Introduction:
The liver is the largest vital organ of the human body. The liver performs many exocrine (glands that make sweat, tears, and digestive fluids) and endocrine functions (complex of glands and organs). Diseased conditions of the liver can lead to many complications and life-threatening conditions. Many symptoms and signs are associated with a diseased liver condition. One of the main complaints of the diseased liver condition is dyspnea (shortness of breath or difficulty in breathing). It is also seen in chronic liver conditions like cirrhosis (end-stage liver disease) and acute liver failure. Regarding the patient with persistent complaints of dyspnea, history and physical examination leads to the correct diagnosis.
What Are the Diseased Liver Conditions That Cause Dyspnea?
HPS (hepato-pulmonary syndrome) and portopulmonary hypertension are the main causes of dyspnea. HPS is a triad of pulmonary shunting, chronic liver disease, and hypoxemia, the main cause of breathlessness in chronic liver disease conditions. Here the dyspnea is always along with platypnea (shortness of breath which worsens in the upright position and improves by lying flat), which becomes worse in chronic liver conditions like portal hypertension or cirrhosis of the liver.
What Causes Dyspnea in Chronic Liver Conditions?
The common sign of all chronic liver conditions is ascites (fluid-filled in the abdomen). This fluid is filled in the peritoneal cavity, and an increase in volume may put pressure on the diaphragm, which affects the lung's capacity to inflate to its maximum capacity and causes shortness of breath. When this fluid translocates to the pleural cavity, a condition called hepatic hydrothorax occurs, which can be treated by Diuretics, and sometimes pleural aspiration occurs.
Why Do I Feel Difficulty and Shortness of Breath?
RBCs (red blood cells) carry oxygenated blood through the blood vessels in the lungs; there are small blood vessels that help in the exchange of oxygen. In HPS syndrome, these vessels get dilated and hinder the oxygen from reaching the blood, which results in less oxygen absorption from the lungs. This low level of oxygen in the blood will result in shortness of breath.
How Is Platypnea Associated With HPS in Chronic Liver Conditions?
People with HPS in chronic liver conditions are sure to have platypnea. Platypnea is troubled breathing in upright positions, that is., while sitting or standing. These conditions can resolve in a flat-lying position (supine) position. This is also termed orthodeoxia.
What Are Other Associated Signs and Symptoms Of Dyspnea With Chronic Liver Disease?
HPS's main sign is shortness of breath which worsens in an upright position and resolves in a supine position. Along with this, other signs are :
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Clubbing (fingers and toenails get affected) - a condition in which tips of fingers spread out and become rounder.
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Spider angiomata (dilated small blood vessels get very close to forming a webbed structure on the surface of the skin).
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Cyanosis (bluish discoloration of the skin) happens due to low oxygen levels in the blood.
Along with these chronic liver conditions, symptoms are also combined.
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Ascites (fluid-filled in the abdomen).
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Nausea and vomiting.
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Unexplained extreme pain in the abdomen.
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Loss of appetite.
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Jaundice (yellowish discoloration of mucous membrane, skin, and whites of eyes).
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Hepatic encephalopathy (nervous system disorder).
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Variceal bleeding (esophageal bleeding).
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Bacterial infection.
What Is the Differential Diagnosis Of Dyspnea in Chronic Liver Disease?
The differential diagnosis of dyspnea in chronic liver disease are:
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Large ascites (fluid-filled in the abdomen) and diaphragmatic sprinting (chronic breathlessness due to increased abdominal size).
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Secondary anemia due to gastrointestinal hemorrhage.
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Hepatic hydrothorax (presence of pleural effusion).
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Cirrhotic myopathy (a serious complication of liver cirrhosis).
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Portopulmonary hypertension (portal hypertension due to chronic liver disease).
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HPS (hepato-pulmonary syndrome) - It is a condition that affects the lungs with advanced liver disease.
Patients who already have a history of cardiac and respiratory disorders are more prone to dyspnea in chronic liver disease. These conditions include:
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Congestive Cardiac Failure: The condition in which the heart muscle fails to pump the blood out of the heart.
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Obstructive Sleep Apnea: When the muscle at the back of your throat relaxes too much.
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Pneumonia: The condition which inflames the air sacs in your lungs.
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Chronic Obstructive Airway Disease: A group of diseases that causes airway blockage.
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Pulmonary Thromboembolic Disease: Blood in the blood vessels gets clotted while entering the lungs.
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Interstitial Lung Disease (ILD): Lung disease caused by scarring of the lung tissue.
How Is Dyspnea in Chronic Liver Disease Evaluated?
Once the HPS is confirmed, the assessment of arterial oxygenation and pulmonary dilation is done. Pulmonary function tests are done.
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Imaging tests: Evidence of intrapulmonary shunting is seen to confirm the diagnosis.
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Chest radiographs (chest X-ray).
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In this condition, hypoxemia can occur during sleep, so it is recommended to assess overnight pulse oximetry.
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Lung perfusion scanning with macro aggregated albumin (MAA scan) - It is done for assessment of the severity of the intrapulmonary vasodilatation.
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Pulmonary angiography - This test is rarely done. (test to see blood circulation in lungs).
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CT (computed tomography) scanning is done: High-resolution CT shows widespread dilation of pulmonary veins but not interstitial lung disease.
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An echocardiogram is done to rule out other associated heart and lung conditions.
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Contrast echocardiogram - A useful diagnostic technique - agitated saline contrast is used to check the intracardiac blood flow.
What Is the Treatment Provided for Dyspnea In Patients with Chronic Liver Disease?
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Initially managed by intravenous (IV) diuretic therapy.
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Supplemental oxygen therapy is the main line of treatment provided for this condition.
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Therapies such as somatostatin which helps in the inhibition of vasodilation, are provided.
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Coil embolization can be done (insertion of the catheter in the femoral artery and navigating to the vascular system).
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Angiography embolization is done (a minimally invasive medical test that can check for vascular system and blood vessels).
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Transplantation of the liver is the only cure for this condition.
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In most cases, the new liver adapts and resolves the damage caused to liver cells. Also, the blood vessels shrink back to their normal position, and shortness of breath disappears. This happens over weeks to months.
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The prognosis is very poor if the treatment is not provided at the right time.
Conclusion:
In chronic end-stage liver disease, chronic dyspnea occurs with a rate of 88 %. This end-stage liver disease causes increased ventilation and a mild reduction in respiratory muscle strength, the cause of this reduction is unknown. This combines various inducing stages of dyspnea in chronic liver conditions. The greatest impact on the prognosis is based on chronic liver disease.
And portal hypertension is associated with dyspnea and hypoxemia along with various mechanisms with pulmonary vascular complications. Though liver transplantation is considered the only cure for the condition, the novel therapeutic approach should be made with emerging evidence that has expanded the scope for patients and improved survival. This condition is rapidly reversible with liver transplantation.
