Aortic Valve Replacement in a Patient with Morquio Syndrome


  • Francesco Nicolini
  • Domenico Corradi
  • Silvia Bosio
  • Tiziano Gherli



Cardiac involvement in Morquio syndrome, mucopolysaccharidosis IV, is characterized by aortic and mitral valve thickening and infiltration of the coronary arteries. There are few reports concerning surgical interventions in patients with mucopolysaccharidoses. We report a case of a patient affected by Morquio syndrome who underwent aortic valve replacement surgery for severe aortic valve stenosis, with an uneventful postoperative course. Cardiac surgery appears safe and feasible in these patients and improves the quality of life, even if the future prognosis related to the underlying disorder should be considered.


Brailsford JF. 1929. Chondro-osteo-dystrophy, roentgenographic and clinical features of a child with dislocation of vertebrae. Am J Surg 7:404-10.nDangel JH. 1998. Cardiovascular changes in children with mucopolysaccharide storage diseases and related disorders: clinical and echocardiographic findings in 64 patients. Eur J Pediatr 157:534-8.nHachida M, Nonoyama M, Bonkohara Y, Hanayama N, Koyanagi H. 1996. Combined aortic and mitral valve replacement in an adult with mucopolysaccharidosis (Maroteaux-Lamy syndrome). Heart Vessels 11:215-7.nIeland MA, Rowlands DB. 1981. Mucopolysaccharidosis type IV as a cause of mitral stenosis in an adult. Br Heart J 46:113-5.nMinakata K, Konishi Y, Matsumoto M, Miwa S. 1998. Surgical treatment for Scheie's syndrome (mucopolysaccharidosis type I-S): report of two cases. Jpn Circ J 62:700-3.nMorquio L. 1929. Sur une forme de dystrophie osseuse familiare. Arch Med Enfants 32:129-40.nShinhar SY, Zablocki H, Madgy DN. 2004. Airway management in mucopolysaccharide storage disorders. Arch Otolaryngol Head Neck Surg 130:233-7.n



How to Cite

Nicolini, F., Corradi, D., Bosio, S., & Gherli, T. (2008). Aortic Valve Replacement in a Patient with Morquio Syndrome. The Heart Surgery Forum, 11(2), E96-E98.