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

Yang, Suck Chul; Lee, Kyung Sang; Yoon, Ho Joo; Shin, Dong Ho; Park, Sung Soo; Lee, Jung Hee; Park, Choong Ki

doi:1996.43.2.251

Tuberc Respir Dis > Volume 43(2); 1996 > Article

Case Report

Tuberculosis and Respiratory Diseases 1996;43(2):251-256.
DOI: https://doi.org/10.4046/trd.1996.43.2.251 Published online April 1, 1996.

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

Suck Chul Yang, Kyung Sang Lee, Ho Joo Yoon, Dong Ho Shin, Sung Soo Park, Jung Hee Lee, Choong Ki Park

¹Department of Internal Medicine, College of Medicine, Hanyang University, Seoul, Korea.
²Department of Diagnostic Radiology, College of Medicine, Hanyang University, Seoul, Korea.

Abstract

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.

Key Words: Kartagener's syndrome, Respiratory failure, Heart failure