Journal of Chest Surgery

The Korean Society for Thoracic and Cardiovascular Surgery (대한심장혈관흉부외과학회)
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Dysfunction of the Prosthetic Aortic Valve in Idiopathic Hypereosinophilic Syndrome -A case report -

인공 대동맥판막 기능부전을 일으킨 특발성 과호산구 증후군 -1예 보고-

  • Yoo, Dong-Gon (Department of Thoracic and Cardiovascular Surgery, Gangneung Asan Hospital) ;
  • Sung, Kyu-Wan (Department of Anesthesiology and Pain Medicine, Gangneung Asan Hospital) ;
  • Jung, Sang-Sig (Department of Thoracic and Cardiovascular Surgery, Gangneung Asan Hospital) ;
  • Kang, Gil-Hyun (Department of Diagnostic Pathology, Gangneung Asan Hospital) ;
  • Kim, Chong-Wook (Department of Thoracic and Cardiovascular Surgery, Gangneung Asan Hospital) ;
  • Park, Chong-Bin (Department of Thoracic and Cardiovascular Surgery, Gangneung Asan Hospital)
  • 유동곤 (강릉아산병원 흉부외과) ;
  • 성규완 (강릉아산병원 마취통증의학과) ;
  • 정상식 (강릉아산병원 흉부외과) ;
  • 강길현 (강릉아산병원 진단병리과) ;
  • 김종욱 (강릉아산병원 흉부외과) ;
  • 박종빈 (강릉아산병원 흉부외과)
  • Published : 2007.04.05
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Abstract

Idiopathic hypereosinophilic syndrome is a rare systemic, leukoproliferative disorder characterized by eosinophilmediated tissue injury causing multiple organ failure, including the heart. Cardiac involvement occurs in more than 75% of patients with hypereosinophilic syndrome. Cardiac manifestations include subendocardial fibrosis, thrombus leading to peripheral emboli, restrictive cardiomyopathy, and valvular dysfunction. It is more common in men than in women (9 : 1), and trends to present between the ages of 20 and 50 years. Presentation in childhood is unusual. We report for the first time a case of a 58-year-old man with idiopathic hypereosinophilic syndrome manifested by prosthetic aortic valve dysfunction that was successfully treated by steroid and hydroxyurea therapy after surgical valvular replacement.

특발성 과호산구 증후군은 호산구 매개에 의한 조직 손상으로 인해 다발성 장기부전, 특히 심장을 침범하는 드문 전신성, 백혈구증식성 질환이다. 심장침범은 특발성 과호산구 증후군 환자의 75% 이상에서 일어난다. 심장증상은 심내막 하 섬유증, 제한성 심근병증, 판막부전, 그리고 말초동맥 혈전증을 유발하는 혈전이다. 이 질환은 남자에서 여자보다 9 : 1로 호발하고, $20{\sim}50$대에서 주로 발현하는 경향이 있으며 소아에서는 매우 드물다. 인공 대동맥판막부전을 나타낸 특발성 과호산구 증후군 환자(58세, 남자)를 인공판막 재치환 수술 후 부신피질호르몬제와 hydroxyurea 투여로 성공적으로 치료하였기에 보고하는 바이다.

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References

  1. Hardy WR, Anderson RE. The hypereosinophilic syndromes. Ann Intern Med 1968;68:1220-9 https://doi.org/10.7326/0003-4819-68-6-1220
  2. Chusid MJ, Dale DC, West DC, Wolff SM. The hypereosinophilic syndrome analysis of fourteen cases with review of the literliture. Medicine 1975;54:1-27 https://doi.org/10.1097/00005792-197501000-00001
  3. Radford DJ, Garlick RB, Pohlner PG. Multiple valvular replacements for hypereosinophilic syndrome. Cardiol Young 2002;12:67-70 https://doi.org/10.1017/S1047951102000136
  4. Weller PF, Bubley GJ. The idiopathic hypereosinophilic syndrome. Blood 1994;83:2759-79
  5. Watanabe K, Tounilhac O, Camilleri LF. Recurrent thrombosis of prosthetic mitral valve in idiopathic hypereosinophilic syndrome. J Heart Valve Dis 2002;11:447-9
  6. Hendren WE, Jones EJ, Smith MD. Aortic and mitral valve replacement in idiopathic hypereosinophilic syndrome. Ann Thorac Surg 1988;46:570-1 https://doi.org/10.1016/S0003-4975(10)64701-7
  7. Felice PV, Sawicki J, Anto J. Endomyocardial disease and eosinophilia. Angiology 1993;44:869-74 https://doi.org/10.1177/000331979304401104
  8. Parrillo JE, Fauci AS, Wolff SM. Therapy of the hypereosinophilic syndrome. Ann Intern Med 1987;89:167-72