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.

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

• 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)

• 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 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.

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.

Progressive pulmonary hypertension and cor pulmonale may lead to:

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

Call your health care provider if you experience shortness of breath or chest pain.

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.

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