A Case of Kartagener Syndrome With Pulmonary Hypertension

Abstract

Primary ciliary dyskinesia (PCD) is a genetically heterogeneous syndrome caused by defect in motile cilia. The true prevalence is unknown but estimated to affect 1:20,000 to 1:100,000 people. Estimated incidence is 1 per 10 to 20,000 births. In 1933, Kartagener described the PCD syndrome as the triad of situs inversus, bronchiectasis, and either nasal polyps or recurrent sinusitis, while the description by Afzelius in 1976 of the defects in the ultrastructure of ciliary dynein arms revealed the basis of this condition. Thus, clinical findings include respiratory distress in neonates, recurrent respiratory tract infections, bronchiectasis, situs inversus, infertility, and heterotaxy in approximately 50%. Here we report a rare case of a young female with recurrent respiratory symptoms, dextrocardia, and situs inversus, who was misdiagnosed as a case of pulmonary tuberculosis and had received three courses of anti-tubercular therapy since year 2003, and finally diagnosed as Kartagener syndrome with severe pulmonary hypertension.

ECG Abnormalities in Patients with Acute Exacerbation of Bronchiectasis and Factors Associated with High Probability of Abnormality

“…These abnormalities correlated mainly with disorders in the function of the right ventricle that carried a specific importance to have a right catheterization for pulmonary hypertension diagnosis after echocardiography was not enough in such patients [36,37]. Another specialty of RAD is the correlation with the cases of PCD, as it is the most ECG abnormality in patients with Kartagener disease [38][39][40]. Three of the patients included in the study had the diagnosis of Kartagener disease, and some had a dextrocardia and RAD on ECG.…”