Cor pulmonale

Answer 1

Definition

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.

Alternative Names

Right-sided heart failure

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.

Any condition that leads to prolonged high blood pressure in the arteries of the lungs (called pulmonary hypertension) puts a strain on the right side of the heart. When the right ventricle is unable to properly pump against these abnormally high pressures, it is called cor pulmonale.

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

• Central sleep apnea
• Chronic obstructive pulmonary disease (COPD)
• Chronic thromboembolic pulmonary disease
• Cystic fibrosis
• Interstitial lung disease
• Kyphoscoliosis
• Obstructive sleep apnea
• Pneumoconiosis
• Primary pulmonary hypertension
• Pulmonary vascular disease
• Secondary pulmonary hypertension

Symptoms

• Chest discomfort, usually in the front of the chest
• Exercise intolerance
• Shortness of breath
• Swelling of the feet or ankles
• Symptoms of underlying disorders (wheezing, coughing)

Signs and tests

• Abnormal fluid collection in the abdomen
• Abnormal heart sounds
• Bluish color to the skin (cyanosis)
• Enlargement of the liver
• Swelling (distension) of the neck veins, indicating high right-heart pressures
• Swelling of the ankles

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

Treatment is directed at the illness that is causing cor pulmonale. Supplemental oxygen may be prescribed to increase the level of oxygen in the blood.

There are many medicines available to treat cor pulmonale.

• Bosentan or sildenafil may be given by mouth
• Calcium channel blockers are often used to treat early cases
• Prostacyclin may be given through injection or breathing in (inhalation)

Blood thinning (anticoagulant) medications may also be prescribed. Surgery may be needed to reverse heart defects that cause the condition. In very advanced cases, a heart and lung transplant may be advised.

Expectations (prognosis)

The outcome depends on the cause of the condition. Giving oxygen often improves symptoms, stamina, and survival.

Treating primary pulmonary hypertension often leads to greater stamina and a longer life. In some cases, a lung transplant or heart-lung transplant can extend survival.

Complications

Progressive pulmonary hypertension and cor pulmonale may lead to:

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

Calling your health care provider

Call your health care provider if you experience 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

Barst RJ. Pulmonary hypertension. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 67.

Answer 2

Definition

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.

Alternative Names

Right-sided heart failure

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 against these higher pressures. 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 disease or condition causing 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

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 health care provider if you experience 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

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.

Reviewed By

Review Date: 05/01/2011

Linda J. Vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assistant Studies, University of Washington, School of Medicine; 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., Inc.