Abstract
Congenital heart disease occurs in 35–50% of patients diagnosed with Noonan syndrome. We present an infant with an unusual combination of congenital heart defects not previously reported, including partial atrioventricular septal defect, polyvalvular dysplasia, and progressive hypertrophic cardiomyopathy. We discuss the possible interaction between these lesions that may have led to the patient's rapid demise.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Burch M, Mann JM, Sharland M, et al (1992) Myocardial disarray in Noonan syndrome.Br Heart J 68:586–588
Eckner FAO, Brown BW, Davidson DL, Glagow S (1969) Dimensions of normal human hearts after standard fixation by controlled pressure coronary perfusion.Arch Pathol 88:497–507
Ehlers KH, Engle MA, Levin AR, Deely WJ (1972) Eccentric ventricular hypertrophy in familial and sporadic instances of 46XX, XY Turner phenotype.Circulation 45:639–652
Freedom RM, Bini M, Rowe RD (1978) Endocardial cushion defect and significant hypoplasia of the left ventricle: a distinct clinical and pathological entity.Eur J Cardiol 7:263–281
Hirsch HD, Gelband H, Garcia O, Gottlieb S, Tamer DM (1975) Rapidly progressive obstructive cardiomyopathy in infants with Noonan's syndrome.Circulation 52:1161–1165
Noonan JA (1968) Hypertelorism with Turner phenotype: a new syndrome with associated congenital heart disease.Am J Dis Child 116:373–380
Noonan JA (1978) Association of congenital heart disease with syndromes or other defects.Pediatr Clin North Am 25:797–816
Nora JJ, Lortscher RH, Spangler RD (1975) Echocardiographic studies of left ventricular disease in Ullrich-Noonan syndrome.Am J Dis Child 129:1417–1420
Nora JJ, Nora AH, Sinha AK, Spangler RD, Lubs HA (1974) The Ullrich-Noonan syndrome (Turner phenotype).Am J Dis Child 27:48–55
Siggers DC, Polani PE (1972) Congenital heart disease in male and female subjects with somatic features of Turner's syndrome and normal sex chromosomes (Ullrich's and related syndromes).Br Heart J 34:41–46
Van der Hauwaert LG, Fryns JP, Dumoulin M, Logghe N (1978) Cardiovascular malformations in Turner's and Noonan's syndrome.Br Heart J 40:500–509
Walther HJ, Siassi B, King J, Wu PYK (1986) Echocardiographic measurements in normal preterm and term neonates.Acta Paediatr Scand 75:563–568
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Feit, L.R., Hansen, K., Oyer, C.E. et al. Unusual combination of congenital heart defects in an infant with Noonan syndrome. Pediatr Cardiol 16, 95–99 (1995). https://doi.org/10.1007/BF00796828
Issue Date:
DOI: https://doi.org/10.1007/BF00796828