Definition

Cor pulmonale is a condition characterized by changes in the structure and function of the heart's right ventricle (RV), which pumps blood to the lungs. The term "cor" is derived from the Latin word for heart, and "pulmonale" means lung, so cor pulmonale refers to a heart complication caused by underlying lung disease. This condition is also known as right heart failure. Cor pulmonale causes the RV to enlarge, making it less effective at pumping blood. As the disease progresses, this will lead to heart failure.

Cor pulmonale can be acute (occurring over a short period) or chronic (lasting for a long period). Chronic cor pulmonale is typically caused by enlargement and dilation of the RV over months to years, which eventually leads to impaired heart function and, potentially, heart failure.

Causes

Under normal conditions, the lungs rely on the heart to circulate blood from the body to the lungs. When there is an increase in pressure in the pulmonary arteries, it will increase the load on the right ventricle. This load is a result of the heart compensating for the high pressure in the lungs so that the ventricle can pump blood into them. This increase in pressure eventually results in cor pulmonale, and the delivery of blood to the lungs becomes ineffective. Thus, there is a decrease in oxygen transportation throughout the body.

Some of the conditions that can cause increased pressure in the blood vessels of the lungs resulting in chronic cor pulmonale include:

1. Chronic Obstructive Pulmonary Disease (COPD)

A lung disease in which the lung passages become inflamed over a long period. This condition is the most common cause of chronic cor pulmonale. COPD is often caused by smoking, exposure to smoky environments, or poor ventilation.

2. Interstitial Lung Disease

This condition involves scarring or inflammation of the tissue around the lungs' air sacs (alveolus), blood vessels, and tubes. Interstitial lung disease can cause the lungs to become stiff and hard to expand, making it difficult to breathe

3. Idiopathic Pulmonary Arterial Hypertension

Increased pressure in the pulmonary arterial blood vessels of unknown cause

4. Obstructive Sleep Apnea (OSA)

Periods of respiratory arrest (apnea) due to the throat muscles constantly relaxing and then blocking your airway while sleeping. This condition can lead to heart problems and chronic fatigue if it persists

5. Kyphoscoliosis

Also known as adult scoliosis, this is a condition where your spine does not have a natural curvature. It can be disease-related, congenital, or related to muscle disorders

6. Scleroderma

A group of diseases that cause hardening and tightening of the skin and connective tissue. The causes and symptoms vary greatly depending on the type of scleroderma you have. In the lungs, this condition can injure and damage lung function. The cause could be genetic, environmental, or an immune reaction

7. Cystic fibrosis

A genetic disorder that causes consistent lung infections and over time will decrease your ability to breathe

8. Obesity hypoventilation syndrome

This is a disorder that some obese people experience when there is too little oxygen and too much carbon dioxide in their blood

Risk factor

There is an increased risk of developing chronic cor pulmonale if you have any risk factors or underlying lung diseases, such as:

• Chronic obstructive pulmonary disease (COPD), especially from smoking
• Exposure to smoke in the home or work environment
• Obesity
• Interstitial lung disease
• Sleep apnea
• Scleroderma
• Cystic fibrosis

Symptoms

Early symptoms of chronic cor pulmonale are typically difficult to detect because the underlying lung disease also causes symptoms, and some of the underlying diseases may present with similar symptoms.  Chronic cor pulmonale symptoms frequently appear as the underlying lung disorder worsens.

Common symptoms of chronic cor pulmonale include:

• Wheezing sounds when breathing
• Coughing
• Difficulty breathing during exercise or even mildly strenuous physical activity (such as walking up stairs)
• General fatigue
• Shortness of breath
• Dizziness
• Swelling of the feet and/or ankles

In addition, some symptoms indicate an emergency and require immediate attention, such as:

• Severe shortness of breath at rest
• Enlarged veins in the neck
• Chest pain
• Syncope (loss of consciousness, fainting)
• Swelling in the abdomen
• Bluish discoloration of the skin, nail beds, lips, or tongue

Diagnosis

When diagnosing chronic cor pulmonale, your doctor will first conduct an interview, inquiring about your symptoms, how long you have had them, and your medical history, particularly previous lung and heart disease.

Next, the doctor will perform a physical examination. They will look for signs of fluid buildup in the legs and body, irregular heartbeat and breathing sounds, and dilation of the neck veins.

The doctor may also suggest some supporting tests including:

• X-ray of the chest to detect blood clots
• Electrocardiogram (ECG) to record the electrical activity in your heart
• Doppler echocardiography to check the speed of blood flow and provide a visual image of the structure of your heart
• Computed tomography (CT) scan
• Magnetic resonance imaging (MRI)
• Cardiac catheterization of the right side of the heart using a small, flexible tube that is inserted into a blood vessel in your arm, groin, or neck and moved to a blood vessel in your heart

Management

The primary treatment of chronic cor pulmonale is to treat the underlying cause, which could be pulmonary hypertension or COPD. The management goals will be to improve oxygenation and right heart ventricle function. Some treatments may include:

• Medications that can help lower blood pressure and encourage oxygen flow to the lungs
• Blood-thinning medications to help prevent blood clots
• Diuretic medications to increase urine output and lower sodium levels in the blood
• Oxygen therapy
• Surgery for patients with severe cor pulmonale, such as heart or lung transplantation

Complications

Chronic cor pulmonale can cause a variety of complications if not treated promptly, including: 

• Enlargement of the pulmonary veins
• Thickening of the wall of the RV, causing the ventricle to narrow and reducing the volume of blood the heart can pump
• Severe shortness of breath
• Excessive fluid in the body
• Shock
• Death

Prevention

Prevention of cor pulmonale is more focused on preventing the underlying lung disease. There are some steps you can take to prevent it, including:

• Stop smoking
• Controlling certain underlying diseases, such as blood clotting disorders and kidney disease
• Avoiding exposure to irritants to the lungs, such as chemical gases and dust
• Treating sleep apnea
• Maintaining an ideal body weight

When to see a doctor?

Consult with a doctor if you experience symptoms suggestive of chronic cor pulmonale. Visit the Emergency Department immediately if you experience emergency symptoms, such as shortness of breath at rest, chest pain, fainting, abdominal swelling, or bluish discoloration of the skin, nails, lips, or tongue.

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