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

  • Alisa A. Arunamata (a1), Charles T. Nguyen (a1), Scott R. Ceresnak (a1), Anne M. Dubin (a1), Inger L. Olson (a1), Daniel J. Murphy (a1) and Elif S. Selamet Tierney (a1)...

Abstract

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 (427±16 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.

Copyright

Corresponding author

Author for correspondence: A. A. Arunamata, MD, Department of Pediatrics, Division of Pediatric Cardiology, Stanford University School of Medicine, 750 Welch Road, Suite #325, Palo Alto, CA 94304, USA. Tel: +1 650 721 5397; Fax: +1 650 725 8343; E-mail: alisa.arunamata@stanford.edu

Footnotes

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Cite this article: Arunamata AA, Nguyen CT, Ceresnak SR, Dubin AM, Olson IL, Murphy DJ, Selamet Tierney ES. (2018) Utility of serial 12-lead electrocardiograms in children with Marfan syndrome. Cardiology in the Young 28: 1009–1013. doi: 10.1017/S1047951118000707

Footnotes

References

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