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Multiple left-sided cardiac lesions in one of Noonan's original patients

Published online by Cambridge University Press:  19 August 2008

Jeffrey S. Danetz ,
Mary T. Donofrio  and
Richard P. Embrey
Jeffrey S. Danetz*
Affiliation:
Division of Pediatric Cardiac SurgeryRichmond, USA
Mary T. Donofrio
Affiliation:
Division of Pediatric Cardiac SurgeryRichmond, USA
Richard P. Embrey
Affiliation:
Division of Pediatric Cardiology, Medical College of Virginia, Richmond, USA
*
Jeffrey S. Danetz, M.D., Department of Surgery, Medical College of Virginia, PO. Box 981393, Richmond, Virginia 23298-1393. USA. Tel. (804) 346-0163; Fax. (804) 828-2760
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Abstract

We describe a complex case of obstruction of the left ventricular outflow tract in one of Dr. Noonan's original patients. Intraoperative findings revealed pathology at the valvar, subvalvar and supravalvar positions. Patients with Noonan syndrome are traditionally described as having right-sided cardiac pathology. Review of the literature revealed left-sided lesions to occur in a substantial number of these patients. We therefore suggest the routine employment of cardiac ultrasonography in all patients with Noonan syndrome with attention directed toward left-sided pathology, as well as the frequent pulmonary valvar pathology.

Keywords

Noonan syndromeleft-sided lesionsaortic stenosis
Type
Brief Reports
Information
Cardiology in the Young , Volume 9 , Issue 6 , November 1999 , pp. 610 - 612
Copyright
Copyright © Cambridge University Press 1999

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References

1.Noonan, JA. Noonan syndrome: An update and review for the primary pediatrician. Clin Pediat 1994;33(9):548–555.CrossRefGoogle ScholarPubMed
2.Siggers, DC, Polani, PE. Congenital heart disease in male and female subjects with somatic features of Turner's syndrome and normal sex chromosomes. BrHeart J 1972;34:41–46.Google ScholarPubMed
3.Rainer-Pope, CR, Cunnignham, RD, Nadas, AS, Crigler, JF. Cardiovascular malformations in Turner's syndrome. Pediatrics 1964;33:919.CrossRefGoogle Scholar
4.Sharland, M, Burch, M, McKenna, WM, Paton, MA. A clinical study of Noonan syndrome. Arch Dis Child 1992;67:178–183.CrossRefGoogle ScholarPubMed
5.Diekmann, L, Pfeiffer, RA, Hilenberg, F, Bender, F, Reploh, HD. Familial cardiomyopathy with congenital webbed neck. Munch Med Wochenschr 1967;109:2638–2645.Google ScholarPubMed
6.Hirsh, HD, Gelband, H, Garcia, O, Gottlieb, S, Tamer, DM. Rapidly progressive obstructive cardiomyopathy in infants with Noonan's syndrome. Circulation 1975;52:1161–1165.CrossRefGoogle Scholar
7.Marino, B, Gagliardi, MG, Digilio, MC, Polletta, B, Grazioli, S, Agartino, D, Giannotti, A, Dallapiccola, B. Noonan syndrome: structural abnormalities of the mitral valve causing subaortic obstruction. Eur J Pediatr 1995;154:949–952.CrossRefGoogle ScholarPubMed
8.Pearl, WCardiovascular anomalies in Noonan's syndrome. Chest 1977;71(5):677–679.CrossRefGoogle ScholarPubMed
9.Chery, M, Philippe, C, Worms, AM, Gilgenkrantz, S. The Noonan syndrome: The Nancy experience revisited. Genetic Coun 1993;4(2):113–118.Google ScholarPubMed
10.Lam, J, Corno, A, Oorthuys, HW, Marcelletti, C. Unusual combination of congenital heart lesions in a child with Noonan's syndrome. Ped Cardiol 1982;3:23–26.CrossRefGoogle Scholar