Pediatric Idiopathic Pulmonary Artery Hypertension Differential Diagnoses

Updated: Feb 07, 2023
  • Author: Stuart Berger, MD; Chief Editor: Girish D Sharma, MD, FCCP, FAAP  more...
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Diagnostic Considerations

Because many of the symptoms of idiopathic pulmonary artery hypertension (IPAH) are nonspecific and the disorder is relatively rare, the diagnosis may be somewhat difficult to make. Failure to make the diagnosis or significant delay in making the diagnosis delays treatment, and early therapy may be beneficial in a subset of children.

Go to Primary Pulmonary Hypertension and Persistent Newborn Pulmonary Hypertension for more complete information on these topics.

Other problems to consider include the following:

  • Pulmonary venous hypertension (due to left heart obstruction)

  • Upper airway obstruction

  • Diminished ventilatory drive

  • Chest wall abnormalities

  • Pulmonary thromboembolism

  • Exogenous substances (eg, anorexic agents, toxic rapeseed oil, cocaine)

  • Pulmonary veno-occlusive disease

Differential Diagnoses