Hostname: page-component-586b7cd67f-dlnhk Total loading time: 0 Render date: 2024-11-23T12:34:51.846Z Has data issue: false hasContentIssue false

Sudden death in a patient with Noonan syndrome

Published online by Cambridge University Press:  22 December 2010

Alper Aydin*
Affiliation:
Faculty of Medicine, Department of Cardiology, Maltepe University, Istanbul, Turkey
Mustafa S. Yilmazer
Affiliation:
Faculty of Medicine, Department of Cardiology, Maltepe University, Istanbul, Turkey
Tayfun Gurol
Affiliation:
Faculty of Medicine, Department of Cardiology, Maltepe University, Istanbul, Turkey
*
Correspondence to: Dr A. Aydin, Faculty of Medicine, Department of Cardiology, Maltepe University, Feyzullah Cad No. 39, 34843 Maltepe, Istanbul, Turkey. Tel: +90 542 585 5519; Fax: +90 216 383 0271; E-mail: [email protected]

Abstract

We report here the case of a 27-year-old woman with Noonan syndrome presenting with ventricular fibrillation. After successful defibrillation, echocardiography revealed hypertrophic cardiomyopathy associated with left ventricular outflow tract obstruction. Normal echocardiographic cardiac structure and function were reported 11 years ago. This case emphasises the importance of regular follow-up in patients with congenital disorders in which cardiac manifestations might develop in early adulthood or later.

Type
Brief Reports
Copyright
Copyright © Cambridge University Press 2010

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.)

References

1. Burch, M, Sharland, M, Shinebourne, E, Smith, G, Patton, M, McKenna, W. Cardiologic abnormalities in Noonan syndrome: phenotypic diagnosis and echocardiographic assessment of 118 patients. J Am Coll Cardiol 1993; 22: 11891192.CrossRefGoogle ScholarPubMed
2. Noonan, JA, Ehmke, DA. Associated non-cardiac malformations in children with congenital heart disease. J Pediatr 1963; 63: 468470.Google Scholar
3. Sreeram, N, Kitchiner, D, Smith, A. Spectrum of valvular abnormalities in Noonan's syndrome: a pathologic study. Cardiol Young 1994; 4: 6266.CrossRefGoogle Scholar
4. Musewe, NN, Robertson, MA, Benson, LN, et al. The dysplastic pulmonary valve: echocardiographic features and results of balloon dilatation. Br Heart J 1987; 57: 364370.CrossRefGoogle ScholarPubMed
5. Maron, BJ, McKenna, WJ, Danielson, GK, et al. ACC/ESC clinical expert consensus document on hypertrophic cardiomyopathy: a report of the American College of Cardiology Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines. J Am Coll Cardiol 2003; 42: 16871713.CrossRefGoogle Scholar
6. Olivotto, I, Gistri, R, Petrone, P, et al. Maximum left ventricular thickness and risk of sudden death in patients with hypertrophic cardiomyopathy. J Am Coll Cardiol 2003; 41: 315321.CrossRefGoogle ScholarPubMed