From the Guidelines
Cor pulmonale is a condition characterized by enlargement and failure of the right side of the heart due to increased pressure in the pulmonary circulation, typically caused by lung disease or other conditions affecting the pulmonary blood vessels, as seen in chronic obstructive pulmonary disease (COPD) and pulmonary arterial hypertension (PAH) 1.
Definition and Causes
Cor pulmonale occurs when the right ventricle must work harder to pump blood through the lungs, leading to enlargement and eventual failure of the right side of the heart. Common causes include:
- Chronic obstructive pulmonary disease (COPD)
- Pulmonary embolism
- Pulmonary hypertension
- Interstitial lung disease
- Sleep apnea
Symptoms and Treatment
Patients with cor pulmonale often experience symptoms such as:
- Shortness of breath
- Fatigue
- Chest pain
- Swelling in the legs and abdomen
- Bluish discoloration of the skin Treatment focuses on addressing the underlying cause while managing symptoms through:
- Oxygen therapy
- Diuretics to reduce fluid retention
- Vasodilators to lower pulmonary pressure
- Anticoagulants if blood clots are involved
Pathophysiology and Management
The condition develops because prolonged high pressure in the pulmonary arteries forces the right ventricle to work harder, causing it to thicken and eventually fail, as described in the evaluation and management of right-sided heart failure 1. Early diagnosis and management of the underlying lung disease or other causes are crucial to prevent progression of cor pulmonale and improve quality of life. Pulmonary rehabilitation has been shown to reduce dyspnea, increase exercise capacity, and improve quality of life in individuals with chronic obstructive pulmonary disease (COPD) 1.
Key Considerations
It is essential to recognize that cor pulmonale is a direct complication of severe COPD, and patients with COPD and PAH have a relatively high frequency of heart failure and coronary heart disease 1. Therefore, early diagnosis and management of the underlying lung disease or other causes are crucial to prevent progression of cor pulmonale and improve quality of life.
From the Research
Definition of Cor Pulmonale
- Cor pulmonale is defined as right ventricular hypertrophy and/or dilation caused by pulmonary hypertension (PH) due to diseases affecting the lung function and structure 2.
- It is characterized by an increase in mean pulmonary arterial pressure (mPAP) above normal physiologic values, which can lead to right-sided heart failure 3.
Causes of Cor Pulmonale
- Chronic obstructive pulmonary disease (COPD) is the leading cause of cor pulmonale, resulting from loss of vascular bed and chronic hypoxic pulmonary vasoconstriction (HPV) due to alveolar wall destruction 2.
- Other causes of cor pulmonale include pulmonary artery hypertension, lung parenchymal diseases, airways disease, pulmonary circulatory disorders, systemic illnesses, and thoracic mechanical abnormalities 3.
Treatment of Cor Pulmonale
- The goals of treatment are to reduce pulmonary arterial hypertension, optimize gas-exchange efficiency, and improve survival 4.
- Treatment strategies include supplemental oxygen, assisted mechanical ventilation, digoxin, diuretics, and pulmonary vasodilator compounds 3, 5.
- Long-term oxygen therapy is the mainstay of treatment, as it selectively vasodilates the pulmonary vascular bed and improves oxygen delivery and mixed venous oxygen tension 5.
Pathophysiology and Outcome
- The right heart is no longer considered an "innocent bystander" in pulmonary hypertension, but rather a key component in its pathophysiology 6.
- The status of right heart function is a major determinant of outcome, and the right heart has become a potential target for novel therapies to treat hypertensive pulmonary vascular disease 6.