What is cor pulmonale (right-sided heart failure due to pulmonary disease)?

From the Guidelines

Cor pulmonale is right-sided heart failure caused by pulmonary disease, characterized by increased pressure and resistance in the pulmonary circulation, leading to right ventricular (RV) dysfunction and failure.

Causes and Pathophysiology

• The condition can result from various pulmonary diseases, including those that cause pulmonary hypertension, such as chronic obstructive pulmonary disease (COPD) and pulmonary embolism 1.
• The RV is adapted to handle low-pressure and high-volume loads, but in cor pulmonale, the increased afterload due to pulmonary hypertension can lead to RV failure 1.
• The relationship between RV and LV stroke volumes and afterload is critical, with the RV being more sensitive to increases in afterload, leading to a steep decline in stroke volume 1.

Clinical Implications

• Cor pulmonale can lead to increased morbidity and mortality due to progressive RV dysfunction and failure 1.
• The condition requires careful assessment and management, taking into account the underlying pulmonary disease and the resulting RV dysfunction 1.
• Elevated filling pressures on the right side of the heart can cause coronary sinus congestion, reducing coronary blood flow and potentially provoking RV ischemia 1.

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, 3, 4.
• It is characterized by right ventricular enlargement secondary to pulmonary hypertension, which can be caused by parenchymal lung disease, derangements of the ventilatory drive, the respiratory pumping mechanism, or the pulmonary vascular bed 4.

Causes of Cor Pulmonale

• Chronic obstructive pulmonary disease (COPD) is the leading cause of cor pulmonale 2, 3, 5.
• Other causes include idiopathic pulmonary fibrosis, obesity-hypoventilation syndrome, and other diseases affecting the lung function and structure 3.
• Pulmonary vascular alterations, including components that are primary lesions of the pulmonary artery, may also contribute to the development of cor pulmonale in some patients with COPD 2.

Pathophysiology of Cor Pulmonale

• Pulmonary hypertension (PH) results in right ventricular enlargement and may lead to right heart failure (RHF) 3.
• Chronic long-standing alveolar hypoxia induces pulmonary vascular remodeling, leading to increased pulmonary vascular resistance (PVR) 3.
• Arterial hypoxemia, hypercapnia, and respiratory acidosis all contribute to the increased afterload on the right ventricle 4.

Diagnosis and Treatment of Cor Pulmonale

• Diagnosis is often difficult, since pulmonary vascular disease, pulmonary hypertension, and cor pulmonale have few specific manifestations, especially early in their evolution 4.
• Echocardiography plays a central role in the diagnostic and therapeutic work-up of patients with cor pulmonale 6.
• Treatment is primarily directed at the underlying pulmonary or ventilatory disorder, rather than at the right ventricular failure per se 4.
• Supplemental oxygen, vasodilators, and other specific therapies are used as indicated to treat the underlying pulmonary disorders 3, 4, 5.