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Twist of Fate: A Case of Kartagener's Syndrome (Review of Literature) FREE TO VIEW

Cristito Alea*, MD; Ma. Paz Mateo, MD
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Philippine Heart Center, Quezon City, Philippines

Chest. 2012;142(4_MeetingAbstracts):1000A. doi:10.1378/chest.1389246
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SESSION TYPE: Miscellaneous Global Case Report Posters

PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION: A disease is composed of constellation of signs and symptoms. They can rule in or rule out a certain diagnosis. This is a case of a young lady whose a simple twist of fate changed her life forever.

CASE PRESENTATION: This is a case of F.P., 20 year old female, single, student, Filipino, residing at Natividad Nueva Ecija, Philippines. She was first seen in our OPD 2 years ago (July 2010) due to chest pain and difficulty of breathing. She had repeated bouts of upper and lower respiratory tract infections occurring >5 x / year since she was 6 years old. She was previously treated with PTB. On examination of the chest, she was noted to have crackles all over lung fileds. She has adynamic precordium located at the 5th ICS right mid clavicular line. CXR showed hyperaerated lung with haziness in the right lower lung field and dextrocardia. CBC showed slight leukocytosis with predominance of neutrophils. PPD was 15mm. 12L ECG done showed Hypertrophy of anteriorly located ventricle;right sided heart with mirror image arrangement. 2D echocardiogram showed congenital heart disease, atrial septal defect secundum type; dextrocardia, mirror image.She was referred to our service for pre-operative evaluation prior to contemplated ASD closure. Chest X-ray done showed right retrocardiac density, hyperaerated lung fileds, with bulk of the heart in the right, right sided aorta, and intact diaphragm. On examination, she has bibasal crackles. PFT done showed severe obstructive lung defect with no significant post-bronchodilator response. X-ray of the paranasal sinus showed sinusitis. Chest CT Scan revealed situs inversus totalis and dextrocardia, the right mainstem bronchus is hyperarterial while the left is eparterial,marked volume loss of the right lower lobe with associated varicose and cystic bronchiectasis, included scan of the upper abdomen showed stomach and spleen to be in the right hemiabdomen while the lung is in the left. Patient was clinically diagnosed as a case of Kartagener’s Syndrome.

DISCUSSION: The prevalence of Kartagener’s Syndrome is hard to ascertain. It has estimates varying from 1 in 4000 to 1 in 40,000. This patient, not only presents with dextrocardia but atrial septal defect as well. However, Atrial Septal defect among dextrocardia is uncommon. This patient was referred to our service for preoperative evaluation for ASD closure. It was on review of her signs and symptoms with the help of imaging studies available that she was clinically diagnosed with Kartagener’s Syndrome. This patient was treated as a case of pulmonary tuberculosis based on the premise of positive PPD test and exposure to someone with TB growing up. When she was tested for sputum AFB and culture, both diagnostics revealed negative results. She was clinically diagnosed Kartagener’s Syndrome because further confirmatory tests proved to be expensive for the patient’s family. Some cases of Kartagener’s Syndrome were diagnosed just based on clinical picture. Patient is improving clinically on current management. Plan for consecutive follow up is to do nasal biopsy when she is infection free. Her immunization record will be updated. Physiotherapy and physical exercise are emphasized to the patient.

CONCLUSIONS: A case of 20 year old female who presented with situs inversis, chronic sinusitis and bronchiectasis, clinically diagnosed with Kartagener’s Syndrome was presented. Screening, Diagnosis, and Management of Kartagener’s Syndrome was done.

1) Arch Dis Child. 2010 Jan;95(1):51-2

2) KARTAGENER’S SYNDROME, Ihsan-ullah Mahsud and Shahab-ud Din, Gomal Journal of Medical Sciences July-Dec 2006, Vol. 4, No. 2

3) Kartagener’s syndrome- A case report, Jayita Poduval* and Murali Poduval, Medical Case Studies Vol. 2(2), pp. 15-18, February 2011

DISCLOSURE: The following authors have nothing to disclose: Cristito Alea, Ma. Paz Mateo

No Product/Research Disclosure Information

Philippine Heart Center, Quezon City, Philippines




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