Cor pulmonale is failure of the right side of the heart brought on by long-term high blood pressure in the pulmonary arteries and right ventricle of the heart.A.D.A.M.

Causes, incidence, and risk factors

Normally, the left side of the heart produces a higher blood pressure in order to pump blood to the body. The right side of the heart pumps blood through the lungs under much lower pressure.

High blood pressure in the arteries of the lungs is called pulmonary hypertension. The right side of the heart has a harder time pumping blood when this happens. If this high pressure is present for a longer period of time, it puts a strain on the right side of the heart, leading to cor pulmonale.

Almost any chronic lung condition that causes prolonged low blood oxygen levels can lead to cor pulmonale. A few of these causes include:

• Chronic obstructive pulmonary disease (COPD)
• Chronic blood clots in the lungs
• Cystic fibrosis
• Scarring of the lung tissue (interstitial lung disease)
• Severe curving of the upper part of the spine (kyphoscoliosis)
• Obstructive sleep apnea, in which pauses occur during breathing because of airway inflammation

If a lung problem is not the cause, it is called idiopathic cor pulmonale.

Symptoms

Shortness of breath or light-headedness during activity is often the first symptom. You may have a fast heart beat and feel like your heart is pounding.

Over time, symptoms occur with lighter activity or even while at rest. They may include:

• Fainting spells with activity
• Chest discomfort, usually in the front of the chest
• Chest pain
• Swelling of the feet or ankles
• Symptoms of underlying disorders (wheezing, coughing)

Signs and tests

The health care provider will perform a physical exam. This may reveal:

• Fluid build up in the belly area (abdomen)
• Abnormal heart sounds
• Bluish skin (cyanosis)
• Liver swelling
• Swelling (distension) of the neck veins, which is a sign of high right-heart pressures
• Ankle swelling

The following tests may help diagnose cor pulmonale:

• Blood antibody tests
• Blood test for brain natriuretic peptide (BNP)
• Chest x-ray
• CT scan of the chest
• Echocardiogram
• Lung biopsy (rarely performed)
• Measurement of blood oxygen by arterial blood gas (ABG)
• Pulmonary function tests
• Right heart catheterization
• Ventilation and perfusion scan of the lungs (V/Q scan)

Treatment

The goal of treatment is to control symptoms. It is important to treat medical disorders that cause pulmonary hypertension, which can lead to cor pulmonale.

Many new treatment options are becoming available. Medicines that may be prescribed include:

• Ambrisentan (Letairis)
• Bosentan (Tracleer)
• Calcium channel blockers
• Diuretics
• Prostacyclin or similar medications
• Sildenafil

Your doctor will decide which medicine is best for you. You will be closely monitored during treatment to watch for side effects and to see how well the medicine works for you. Never stop taking your medicines without talking to your doctor.

Other treatments may include:

• Blood thinners to reduce the risk of blood clots
• Oxygen, in some cases
• A lung or heart-lung transplant, if medication does not work

As the illness gets worse, you will need to make changes in the home environment and get more help around the home.

Other important tips to follow:

• Avoid strenuous activities and heavy lifting
• Avoid traveling to high altitudes
• Get yearly flu and pneumonia vaccines
• Stop smoking
• Talk to your doctor before becoming pregnant

Expectations (prognosis)

How well you do depends on what caused the condition.

Complications

Cor pulmonale may lead to:

• Life-threatening shortness of breath
• Severe fluid retention
• Shock
• Death

Calling your health care provider

Call your doctor or nurse if you have shortness of breath or chest pain.

Prevention

Avoiding behaviors that lead to chronic lung disease (especially cigarette smoking) may prevent the eventual development of cor pulmonale. Careful evaluation of childhood heart murmurs may prevent cor pulmonale caused by certain heart defects.

References

1. McGlothlin D, De Marco T. Cor pulmonale. In: Mason RJ, Broaddus VC, Martin TR, et al. Murray & Nadel's Textbook of Respiratory Medicine. 5th ed. Philadelphia, Pa: Saunders Elsevier; 2010:chap 56.
2. Massie BM. Heart failure: pathophysiology and diagnosis. In:Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: SaundersElsevier; 2011:chap 58.

Review Date: 5/29/2012.

Reviewed by: Linda J. Vorvick, MD, Medical Director and Director of Didactic Curriculum, MEDEX Northwest Division of Physician Assistant Studies, Department of Family Medicine, UW Medicine, School of Medicine, University of Washington. Denis Hadjiliadis, MD, Assistant Professor of Medicine, Division of Pulmonary, Allergy and Critical Care, University of Pennsylvania, Philadelphia, PA. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.