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Cor Pulmonale

The World Health Organization defines chronic cor pulmonale as "hypertrophy of the right ventricle resulting from diseases affecting the function and/or the structure of the lungs, except when these pulmonary alterations are the result of diseases that primarily affect the left side of the heart or of congenital heart disease."

Causes

Almost any chronic lung disease or condition causing prolonged low blood oxygen can lead to cor pulmonale. Some common causes of cor pulmonale are:

• Chronic obstructive pulmonary disease ( COPD )
• obstructive lung diseases - for example, bronchiectasis and cystic fibrosis.
• Central sleep apnea
• Mountain sickness
• obesity hypoventilation syndrome (pickwickian syndrome) and upper airway obstruction .
• Primary pulmonary hypertension
• Pneumoconiosis
• Kyphoscoliosis
• living at high altitudes (chronic mountain sickness).
• Diffuse interstitial pulmonary fibrosis
• Chronic thromboembolic pulmonary disease
• Pulmonary vascular disease
• Pulmonary hypertension

Signs and symptoms

As long as the heart can compensate for the increased pulmonary vascular resistance, clinical features reflect the underlying disorder and occur mostly in the respiratory system. They include chronic productive cough, exertional dyspnea, wheezing respirations, fatigue, and weakness.

Progression of cor pulmonale is associated with dyspnea (even at rest) that worsens on exertion, tachypnea, orthopnea, edema, weakness, and right upper quadrant discomfort. Chest examination reveals findings characteristic of the underlying lung disease.

Signs of cor pulmonale and right­sided heart failure include dependent edema; distended neck veins; enlarged, tender liver; prominent parasternal or epigastric cardiac impulse; hepatojugular reflux; and tachycardia.

Decreased cardiac output may cause a weak pulse and hypotension.

Thoracic assessment

Chest examination yields various findings, depending on the underlying cause of cor pulmonale. In COPD, auscultation reveals wheezing, rhonchi, and diminished breath sounds. When the disease is secondary to upper airway obstruction or damage to central nervous system respiratory centers, chest findings may be normal except for a right ventricular lift, gallop rhythm, and loud pulmonic component of S2.

Tricuspid insufficiency produces a pansystolic murmur heard at the lower left sternal border; its intensity increases on inspiration, distinguishing it from a murmur caused by mitral valve disease. A right ventricular early murmur that increases on inspiration can be heard at the left sternal border or over the epigastrium. A systolic pulmonic ejection click may also be heard.

Drowsiness and alterations in consciousness may occur.

Diagnosis

• Echocardiography or angiography indicates right ventricular enlargement, and echocardiography can estimate PAP.
• Chest X-ray shows large central pulmonary arteries and suggests right ventricular enlargement by rightward enlargement of cardiac silhouette on an anterior chest film.
• Electrocardiography frequently shows arrhythmias, such as premature atrial and ventricular contractions and atrial fibrillation during severe hypoxia; it may also show right bundle-branch heart block, right axis deviation, prominent P waves and an inverted T wave in right precordial leads, and right ventricular hypertrophy.

Other tests used to support a diagnosis of cor pulmonale may include arterial blood gas analysis , pulmonary function tests, and hematocrit .

Treatment

Treatment of cor pulmonale is aimed at increasing a patient's exercise tolerance and improving oxygen levels of the arterial blood. Treatment is also aimed at the underlying condition that is producing cor pulmonale. Common treatments include antibiotics for respiratory infection; anticoagulants to reduce the risk of thromboembolism; and digitalis, oxygen, and phlebotomy to reduce red blood cell count. A low-salt diet and restricted fluids are often prescribed.

Alternative treatment

Co-management of the patient with cor pulmonale should be coordinated between the medical doctor and the alternative practitioner. The first step in treatment is to determine the cause of the condition and to evaluate all organ systems of the body. Dietary considerations, for example, a low-salt diet and reduced fluid intake aimed at reducing the edema associated with cor pulmonale, can be supportive aspects of treatment.

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.