Successful C1 inhibitor short-term prophylaxis during redo mitral valve replacement in a patient with hereditary angioedema

J Cardiothorac Surg. 2010 Oct 18:5:86. doi: 10.1186/1749-8090-5-86.

Abstract

Hereditary angioedema is characterized by sudden episodes of nonpitting edema that cause discomfort and pain. Typically the extremities, genitalia, trunk, gastrointestinal tract, face, and larynx are affected by attacks of swelling. Laryngeal swelling carries significant risk for asphyxiation. The disease results from mutations in the C1 esterase inhibitor gene that cause C1 esterase inhibitor deficiency. Attacks of hereditary angioedema result from contact, complement, and fibrinolytic plasma cascade activation, where C1 esterase inhibitor irreversibly binds substrates. Patients with hereditary angioedema cannot replenish C1 esterase inhibitor levels on pace with its binding. When C1 esterase inhibitor is depleted in these patients, vasoactive plasma cascade products cause swelling attacks. Trauma is a known trigger for hereditary angioedema attacks, and patients have been denied surgical procedures because of this risk. However, uncomplicated surgeries have been reported. Appropriate prophylaxis can reduce peri-operative morbidity in these patients, despite proteolytic cascade and complement activation during surgical trauma. We report a case of successful short-term prophylaxis with C1 esterase inhibitor in a 51-year-old man with hereditary angioedema who underwent redo mitral valve reconstructive surgery.

Publication types

  • Case Reports

MeSH terms

  • Angioedemas, Hereditary / complications*
  • Cardiac Valve Annuloplasty
  • Complement C1 Inactivator Proteins / administration & dosage*
  • Complement C1 Inactivator Proteins / analysis
  • Complement C1 Inhibitor Protein
  • Edema / etiology
  • Edema / prevention & control*
  • Humans
  • Male
  • Middle Aged
  • Mitral Valve / surgery*
  • Mitral Valve Insufficiency / complications
  • Mitral Valve Insufficiency / surgery*
  • Postoperative Complications / prevention & control*
  • Reoperation

Substances

  • Complement C1 Inactivator Proteins
  • Complement C1 Inhibitor Protein
  • SERPING1 protein, human