Abstract
Atrial flutter (AFL) in children and adolescents beyond the neonatal period in the absence of any underlying myocardial disease (“lone AFL”) is rare and data is limited. Our study aims to present clinical and electrophysiological data of presumed “lone AFL” in pediatric patients and discuss the role of endomyocardial biopsy (EMB) and further follow-up. Since July 2005, eight consecutive patients at a median age of 12.7 (range 10.4–16.7) years presenting with presumed “lone AFL” after negative non-invasive diagnostic work-up had electrophysiological study (EPS) and induction of cavotricuspid isthmus (CTI) conduction block by radiofrequency (RF) current application. In 6/8 patients EMB could be taken. Induction of CTI conduction block was achieved in all patients. Histopathological examination of EMB from the right ventricular septum exhibited myocarditis or cardiomyopathy in 4/6 patients, respectively. During follow-up, 4/8 patients had recurrent arrhythmia (AFL n = 2, wide QRS complex tachycardia n = 1, monomorphic premature ventricular contractions n = 1) after the ablation procedure. 3/4 patients with recurrent arrhythmia had pathological EMB results. The remaining patient with recurrent arrhythmia had a negative EMB but was diagnosed with Brugada syndrome during further follow-up. Taking together results of EMB and further clinical course, only 3/8 patients finally turned out to have true “lone AFL”. Our study demonstrates that true “lone AFL” in children and adolescents is rare. EMB and clinical course revealed an underlying cardiac pathology in the majority of the individuals studied. EMB was very helpful in order to timely establish the diagnosis of myocarditis or cardiomyopathy.
Highlights
In pediatric patients, atrial flutter (AFL) without any obvious cardiac abnormatility (“lone AFL”) is rare and most frequently occurs in the neonatal period [1]
As actual data on AFL in pediatric patients beyond the neonatal period without any obvious underlying myocardial disease is limited, our study aims to present the clinical features and management of presumed “lone AFL” in this group of patients focussing on the value of endomyocardial biopsy (EMB) and the further clinical course
All patients had experienced symptoms during AFL (Table 1). 6/8 patients (75%) had suffered from sustained (≥ 30 s) AFL episodes
Summary
Atrial flutter (AFL) without any obvious cardiac abnormatility (“lone AFL”) is rare and most frequently occurs in the neonatal period [1]. Beyond this age, AFL primarily occurs in the presence of congenital heart disease (CHD) [1,2,3], in patients with accessory atrioventricular (AV) pathways [4], in myocarditis [5, 6] and in patients with various types of cardiomyopathy [7]. Adult patients either have a history of ischaemic heart disease or prior cardiac surgery [10]. A familial risk has been described in patients diagnosed before 50 years of age [11], and a genetic mutation predisposing for AFL has been identified [12]
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