Cor pulmonale

Alternative names
Right-sided heart failure - cor pulmonale

Definition

Failure of the right side of the heart caused by prolonged high blood pressure in the pulmonary artery and right ventricle of the heart.

Causes, incidence, and risk factors

Normally, the left side of the heart produces a higher level of blood pressure in order to pump blood to the body; the right side pumps blood through the lungs under much lower pressure. Any condition that leads to prolonged high blood pressure in the arteries or veins of the lungs (called pulmonary hypertension) will be poorly tolerated by the right ventricle of the heart. When this right ventricle fails or is unable to properly pump against these abnormally high pressures, this is called cor pulmonale.

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

     
  • Chronic obstructive pulmonary disease (COPD)  
  • Obstructive sleep apnea  
  • Central sleep apnea  
  • Chronicmountain sickness  
  • Cystic fibrosis  
  • Primary pulmonary hypertension  
  • Pneumoconiosis  
  • Kyphoscoliosislung disease  
  • Diffuse pulmonary interstitial fibrosis  
  • Chronic thromboembolic pulmonary disease  
  • Pulmonary vascular disease; pulmonary hypertension

Symptoms

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

Signs and tests

     
  • Bluish color to the skin (cyanosis)  
  • Distension of the neck veins indicating high right-heart pressures  
  • Abnormal fluid collection in the abdomen, enlargement of the liver  
  • Edema (swelling) of the ankles  
  • Abnormal heart sounds

Some tests that may help diagnose cor pulmonale include:

     
  • Echocardiogram (heart ultrasound)  
  • Chest X-ray  
  • CAT scan of the chest  
  • Pulmonary function tests  
  • Swan-Ganz catheterization  
  • V/Q scan  
  • Measurement of blood oxygen by arterial blood gas (ABG)  
  • Lung biopsy (rarely performed)  
  • Blood antibody tests

Treatment

Treatment is directed at the underlying illness. Supplemental oxygen may be prescribed to increase the level of oxygen in the blood.

A diet low in salt is often recommended. Diuretics may be given to remove excess fluid from the body. Calcium channel blockers, intravenous prostacyclin, or the oral medication bosentan may be used to treat pulmonary hypertension.

Surgery may be used to reverse heart defects that cause cor pulmonale. Blood thinning (anticoagulant) medications may also be prescribed.

Expectations (prognosis)

The outcome depends on the underlying cause. Administering oxygen often results in improved symptoms, more stamina, and longer survival.

Treatment of Primary pulmonary hypertension often leads to more stamina and longer life. In some cases, lung transplant or heart-lung transplant can result in longer survival.

Complications
Progressive pulmonary hypertension and cor pulmonale may lead to severe fluid retention, life-threatening shortness of breath, shock, and 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.

Johns Hopkins patient information

Last revised: December 6, 2012
by Dave R. Roger, M.D.

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