Hepatopulmonary syndrome diagnostic study of choice

Hepatopulmonary syndrome diagnostic triad. Diagram by Soroush Seifirad, MD.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Soroush Seifirad, M.D.[2]

Overview

There is no single diagnostic study of choice for the diagnosis of hepatopulmonary syndrome, but hepatopulmonary syndrome can be diagnosed based on history of liver disease, atrial blood gas analysis (widened alveolar-arterial oxygen gradient measurement); and evidences of intra-pulmonary vascular dilation or arterio-venous communications that result in a right-to-left intrapulmonary shunt.

Diagnostic Study of Choice

Study of choice:

There is no single diagnostic study of choice for the diagnosis of hepatopulmonary syndrome, but hepatopulmonary syndrome can be diagnosed based on history of liver disease, atrial blood gas analysis (widened alveolar-arterial oxygen gradient measurement); and evidences of intra-pulmonary vascular dilation or arterio-venous communications that result in a right-to-left intrapulmonary shunt.

Investigations:

• Among the patients who present with clinical signs of hepatopulmonary syndrome, the contrast-enhanced transthoracic echocardiography with agitated saline is the most specific test for the diagnosis.
• Among the patients who present with clinical signs of hepatopulmonary syndrome, the atrial blood gas analysis is the most sensitive test for diagnosis.

Diagnostic results

The following findings are confirmatory for hepatopulmonary syndrome: (discussed in details)

• Presence of bubbles on the left heart in contrast-enhanced transthoracic echocardiography with agitated saline
• Presence of radioactivity on the left heart in Technetium 99m-labeled macroaggregated albumin scanning
• Diffusion defect in atrial blood gas analysis particularly studying
• Abnormal liver function studies
• Abnormal pulmonary function test