Hepatopulmonary Syndrome

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Contents 1 Introduction 2 Pathology 3 Laboratory 4 Diagnostic-procedures 5 Radiology 6 Management 7 More General Terms 8 References

Introduction

• Arterial hypoxemia in patients with cirrhosis.

Pathology

• low pulmonary vascular tone
• poor or absent hypoxic pressor response
• marked dilation of pulmonary vasculature
• intrapulmonary shunting

Laboratory

• arterial blood gas

• pO2 < 70 mm Hg
• Aa oxygen gradient > 20 mmg Hg

Diagnostic-procedures

• echocardiography bubble study

• bubbles of agitated saline in the left side of the heart within 3-6 beats [2]
• not clear why this documents intrapulmonary shunting

Radiology

• 99Tc macro-aggregated albumin lung perfusion scan

• detection of IV albumin in the brain suggests presence of an intrapulmonary shunt [2]
• not clear why this documents intrapulmonary shunting

Management

• liver transplantation may be curative

More General Terms

• lung disease
• cirrhosis

References

1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 792
2. Medical Knowledge Self Assessment Program (MKSAP) 15, American College of Physicians, Philadelphia 2009

hepatopulmonary syndrome