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What Is Cor Pulmonale?

Medically Reviewed by Dan Brennan, MD on June 18, 2021

Cor pulmonale is a loosely defined term that describes the change in structure or function of the right ventricle of the heart. The right ventricle of the heart is responsible for carrying deoxygenated blood to the lungs.

A primary underlying lung condition causes cor pulmonale. It typically presents itself in chronic, slowly progressive ways but can also be onset and acute. Issues with the left side of the heart are never considered cor pulmonale.

Causes

It is best to think of cor pulmonale as a response to another primary condition that can then cause other symptoms. 

The symptom that causes these other symptoms or conditions is called pulmonary hypertension. Pulmonary hypertension is a type of high blood pressure that makes the blood vessels in your lungs become damaged or break. This damage slows down the blood’s journey through the lungs, and your heart has to exert more energy to pump blood through your lungs. Long term, this causes your heart’s muscles to weaken.

Some of these primary conditions include: 

  • Chronic obstructive pulmonary disease. This is a lung disease in which the bronchial tubes of the lung become inflamed. It is the most common cause of cor pulmonale. It is often caused by smoking or being exposed to smoky or poorly ventilated environments. Its symptoms include wheezing, chest pain, trouble breathing, respiratory infections, lethargy, weight loss, and swelling of the lower limbs.  
  • Interstitial lung disease. This condition involves scarring or inflammation of the tissue surrounding the lung’s air sacs, blood vessels, and passageways. The effects of interstitial lung disease on the lungs can make them stiff, which makes breathing difficult. Symptoms for this include difficulty breathing, coughing, lessened ability to exercise, fatigue, and weight loss.
  • Idiopathic pulmonary arterial hypertension. Idiopathic pulmonary arterial hypertension is simply just pulmonary hypertension in which the cause is unknown.
  • Obstructive sleep apnea. This type of sleep apnea is in which your throat muscles constantly relax and then block your throat. The block can cause your airway to be closed off and become a serious condition. It can cause cardiovascular problems and chronic fatigue. Symptoms include fatigue, snoring, gasping for air during sleep, sore throat or mouth, headaches, mood changes, high blood pressure, sweating, and lessened libido.
  • Kyphoscoliosis. Kyphoscoliosis or adult scoliosis is a condition of the spine in which your spine does not have a natural curvature. The causes can be unknown, disease-related, congenital, or muscle-related. It is associated with pulmonary hypertension through its connection with the chest wall. 
  • Scleroderma. Scleroderma is not one disease but a group of them. These diseases involve the skin and connective tissues hardening and tightening. The causes and symptoms vary greatly depending on which type of scleroderma you have. In the lungs, it can scar and impair the lung’s functioning. Its causes can be genetic, environmental, or immune.
  • Cystic fibrosis. A genetic disorder causes cystic fibrosis. It causes consistent lung infections and, over time, decreases your ability to breathe. Its symptoms can be salty skin, coughing, phlegm, lung infections, difficulty breathing, weight gain, irregular bowel movements, and male infertility.
  • Obesity hypoventilation syndrome. This is a disorder that some people who are obese get when they have too little oxygen and too much carbon dioxide in their blood. Not much is known about its causes other than it occurs only in obese people. It is characterized by obesity, hypoventilation in the day, and disordered breathing during sleep. 
  • Massive pulmonary embolism. Also known as a heart attack. This is the most common cause of acute cor pulmonale. It often can look like a myocardial infarction or a disturbance to the muscle tissue within the heart. Massive pulmonary embolism refers to a type of clotting that starts in your legs and arteries and travels to your lungs. These lung clots then cause pulmonary hypertension due to the effort the heart must make to pump the clotted blood in and out of your lungs.

Symptoms

Some of the symptoms of cor pulmonale are:

  • Trouble breathing during exercise
  • Fatigue
  • Lethargy (lack of energy)
  • Passing out during exertion (physical or mental effort)
  • Chest pain during physical activity
  • Excess fluid that leads to swelling in the body and lower extremities
  • Swelling in various parts of the body

Upon further examination, your doctor might find that you have some other symptoms of cor pulmonale.

These symptoms can include: 

  • Jugular venous distension. This happens when your venous system is being put under so much pressure that it causes your jugular vein to bulge. Often, you might be able to see the bulge on the right side of your neck, but to be sure; you should get tested by a medical provider. 
  • Peripheral edema. This is a symptom of your heart weakening and not properly pumping your blood through your body. Peripheral edema is when you have a build-up or builds up in fluid in your arms and lower extremities.
  • Irregular cardiovascular health. Cor pulmonale causes your heart to work differently. Due to this, you will have an irregular heartbeat, and your heart might sound different than before. 
  • Swelling in the abdomen. There are several conditions related to cor pulmonale that you might develop, like swelling in the liver and peritoneal cavity that will make your abdomen swell. 

Testing and Diagnosis

Under normal conditions, the right side of the heart pumps in a more relaxed, low-pressure way at about one-tenth of the power that other parts of the systemic arteries. Having to pump harder due to cor pulmonale causes shifts in other related vascular factors such as muscle production in the arteries, levels of nitric oxide, and endothelin 1 (ET1). Nitric oxide and ET1 are chemicals that help the arteries. Cor pulmonale also impacts the levels of platelets in your blood.

To understand whether or not you have cor pulmonale, your doctor may order the following tests:

  • Chest radiograph, a kind of X-ray {Food and Drug Administration: "Radiography."}.  
  • Electrocardiogram (ECG or EKG), which records the electrical signals in your heart.
  • Doppler echocardiography, which checks the velocities of your blood flow.
  • Computed tomography (CT) Scan
  • Magnetic resonance imaging ( MRI)
  • Cardiac catheterization for the right side of your heart, when a small, flexible, hollow tube is put into a blood vessel in your arm, groin, or neck and moved to a blood vessel in your heart.

Treatment

Usually, treatment for cor pulmonale aims to treat the primary condition that causes it.

Often this includes:

  • Oxygen therapy. This is a therapy in which you can intake more oxygen. You can either receive the extra oxygen through nasal prongs or a face mask.
  • Diuretics.Diuretics help decrease the volume around the cavity of the lungs; this allows the whole system to be less clogged. 
  • Cardiac glycosides. This is less popular and has yet to be completely adopted as a treatment by the medical field as a whole but has shown to help reduce pulmonary hypertension.

While there are many treatments for the various underlying conditions that cause cor pulmonale, often the quality of life for people with this condition is poor, and it is hard to utterly get rid of all the symptoms.

WebMD Medical Reference

Sources

SOURCES:

American Heart Association: "How the Healthy Heart Works."

American Lung Association: “Oxygen Therapy.”

American Thoracic Society: “Obesity Hypoventilation Syndrome.”

Cystic Fibrosis Foundation: “About Cystic Fibrosis.”

Cleveland Clinic: “Interstitial Lung Disease.”

Food and Drug Administration: "Radiography."

Gopal, S.; Nagalli, S. StatPearls: Jugular Venous Distention. StatPearls Publishing, 2021.

Heart Failure Reviews: "Cor pulmonale: the role of traditional and advanced echocardiography in the acute and chronic settings."

Issac, S.; Das, J. StatPearls: Kyphoscoliosis. StatPearls Publishing, 2021.

Johns Hopkins Medicine: "Cardiac Catheterization."

Garrison, D.; Satish Pendela V.; Memon, J. StatPearls: Cor Pulmonale. StatPearls Publishing, 2020.

Mayo Clinic: “COPD,” “Edema," "Electrocardiogram (ECG or EKG)," “Obstructive sleep apnea,” “Pulmonary hypertension,” “Scleroderma."

Mayo Clinic Proceedings: "Doppler Echocardiography: Theory, Instrumentation, Technique, and Application."

Merck Manual: "Cor Pulmonale."

Merriam-Webster: "exertion," "lethargy."

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