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Bronchial compression in an infant with isolated secundum atrial septal defect associated with severe pulmonary arterial hypertension.

 Sung-Hee Park  ;  So Young Park  ;  Sung-Hee Park  ;  So Young Park  ;  Nam Kyun Kim  ;  Su-Jin Park  ;  Han Ki Park  ;  Young Hwan Park  ;  MD  ;  Jae Young Choi  ;  Nam Kyun Kim  ;  Su-Jin Park  ;  Han Ki Park  ;  Young Hwan Park Young Choi  ;  MD 
 Korean Journal of Pediatrics, Vol.55(8) : 297-300, 2012 
Journal Title
 Korean Journal of Pediatrics 
Issue Date
Airway obstruction ; Infant ; Pulmonary arterial hypertension ; Secundum atrial septal defect
Symptomatic pulmonary arterial hypertension (PAH) in patients with isolated atrial septal defect (ASD) is rare during infancy. We report a case of isolated ASD with severe PAH in an infant who developed airway obstruction as cardiomegaly progressed. The patient presented with recurrent severe respiratory insufficiency and failure to thrive before the repair of the ASD. Echocardiography confirmed volume overload on the right side of heart and severe PAH (tricuspid regurgitation [TR] with a peak pressure gradient of 55 to 60 mmHg). The chest radiographs demonstrated severe collapse of both lung fields, and a computed tomography scan showed narrowing of the main bronchus because of an intrinsic cause, as well as a dilated pulmonary artery compressing the main bronchus on the left and the intermediate bronchus on the right. ASD patch closure was performed when the infant was 8 months old. After the repair of the ASD, echocardiography showed improvement of PAH (TR with a peak pressure gradient of 22 to 26 mmHg), and the patient has not developed recurrent respiratory infections while showing successful catch-up growth. In infants with symptomatic isolated ASD, especially in those with respiratory insufficiency associated with severe PAH, extrinsic airway compression should be considered. Correcting any congenital heart diseases in these patients may improve their symptoms.
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1. College of Medicine (의과대학) > Dept. of Pediatrics (소아과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Thoracic and Cardiovascular Surgery (흉부외과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Nam Kyun(김남균)
Park, Su Jin(박수진)
Park, Young Hwan(박영환) ORCID logo https://orcid.org/0000-0001-9802-8017
Park, Han Ki(박한기) ORCID logo https://orcid.org/0000-0002-7472-7822
Choi, Jae Young(최재영) ORCID logo https://orcid.org/0000-0002-1247-6669
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