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Rapid progression of mitral valve disease in a child with Noonan syndrome

Published online by Cambridge University Press:  28 September 2020

Pezad N. Doctor Open the ORCID record for Pezad N. Doctor [Opens in a new window] ,
David K. Lawrence  and
Amrit Misra Open the ORCID record for Amrit Misra [Opens in a new window]
Pezad N. Doctor*
Affiliation:
Department of Pediatrics, Children’s Hospital of Michigan, Detroit, MI, USA
David K. Lawrence
Affiliation:
Division of Pediatric Cardiology, Department of Pediatrics, Children’s Hospital of Michigan, Detroit, MI, USA
Amrit Misra
Affiliation:
Division of Pediatric Cardiology, Department of Pediatrics, Children’s Hospital of Michigan, Detroit, MI, USA
*
Author for correspondence: Pezad Doctor, MD, Department of Pediatrics, Children’s Hospital of Michigan, 3901 Beaubien Boulevard, Detroit, MI48201, USA. Tel: +1 203 868 6048. E-mail: [email protected]
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Abstract

Noonan syndrome is the second most common genetic syndrome associated with congenital heart disease after Trisomy 21. The two most common cardiac lesions associated with Noonan syndrome are pulmonary stenosis and hypertrophic cardiomyopathy. Although the incidence of mitral valve disease in Noonan syndrome ranges between 2 and 6%, rapid progression of mitral valve dysplasia causing severe mitral valve regurgitation and left atrial dilatation is seldom seen. Most cases of mitral valve disease have been diagnosed either on routine echocardiographic surveillance or when presented with heart failure symptoms. We describe an 18-month-old boy with Noonan syndrome presenting in atrial flutter due to a massively enlarged left atrium caused by severe mitral valve regurgitation which developed and progressed in less than 17 months.

Keywords

Noonan syndromemitral valveatrial flutter
Type
Brief Report
Information
Cardiology in the Young , Volume 30 , Issue 12 , December 2020 , pp. 1964 - 1966
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Copyright
© The Author(s), 2020. Published by Cambridge University Press

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