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Internal Medicine

Portopulmonary syndrome

Portopulmonary hypertension is defined by the coexistence of portal and pulmonary hypertension.

  • Pulmonary artery pressure: >25 mmHg
  • Portal pressure: > 12 mmHg

Clinical features

  • Dyspnea
  • Syncope

Diagnosis

Hemodynamic criteria:

  1. Portal hypertension and/or liver disease
    • Clinical diagnosis: Ascites/varices/splenomegaly
  2. Mean pulmonary artery pressure (MPAP) > 25 mmHg at rest
  3. Pulmonary vascular resistance (PVR) > 240 dynes s cm−5
  4. Pulmonary artery occlusion pressure (PAOP) < 15mmHg or transpulmonary gradient (TPG) > 12 mmHg where TPG = MPAP − PAOP

Imaging

  • Echocardiography

Management

Combination of medical therapy and liver transplantation.

 

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