HEPATOPULMONARY SYNDROME

Hepatopulmonary Syndrome Type I/II; Hepato Pulmonary Syndrome

Hepatopulmonary syndrome (HPS) is a severe condition involving shortness of breath and hypoxemia in people with chronic liver disease that has advanced to the point that it affects their lungs. People with this disorder have low arterial blood oxygen levels (hypoxemia) caused by expansion (dilation) of the blood vessels in the lungs. The expanded blood vessels make it difficult for the lungs to deliver an adequate supply of oxygen to the body. HPS affects both liver and pulmonary (lung) functioning.

Diagnostic testing: The following tests are helpful in establishing the diagnosis of HPS:

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  Contrast enhanced echocardiogram with evidence of intrapulmonary vascular dilation provides a definitive diagnosis of HPS;

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  Arterial blood gas analysis;

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  Chest x-rays;

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  Computed tomography (CT) scans; and

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  Transthoracic echocardiography (TTE) .

Physical findings:

The signs of impaired liver functioning may include:

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  Gastrointestinal bleeding;

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  Esophageal varices;

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  Ascites;

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  Palmar erythema;

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  Spider nevi; and

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  Enlarged spleen (splenomegaly).

The signs of pulmonary involvement may include:

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  Digital clubbing (the appearance of changes in the areas under and around the toenails and fingernails);

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  Cyanosis (a condition in which the lips, fingers, and toes appear blue);

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  Dyspnea (shortness of breath);

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  Platypnea (shortness of breath that is relieved when lying down and worsens when standing or sitting); and

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  Orthodeoxia (fall in arterial blood oxygen while in the upright position).

ICD-9: 573.5, 573.8

HPS worsens the prognosis of individuals with cirrhosis and other liver diseases. Individuals who are not candidates for liver transplantation have a median survival of 2 years. Mortality is usually associated with complications of hepatic disease.

Liver transplantation is the only definitive treatment for HPS. Alternative treatments are supportive and symptomatic. Supplemental oxygen or somatostatin inhibits vasodilation (dilation of the blood vessels).

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation:

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  Clinical history and physical examination that describes the diagnostic features of the impairment;

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  Pulmonary function tests including diffusing capacity (DLCO), spirometry, and arterial blood gas studies (ABGs); and

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  Documentation of intrapulmonary arteriovenous shunting by contrast-enhanced echocardiography or macroaggregated albumin lung perfusion scan.

REMARKS

Listing level severity must be documented.

Listing level severity must be documented.