A Case of Kartagener's Syndrome Presenting as Respiratory and Right Heart Failures

호흡부전과 우심부전을 동반한 Kartagener 증후군 1예

Kartagener's syndrome, a congenital disease transmitted as an autosomal recessive illness with a prevalence of approximately 1:20,000 persons, is characterized by the triple association of situs inversus, bronchiectasis, and sinusitis. Affected persons have an incoordination of ciliary motility that leads to defective mucociliary transport, chronic bronchial infections. Kartagener's syndrome is a subset of the immotile cilia syndrome and therefore all patients with Kartagener's syndrome have immotile cilia with obvious ultrastructural defects in the ciliary axoneme. In the respiratory tract this inability presumably causes impaired clearance of mucus and inhaled particles and results in the chronic infections of the sinuses and bronchial trees that are characterized of the disease. The end-stage phenomenon in Kartagener's syndrome, respiratory or heart failure is a less common event and heart-lung transplantation is becoming an accepted therapy for patients with end-stage pulmonary disease in Kartagener's syndrome in many institutes. We report one case of Kartagener's syndrome in a 25-year-old young woman who was presented as respiratory and right heart failures, with review of literatures.

Kartagener 증후군은 역위, 부비동염 및 기관지 확장증을 보이는 질환으로 일종의 섬모운동장애증후군으로 여겨지고 있다. 저자들은 어렸을 때부터 호흡기 감염이 빈번한 젊은 여성에서 호흡부전과 우심부전을 동반한 Kartagener 증후군 1예를 경험하였기에 이에 문헌고찰과 함께 보고하는 바이다.