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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 [1]
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
Related
orthodeoxia
platypnea
General
syndrome
References
- Medical Knowledge Self Assessment Program (MKSAP) 17, 18.
American College of Physicians, Philadelphia 2015, 2018.
- Kubler P, Gibbs H, Garrahy P.
Platypnoea-orthodeoxia syndrome.
Heart. 2000 Feb;83(2):221-3
PMID: 10648502