Kartagener's syndrome

Question 1

What is Kartagener’s syndrome?

Answer 1

Kartagener’s syndrome (also known as primary ciliary dyskinesia) was first described in 1933 and most frequently occurs in examinations due to its association with dextrocardia (e.g. ‘quiet heart sounds’, ‘small volume complexes in lateral leads’)

Pathogenesis

• dynein arm defect results in immotile cilia

Question 2

Features of kartagener’s syndrome?

Answer 2

• dextrocardia or complete situs inversus
• bronchiectasis
• recurrent sinusitis
• subfertility (secondary to diminished sperm motility and defective ciliary action in the fallopian tubes)

Question 3

What may an xray show of a patient with Kartagener’s syndrome?

Answer 3

This patient has x-ray findings consistent with dextrocardia and bronchiectasis (tram-track opacities). Hyperinflation is also seen in this film.