Tuberculosis and Respiratory Diseases

  • 제65권5호
  • /
  • Pages.430-434
  • /
  • 2008
  • /
  • 1738-3536(pISSN)
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  • 2005-6184(eISSN)
대한결핵및호흡기학회 (The Korean Academy of Tuberculosis and Respiratory Diseases)
권호 표지

선천성 우측 폐동맥 형성부전증 1예

A Case of Isolated Congenital Absence of Right Pulmonary Artery

  • 라성수 (단국대학교 의과대학 내과학교실) ;
  • 김소미 (단국대학교 의과대학 내과학교실) ;
  • 김도형 (단국대학교 의과대학 내과학교실)
  • La, Sung Soo (Department of Internal Medicine, Dankook University College of Medicine) ;
  • Kim, So Mi (Department of Internal Medicine, Dankook University College of Medicine) ;
  • Kim, Doh Hyung (Department of Internal Medicine, Dankook University College of Medicine)
  • 투고 : 2008.08.01
  • 심사 : 2008.09.01
  • 발행 : 2008.11.30
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초록

저자들은 간헐적인 혈담을 주소로 내원한 젊은 남자 환자에서 폐동맥 고혈압을 동반하지 않은 선천성 편측 폐동맥 형성부전증을 진단하여 보고하는 바이다. 선천성 편측폐동맥 형성부전증은 심혈관계 기형 없이 단독으로 일어나는 경우 대부분의 환자에서 경과 관찰 중 특이한 증상 없이 잘 지내지만, 일부 환자에서는 대량 객혈, 심각한 폐동맥 고혈압과 심부전증이 발생하기도 한다. 따라서 조기진단과 주의 깊은 경과 관찰이 중요하다. 폐동맥 고혈압을 가지고 있는 환자에서는 치료 결정에 있어 심도자 검사 및 폐혈관 쐐기조영술을 시행하여 수술적 치료 또는 폐동맥 고혈압에 대한 약물 치료가 고려되어야 한다.

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital anomaly that occurs in association with other cardiovascular anomalies, such as tetralogy of Fallot or ventricular septal defects. On the other hand, it is less commonly found as an isolated finding without accompanying diseases. Isolated UAPA is a rare cause of hemoptysis, and massive hemoptysis has been reported to occur in approximately 18~20% of UAPA patients during their clinical course. Even if a lung resection is considered a treatment option to control life-threatening hemoptysis, the procedure is more difficult than an ordinary lung resection because of the excessive collateral vessels from the systemic circulation. We encountered an isolated UAPA occurring in a young male patient suffering from intermittent blood tinged sputum. To our knowledge, only a few cases of isolated UAPA have been reported in Korea. This case is expected to be a good example to help clinicians better understand isolated UAPA as an unusual cause of hemoptysis.

키워드

참고문헌

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