Hostname: page-component-586b7cd67f-l7hp2 Total loading time: 0 Render date: 2024-11-28T00:00:46.088Z Has data issue: false hasContentIssue false
  • English
  • Français

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

Published online by Cambridge University Press:  04 July 2018

Alisa A. Arunamata Open the ORCID record for Alisa A. Arunamata [Opens in a new window] ,
Charles T. Nguyen ,
Scott R. Ceresnak ,
Anne M. Dubin ,
Inger L. Olson ,
Daniel J. Murphy  and
Elif S. Selamet Tierney
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: [email protected]
Get access Rights & Permissions [Opens in a new window]

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.

Keywords

Marfan syndromeelectrocardiogramoutpatient paediatric cardiologypaediatric echocardiogram
Type
Original Article
Information
Cardiology in the Young , Volume 28 , Issue 8 , August 2018 , pp. 1009 - 1013
Check for updates
Copyright
© Cambridge University Press 2018 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

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 Young 28: 1009–1013. doi: 10.1017/S1047951118000707

References

1. Dietz, HC. Marfan syndrome. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K, Amemiya A, (eds) GeneReviews. Seattle, WA, USA, 2016; 1993–2018.Google Scholar
2. Loeys, BL, Dietz, HC, Braverman, AC, et al. The revised Ghent nosology for the Marfan syndrome. J Med Genet 2010; 47: 476–485.Google Scholar
3. Yetman, AT, Bornemeier, RA, McCrindle, BW. Long-term outcome in patients with Marfan syndrome: is aortic dissection the only cause of sudden death? J Am Coll Cardiol 2003; 41: 329–332.Google Scholar
4. Savolainen, A, Kupari, M, Toivonen, L, Kaitila, I, Viitasalo, M. Abnormal ambulatory electrocardiographic findings in patients with the Marfan syndrome. J Intern Med 1997; 241: 221–226.CrossRefGoogle ScholarPubMed
5. Chen, S, Fagan, LF, Nouri, S, Donahoe, JL. Ventricular dysrhythmias in children with Marfan’s syndrome. Am J Dis Child 1985; 139: 273–276.Google ScholarPubMed
6. Phornphutkul, C, Rosenthal, A, Nadas, AS. Cardiac manifestations of Marfan syndrome in infancy and childhood. Circulation 1973; 47: 587–596.CrossRefGoogle ScholarPubMed
7. Wyatt, HL, Meerbaum, S, Heng, MK, Gueret, P, Corday, E. Cross-sectional echocardiography. III. Analysis of mathematic models for quantifying volume of symmetric and asymmetric left ventricles. Am Heart J 1980; 100: 821–828.CrossRefGoogle ScholarPubMed
8. Sluysmans, T, Colan, SD. Structural measurements and adjustment for growth. In: Lai WW, Cohen MS, Geva T, Mertens L, (eds) Echocardiography in Pediatric and Congenital Heart Disease. Wiley-Blackwell, West Sussex, 2009: 61–72.Google Scholar
9. Colan, SD. Normal echocardiographic values for cardiovascular structures. In: Lai WW, Cohen MS, Geva T, Mertens L, (eds) Echocardiography in Pediatric and Congenital Heart Disease. Wiley-Blackwell, West Sussex, 2009: 765–785.Google Scholar
10. Vieira-Filho, NG, Mancuso, FJ, Oliveira, WA, et al. Simplified single plane echocardiography is comparable to conventional biplane two-dimensional echocardiography in the evaluation of left atrial volume: a study validated by three-dimensional echocardiography in 143 individuals. Echocardiography 2014; 31: 265–272.CrossRefGoogle ScholarPubMed
11. Lang, RM, Bierig, M, Devereux, RB, et al. Recommendations for chamber quantification: a report from the American Society of Echocardiography’s Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiography, a branch of the European Society of Cardiology. J Am Soc Echocardiogr 2005; 18: 1440–1463.CrossRefGoogle Scholar
12. Bhatla, P, Nielsen, JC, Ko, HH, Doucette, J, Lytrivi, ID, Srivastava, S. Normal values of left atrial volume in pediatric age group using a validated allometric model. Circ Cardiovasc Imag 2012; 5: 791–796.Google Scholar
13. Davignon, A, Rautaharju, P, Boise, E, Soumis, F, Megelas, M, Choquette, A. Normal ECG standards for infants and children. Pediatr Cardiol 1979; 80: 123–134.Google Scholar
14. Hancock, EW, Deal, BJ, Mirvis, DM, et al. AHA/ACCF/HRS recommendations for the standardization and interpretation of the electrocardiogram: part V: electrocardiogram changes associated with cardiac chamber hypertrophy: a scientific statement from the American Heart Association Electrocardiography and Arrhythmias Committee, Council on Clinical Cardiology; the American College of Cardiology Foundation; and the Heart Rhythm Society. Endorsed by the International Society for Computerized Electrocardiology. J Am Coll Cardiol 2009; 53: 992–1002.Google Scholar
15. Towbin, JA, Bricker, JT, Garson, A. Electrocardiographic criteria for diagnosis of acute myocardial infarction in childhood. Am J Cardiol 1992; 69: 1545–1548.CrossRefGoogle ScholarPubMed
16. Rautaharju, PM, Surawicz, B, Gettes, LS, et al. AHA/ACCF/HRS recommendations for the standardization and interpretation of the electrocardiogram: part IV: the ST segment, T and U waves, and the QT interval: a scientific statement from the American Heart Association Electrocardiography and Arrhythmias Committee, Council on Clinical Cardiology; the American College of Cardiology Foundation; and the Heart Rhythm Society. Endorsed by the International Society for Computerized Electrocardiology. J Am Coll Cardiol 2009; 53: 982–991.Google Scholar
17. Schwartz, PJ, Moss, AJ, Vincent, GM, Crampton, RS. Diagnostic criteria for the long QT syndrome: an update. Circulation 1993; 88: 782–784.Google Scholar
18. Wagner, GS, Macfarlane, P, Wellens, H, et al. AHA/ACCF/HRS recommendations for the standardization and interpretation of the electrocardiogram: part VI: acute ischemia/infarction: a scientific statement from the American Heart Association Electrocardiography and Arrhythmias Committee, Council on Clinical Cardiology; the American College of Cardiology Foundation; and the Heart Rhythm Society. Endorsed by the International Society for Computerized Electrocardiology. J Am Coll Cardiol 2009; 53: 1003–1011.Google Scholar
19. Pyeritz, RE, McKusick, VA. The Marfan syndrome: diagnosis and management. N Engl J Med 1979; 300: 772–777.Google Scholar
20. Martins De Oliveira, J, Sambhi, MP, Zimmerman, HA. The electrocardiogram in pectus excavatum. Br Heart J 1958; 20: 495–501.Google Scholar