platypnea-orthodeoxia syndrome

Etiology

• liver disease (hepatic cirrhosis)
  • hepatopulmonary syndrome
• severe pulmonary fibrosis
• patent foramen ovale or atrial septal defect
  • pneumonectomy in patients with patent foramen ovale
• may be associated with inferior wall & right ventricular myocardial infarction with tricuspid regurgitation

Pathology

• right ventricular enlargement leads to tricuspid regurgitation
  • foramen ovale stretches & becomes patent
  • preferential flow of blood across patent foramen ovale in upright position

Clinical manifestations

• platypnea
• orthodeoxia

• may be soft holosystolic murmur that increases with inspiration
• central venous pressure may be elevated

Radiology

• echocardiography with agitated saline

Management

• closure of patent foramen ovale &/or atrial septal defect
• referral for liver transplantation for hepatopulmonary syndrome

More general terms

• syndrome

Additional terms

• orthodeoxia
• platypnea