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Published on: November 18, 2018
Bronchial compression in an infant with isolated secundum atrial septal defect associated with severe pulmonary
Sung-Hee Park1, So Young Park, Nam Kyun Kim
1Division of Pediatirc Cardiology, Congenital Heart Disease Center, Severance Cardiovascular Hospital, Department of Pediatrics, Yonsei University Collage of Medicine, Seoul, Korea.
Insights
Pulmonary arterial hypertension (PAH) in infants with atrial septal defect (ASD) can cause airway obstruction. Surgical repair of ASD improved PAH and resolved airway compression in a pediatric case.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Pulmonary Hypertension
Background:
- Symptomatic pulmonary arterial hypertension (PAH) in infants with isolated atrial septal defect (ASD) is uncommon.
- Cardiomegaly in infants with isolated ASD can lead to airway obstruction.
- Severe PAH can present with respiratory insufficiency and failure to thrive.
Purpose of the Study:
- To report a rare case of isolated ASD with severe PAH in an infant.
- To highlight the association between cardiomegaly, PAH, and airway compression.
- To emphasize the importance of considering extrinsic airway compression in infants with symptomatic ASD and PAH.
Main Methods:
- Case report of an infant with isolated ASD and severe PAH.
- Echocardiography to assess right heart volume overload and PAH severity (TR pressure gradient).
- Chest radiography and CT scan to evaluate lung fields and airway compression by the dilated pulmonary artery.
Main Results:
- The infant presented with severe respiratory insufficiency and failure to thrive due to extrinsic airway compression from a dilated pulmonary artery.
- Echocardiography revealed severe PAH (TR gradient 55-60 mmHg).
- Following ASD patch closure at 8 months, PAH significantly improved (TR gradient 22-26 mmHg), and respiratory symptoms resolved with catch-up growth.
Conclusions:
- Infants with symptomatic isolated ASD and severe PAH may experience extrinsic airway compression.
- Surgical correction of ASD can effectively improve PAH and alleviate airway obstruction.
- Early diagnosis and management of congenital heart disease are crucial for symptomatic infants.
Abstract:
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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