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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
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. Journal Papers (연구논문) > 1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실)
1. Journal Papers (연구논문) > 1. College of Medicine (의과대학) > Dept. of Thoracic and Cardiovascular Surgery (흉부외과학교실)
Yonsei Authors
김남균(Kim, Nam Kyun)
박수진(Park, Su Jin)
박영환(Park, Young Hwan)
박한기(Park, Han Ki)
최재영(Choi, Jae Young) ORCID logo https://orcid.org/0000-0002-1247-6669
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