Mitral Valve Replacement and Hunter Syndrome: Case Report
AbstractWe report a rare case of mitral valve stenosis secondary to Hunter syndrome, mucopolysaccharoidosis (MPS) type II in a 33-year-old man. Anatomical abnormalities in patients with MPS present anesthetic and surgical challenges during cardiac surgery. Management of this particular patient was complicated by excessive oral secretions and atrial fibrillation. With a detailed preoperative assessment and planning for airway management, this patient successfully underwent mitral valve replacement and had an uncomplicated hospital course. After 6 months of follow-up, the patient was still in stable condition.
Baines D, Keneally J. 1983. Anaesthetic implications of the mucopolysaccharidoses: a fifteen-year experience in a children's hospital. Anaesth Intens Care 11:198-202.nBhattacharya K, Gibson S, Pathi V. 2005. Mitral valve replacement for mitral stenosis secondary to Hunter syndrome. Ann Thorac Surg 80:1911-2.nBrown TC. 1984. The airway in mucopolysaccharidoses. Anaesth Intens Care 12:178.nFischer TA, Lehr H-A, Nixdorff U, Meyer J. 1999. Combined aortic and mitral stenosis in mucopolysaccharidosis type I-S (Ullrich-Sheie syndrome). Heart 81:97-9.nGross DM, Williams JC, Caprioli C, et al. 1988. Echocardiographic abnormalities in the mucopolysaccharide storage disease. Am J Cardiol 61;170-6.nHunter CA. 1917. A rare disease in two brothers. Proc R Soc Med 10:104-16.nJohnson GL, Vine DL, Cottrill CM, Noonan JA. 1981. Echocardiographic mitral valve deformity in the mucopolysaccharidoses. Pediatrics 67(3):401-6.nKempthorne PM, Brown TC. 1983. Anaesthesia and the mucopolysaccharidoses: a survey of techniques and problems. Anaesth Intensive Care 11:203-7.nKing DH, Jones RM, Barnett MB. 1984. Anesthetic considerations in the mucopolysaccharidoses. Anesthesia 39:126-31.nMayer J, Boldt J, Schollhrn T, et al. 2007. Semi-invasive monitoring of cardiac output by a new device using arterial pressure waveform analysis: a comparison with intermittent pulmonary artery thermodilution in patients undergoing cardiac surgery. Br J Anaesth 98(2):176-82.nMoores C, Rogers JG, McKenzie IM, Brown TC. 1996. Anaesthesia for children with mucopolysaccharidoses. Anaesth Intens Care 24:459-63.nSchwartz IV, Ribeiro MG, Mota JG, et al. 2007. A clinical study of 77 patients with mucopolysaccharidosis type II. Acta Paediatr Suppl. 96(455):63-70.nSemenza GL, Pyeritz RE. 1988. Respiratory complications of mucopolysaccharide storage disorders. Medicine 67:209-19.nWalker RW, Darowski M, Morris M, Wraith E. 1994. Anaesthesia and mucopolysaccharidoses. Anaesthesia 49:1078-84.nZimmermann B, Lally EV, Sharma SC, et al. 1988. Severe aortic stenosis in systemic lupus erythematosus and mucopolysaccharidosis type mucopolysaccharidosis type II (Hunter's syndrome). Clin Cardiol 11(10):723-5.n
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