Abstract
A case of constrictive pericarditis with marantic endocarditis in a patient with the Noonan syndrome is reported. Congenital heart defects are often diagnosed in the Noonan syndrome and are undoubtedly the most problematic of its pathologies. Our patient had surgery for pulmonic stenosis at age 10 and 16 years. Over a period of 1–2 years prior to death at age 23 years, he developed elevated jugular venous pressure, hypoproteinaemia, pedal oedema and pleural effusions. The hypoproteinaemia and resulting signs were initially attributed to intestinal lymphangiectasia. The latter, unlike constrictive pericarditis, has been reported in the Noonan syndrome. Post-mortem examination revealed constrictive pericarditis with a marantic endocarditis. There was no evidence of intestinal lymphangiectasia.
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Caralis, D.G.,Char, F.,Graber, J.D.,Voig, G.C. Delineation of multiple cardiac anomalies associated with the Noonan syndrome in an adult and review of the literature. Johns Hopkins Med. J. 1974;.134:.346–55.
Nora, J. J., Nora, A. H,, Sinha, A. K., Spangler, R. D., Lub, H. A. The Ullrich-Noonan syndrome (Turner phenotype). Am. J. Dis. Child 1974; 127: 48–55.
Engle, M. A., Ehlers, K. H. Cardiovascular malformations in the syndrome of Turner phenotype with normal karyotype. BD:OAS 1972; 8(5): 104–109.
Bolton, M. R., Pugh, D. M., Mattioli, L. J., Dunn, M. I., Schimke, R. N. The Noonan syndrome: A family study. Ann. Intern. Med. 1974; 80: 626–629.
Shabetai, R. Diseases of the pericardium. In Wyngarden, J. B., Smith, L. H. eds. Cecil textbook of medicine. Philadelphia. 1988: 362–367.
Mendez, H. M. M., Opitz, J. M. Noonan syndrome: A review. Am. J. Med. Genetics 1985; 21: 493–506.
Vallet, H. L., Holtzapple, P. G., Eberlein, W. R., Yakovac, W. C, Moshang, T., Bongiovanni, A. M. Noonan syndrome with intestinal lymphangiectasia. J. Pediatr. 1972; 80: 269–274.
Durrack, D. T. Marantic endocarditis. In Wyngarden, J.B., L. H. Smith eds. Cecil textbook of medicine. Philadelphia. 1988: 1586–1596.
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O’Sullivan, T., Hally, M., Cronin, C.C. et al. Constrictive pericarditis simulating intestinal lymphangiectasia in a patient with the noonan syndrome. I.J.M.S. 162, 180–181 (1993). https://doi.org/10.1007/BF02945180
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DOI: https://doi.org/10.1007/BF02945180