Syndrome of shortness of breath and hypoxemia caused by vasodilation in the lungs of patients with liver disease.
- Triad:
- Chronic liver disease or portal hypertension
- Alteration of arterial oxygenation
- Widened age-corrected alveolar arterial oxygen gradient ± arterial hypoxemia
- Intrapulmonary vascular dilatations
Pathophysiology
Clinical features
Symptoms
- Dyspnea
- Platypnea
- Dyspnea induced in upright position and relieved by recumbency
- Orthodeoxia
- Arterial deoxygenation accentuated in upright position and relieved by recumbency
Signs
Diagnosis
- Contrast echocardiography
- M/sensitive test to demonstrate intrapulmonary shunting
Management
- Liver transplantation
- Only established effective therapy
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