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

Published online by Cambridge University Press:  04 July 2018

Alisa A. Arunamata*
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, Stanford University School of Medicine, Palo Alto, CA, USA
Charles T. Nguyen
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, Stanford University School of Medicine, Palo Alto, CA, USA
Scott R. Ceresnak
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, Stanford University School of Medicine, Palo Alto, CA, USA
Anne M. Dubin
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, Stanford University School of Medicine, Palo Alto, CA, USA
Inger L. Olson
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, Stanford University School of Medicine, Palo Alto, CA, USA
Daniel J. Murphy
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, Stanford University School of Medicine, Palo Alto, CA, USA
Elif S. Selamet Tierney
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, Stanford University School of Medicine, Palo Alto, CA, USA
*
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

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.

Type
Original Article
Copyright
© Cambridge University Press 2018 

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Footnotes

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 Young28: 1009–1013. doi: 10.1017/S1047951118000707

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