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Mitral valve replacement and Hunter syndrome: case report

Theophani Antoniou1, G Kirvassilis, L Tsourelis

  • 1Department of Anesthesiology, Onassis Cardiac Surgery Center, Athens, Greece. ktheodoraki@hotmail.com

The Heart Surgery Forum
|February 24, 2009
PubMed

Insights

This case study details a rare instance of mitral valve stenosis caused by Hunter syndrome (mucopolysaccharoidosis type II). Successful mitral valve replacement was achieved despite anesthetic challenges, with the patient remaining stable post-surgery.

Area of Science:

  • Cardiology
  • Genetics
  • Anesthesiology

Background:

  • Hunter syndrome (mucopolysaccharoidosis type II) is a rare genetic disorder.
  • Mucopolysaccharoidosis type II can lead to cardiac complications, including mitral valve stenosis.
  • Anatomical abnormalities in MPS patients pose significant anesthetic and surgical challenges.

Observation:

  • A 33-year-old male patient with Hunter syndrome presented with mitral valve stenosis.
  • The patient exhibited excessive oral secretions and atrial fibrillation, complicating management.
  • Preoperative assessment and meticulous airway management planning were crucial.

Findings:

  • The patient successfully underwent mitral valve replacement surgery.
  • The hospital course was uncomplicated following the procedure.
  • At 6-month follow-up, the patient remained in stable condition.

Implications:

  • This case highlights the importance of comprehensive preoperative evaluation for patients with rare genetic disorders undergoing cardiac surgery.
  • Effective airway management strategies are critical for mitigating anesthetic risks in MPS patients.
  • Successful surgical outcomes are achievable with careful planning and multidisciplinary care.

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