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.

Korean Journal of Pediatrics
|September 15, 2012
PubMed

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.

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