Abstract
The cardiovascular lesions commonly seen in Marfan syndrome can frequently be the primary cause of premature death. Cardiac lesions involving both the mitral valve and the aortic root are commonly observed among patients diagnosed during early infancy, as so-called infantile Marfan syndrome. Since the lesions tend to progress rapidly with the end results of high morbidity and mortality, the majority of patients require surgical intervention at a young age. However, patients who undergo surgical intervention for both lesions during the first decade of life have been rarely reported in literature. In this report, we present a case of a 9-year-old boy who underwent aortic root replacement with a composite graft at 3.5 years after a prior prosthetic valve replacement of the mitral valve. Although the immediate result was satisfactory, the long-term result remains to be seen.
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Murdoch JL, Walker BA, Halpern BL, Kuzma JW, McKusick VA. Life expectancy and causes of death in the Marfan syndrome. N Engl J Med 1972; 286: 804–8.
Geva T, Hegesh J, Frand M. The clinical course and echocardiographic features of Marfan’s syndrome in childhood. Am J Dis Child 1987; 141: 1179–82.
Miura M, Shimazaki Y, Watanabe T, Iijima Y, Kuraoka S, Inui K, et al. Bentall operation for a child with Marfan syndrome: a case report. J Cardiac Surg 1997; 12: 116–9.
Gillinov AM, Zehr KJ, Redmond JM, Gott VL, Deitz HC, Reitz BA, et al. Cardiac operations in children with Marfan’s syndrome: indications and results. Ann Thorac Surg 1997; 64: 1140–4.
Geva T, Sanders SP, Diogenes MS, Rockenmacher S, Van PR. Two-dimensional and Doppler echocardiographic and pathologic characteristics of the infantile Marfan syndrome. Am J Cardiol 1990; 65: 1230–7.
Gillinov AM, Hulyalkar A, Cameron DE, Cho PW, Greene PS, Reitz BA, et al. Mitral valve operation in patients with the Marfan syndrome. J Thorac Cardiovasc Surg 1994; 107: 724–31.
Gott VL, Pyeritz RE, Cameron DE, Greene PS, McKusick VA. Composite graft repair of Marfan aneurysm of the ascending aorta: results in 100 patients. Ann Thorac Surg 1991; 52: 38–44.
David TE. Current practice in Marfan’s aortic root surgery: reconstruction with aortic valve preservation or replacement? What to do with the mitral valve? J Cardiac Surg 1997; 12: 147–50.
Chiba Y, Muraoka R, Kado M, Ihaya A, Kimura T, Saito S. The successful repair of annuloaortic ectasia using Cabrol’s operation in a 5-year-old child with Marfan’s syndrome of the forme fruste type. Surg Today 1995; 25: 268–71.
Gott VL, Cameron DE, Pyeritz RE, Gillinov AM, Greene PS, Stone CD, et al. Composite graft repair of Marfan aneurysm of the ascending aorta: results in 150 patients. J Cardiac Surg 1994; 9: 482–9.
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Kamikubo, Y., Murashita, T., Yasuda, K. et al. Mitral valve replacement and subsequent composite graft replacement of the aortic root for infantile Marfan syndrome. Jpn J Thorac Caridovasc Surg 48, 366–369 (2000). https://doi.org/10.1007/BF03218157
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DOI: https://doi.org/10.1007/BF03218157