Unraveling the Complexities of Kartagener's Syndrome: A Case of Bronchiectasis, Isolated Dextrocardia, and Primary Ciliary Dyskinesia in an Adult With Chronic Respiratory Symptoms

Kartagener's Syndrome (KS), a rare autosomal recessive disorder and a subset of Primary Ciliary Dyskinesia (PCD), is characterized by chronic sinusitis, bronchiectasis, and, in approximately 50% of cases, situs inversus. This condition arises from genetic mutations that impair motile cilia function, leading to defective mucociliary clearance, recurrent respiratory infections, and progressive lung damage. While KS is typically diagnosed in childhood, its recognition in adults is often delayed due to symptom overlap with common respiratory conditions such as asthma, chronic obstructive pulmonary disease (COPD), or cystic fibrosis. This case report describes a 40-year-old male who presented with a 10-12-day history of productive cough, intermittent left-sided chest pain, and a longstanding history of recurrent respiratory infections and chronic sinusitis. Diagnostic imaging, including chest X-rays and computed tomography (CT) scans, revealed bilateral bronchiectasis, right lung atelectasis, and isolated dextrocardia, with all other organs maintaining normal anatomical positions (situs solitus), an atypical presentation of KS. Laboratory findings indicated mild neutrophilic leukocytosis, and sputum cultures confirmed <i>Pseudomonas aeruginosa</i> infection, a common pathogen in bronchiectasis. The patient was managed with inhaled bronchodilators, corticosteroids, targeted antibiotic therapy, and chest physiotherapy to enhance mucociliary clearance. A multidisciplinary team, including pulmonologists and cardiologists, was engaged to address the complex, multisystem nature of KS and provide long-term care. This case underscores the diagnostic challenges of identifying KS in adults, particularly with atypical features like isolated dextrocardia, and emphasizes the critical need for comprehensive clinical evaluation, advanced imaging, and consideration of rare genetic disorders in patients with chronic respiratory symptoms.

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Journal
Clinical Case Reports
Published
2026-02-25
DOI
https://doi.org/10.1002/ccr3.72086
Primary Topic
Cystic Fibrosis Research Advances
Type
article
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article

Unraveling the Complexities of Kartagener's Syndrome: A Case of Bronchiectasis, Isolated Dextrocardia, and Primary Ciliary Dyskinesia in an Adult With Chronic Respiratory Symptoms

Imran Hossain, Sairah Ahmed, Ibrahim Khalil, Sakib Abrar
Clinical Case Reports
Cystic Fibrosis Research Advances
article

Unraveling the Complexities of Kartagener's Syndrome: A Case of Bronchiectasis, Isolated Dextrocardia, and Primary Ciliary Dyskinesia in an Adult With Chronic Respiratory Symptoms

Imran Hossain, Sairah Ahmed, Ibrahim Khalil, Sakib Abrar
article en

Abstract

Kartagener's Syndrome (KS), a rare autosomal recessive disorder and a subset of Primary Ciliary Dyskinesia (PCD), is characterized by chronic sinusitis, bronchiectasis, and, in approximately 50% of cases, situs inversus. This condition arises from genetic mutations that impair motile cilia function, leading to defective mucociliary clearance, recurrent respiratory infections, and progressive lung damage. While KS is typically diagnosed in childhood, its recognition in adults is often delayed due to symptom overlap with common respiratory conditions such as asthma, chronic obstructive pulmonary disease (COPD), or cystic fibrosis. This case report describes a 40-year-old male who presented with a 10-12-day history of productive cough, intermittent left-sided chest pain, and a longstanding history of recurrent respiratory infections and chronic sinusitis. Diagnostic imaging, including chest X-rays and computed tomography (CT) scans, revealed bilateral bronchiectasis, right lung atelectasis, and isolated dextrocardia, with all other organs maintaining normal anatomical positions (situs solitus), an atypical presentation of KS. Laboratory findings indicated mild neutrophilic leukocytosis, and sputum cultures confirmed <i>Pseudomonas aeruginosa</i> infection, a common pathogen in bronchiectasis. The patient was managed with inhaled bronchodilators, corticosteroids, targeted antibiotic therapy, and chest physiotherapy to enhance mucociliary clearance. A multidisciplinary team, including pulmonologists and cardiologists, was engaged to address the complex, multisystem nature of KS and provide long-term care. This case underscores the diagnostic challenges of identifying KS in adults, particularly with atypical features like isolated dextrocardia, and emphasizes the critical need for comprehensive clinical evaluation, advanced imaging, and consideration of rare genetic disorders in patients with chronic respiratory symptoms.

Clinical Case ReportsVol. 14(3)
University of Dhaka (BD), Dhaka Medical College and Hospital (BD)
Openalex Percentile: Top 40%
Cystic Fibrosis Research Advances
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