomegaly. Echocardiogram revealed deterioration of left ventricular systolic function. We suspected progressive myocardial disease with Stokes-Adams attack. When we were preparing a temporary pacemaker, paroxysmal atrioventricular block with asystole for 15 seconds and convulsion occurred. Electrophysiological study showed His-ventricular block and sinus node dysfunction. A permanent pacemaker was implanted. In systemic sclerosis, progression of ventricular conduction defect may warrant prompt electrophysiological study and prophylactic pacemaker implantation.

Envolvimento das musculaturas esquelética e cardíaca na esclerose sistêmica

“…4,7 Thus, considering that association and the poor prognosis of heart involvement in SSc, cardiac monitoring in patients with SSc and skeletal myopathy should be performed regularly, 7 including the assessment of prophylactic pacemaker indication in high-risk patients. 9 The association between muscle involvement and LVD was also observed in our study. Of the patients with LVD, 75% had an alteration in muscle strength, atrophy and/or CPK elevation.…”

Complete heart block and permanent pacemaker implantation in a patient with systemic sclerosis

“…Cardiac manifestations are common in SSc, with a prevalence of 15-35% and includes pericarditis, myocardial disease, conduction abnormalities, and arrhythmia (2). However, complete atrioventricular (AV) block is rarely reported (3)(4)(5). Several pathophysiological mechanisms were explained regarding dysrhythmias in SSc patients such as direct myocardial or conduction system involvement by fibrotic process.…”

“…The progressive fibrotic replacement and degenerative changes in the AV node, His bundle, or in the bundle branch can finally cause their fibrotic destruction. However, complete heart block fortunately occurs very rarely and has been reported in only a few cases of SSc patients so far (3)(4)(5).…”