Syncope as the first manifestation of acute rheumatic fever: report of an adolescent case

Key words: Acute rheumatic fever, Complete AV block, Syncope, Temporary pacemaker

Background - Acute rheumatic fever (ARF) is an autoimmune disorder following group A streptococcal pharyngitis that can lead to arthritis, carditis with mitral and/or aortic valve insufficiency, chorea and skin manifestations (erythema marginatum and/or subcutaneous nodules). It is known that the majority of ARF patients have abnormalities of the conduction system, mostly prolongation of the PR interval. In a paucity of subjects a complete atrioventricular (AV) block can occur and these patients may present with dramatic symptoms such as syncope.
Case presentation - The article reports the case of a 14-year-old girl who was referred to the Emergency Room because she presented with 2 episodes of syncope while at school. The electrocardiogram revealed a complete AV block. The first echocardiogram on 1st day was normal but subsequent echocardiograms on 2nd and 3rd days showed pancarditis with mitral insufficiency, aortic insufficiency, pericardial effusion and reduction of the ejection fraction. A temporary pacemaker was implanted and medical therapy for ARF was started.
Conclusion - ARF should be considered in the diagnostic work-up of patients with complete AV block, especially those in paediatric age. An echocardiogram should be performed in all cases of acquired AV block and should be repeated. In rare cases ARF patients present with complete AV block. Although it is usually transient, it may lead to dramatic symptoms such as syncope. In these cases a temporary pacemaker implantation must be considered.