Utility of serial 12-lead electrocardiograms in children with Marfan syndrome

作者:Arunamata Alisa A*; Nguyen Charles T; Ceresnak Scott R; Dubin Anne M; Olson Inger L; Murphy Daniel J; Tierney Elif S Selamet
来源:Cardiology in the Young, 2018, 28(8): 1009-1013.
DOI:10.1017/S1047951118000707

摘要

Objectives: The goal of this study was to assess the utility of serial electrocardiograms in routine follow-up of paediatric Marfan patients. Methods: Children <= 18 years who met the revised Ghent criteria for Marfan syndrome and received a 12-lead electrocardiogram and echocardiogram within a 3-month period were included. Controls were matched by age, body surface area, gender, race, and ethnicity, and consisted of patients assessed in clinic with a normal cardiac evaluation. Demographic, clinical, echocardiographic, and electrocardiographic data were collected. Results: A total of 45 Marfan patients (10.8 [2.4-17.1] years) and 37 controls (12.8 [1.3-17.1] years) were included. Left atrial enlargement and left ventricular hypertrophy were more frequently present on 12-lead electrocardiogram of Marfan patients compared with controls (12 (27%) versus 0 (0%), p<0.001; and 8 (18%) versus 0 (0%), p=0.008, respectively); however, only two patients with left atrial enlargement on 12-lead electrocardiogram were confirmed to have left atrial enlargement by echocardiogram, and one patient had mild left ventricular hypertrophy by echocardiogram, not appreciated on 12-lead electrocardiogram. QTc interval was longer in Marfan patients compared with controls (42716 versus 417 +/- 22 ms, p=0.03), with four Marfan patients demonstrating borderline prolonged QTc intervals for gender. Conclusions: While Marfan patients exhibited a higher frequency of left atrial enlargement and left ventricular hypertrophy on 12-lead electrocardiograms compared with controls, these findings were not supported by echocardiography. Serial 12-lead electrocardiograms in routine follow-up of asymptomatic paediatric Marfan patients may be more appropriate for a subgroup of Marfan patients only, specifically those with prolonged QTc interval at their baseline visit.