Cor Pulmonale

Background

• Cor pulmonale is characterized by changes in right ventricular function and/or structure due to an increase in right ventricular afterload caused by increased pulmonary vascular resistance in the setting of pulmonary hypertension as a result of an underlying lung disease.
• Cor pulmonale can be acute as a result of a rapid increase in right ventricular pressure overload (most commonly caused by acute pulmonary embolism) or chronic as a results of a gradual and progressive increase in right ventricular pressure overload (most commonly caused by chronic obstructive pulmonary disease [COPD]).
• Both acute and chronic cor pulmonale are associated with increased mortality.

Evaluation

• Suspect acute cor pulmonale in patients with acute right heart failure who have acute pulmonary disease, such as pulmonary embolism, acute respiratory distress syndrome (ARDS), or nonthrombotic pulmonary embolism.
  • Acute pulmonary embolism (PE) can cause acute right heart failure within minutes, which may present as syncope and right-sided atrial arrhythmias.
  • Presentation of acute right heart failure may include diaphoresis, listlessness, cyanosis, cool extremities, hypotension, and tachycardia.
  • Presentation of cor pulmonale may include hemoptysis, hoarseness, and pulmonary ejection click.
• Suspect chronic cor pulmonale in patients with pulmonary hypertension due to lung disease (such as chronic obstructive pulmonary disease) who have signs and/or symptoms of right heart failure (such as elevated jugular venous pressure or fatigue).
• Overt signs of cor pulmonale are rare, but patients with severe disease may present with peripheral edema, increased abdominal girth, or weight gain.
• Perform echocardiogram as the first-line test to evaluate alterations to the right ventricular structure and function (Strong recommendation).
  • Right ventricular free wall diastolic thickness may be close to normal (< 5 mm) in patients with acute cor pulmonale and dilated (> 9 mm) in patients with chronic cor pulmonale.
  • The combination of a dilated right ventricle and septal dyskinesia in the presence of both systolic and diastolic dysfunction is considered a hallmark of acute cor pulmonale due to acute respiratory distress syndrome (ARDS).
• Consider right heart catheterization in patients who have an unclear diagnosis after echocardiography or refractory right heart failure (Weak recommendation).
• Consider additional non-invasive imaging:
  • Chest x-ray to assess for enlarged right ventricle and pulmonary artery.
  • Chest computed tomography (CT) to asses right ventricle size and function.
  • Cardiac magnetic resonance imaging (MRI) to assess right ventricle size and function as well as potential cause of right heart failure.
• Blood tests and biomarkers are nonspecific for cor pulmonale but may include brain natriuretic peptide (BNP) and N-terminal pro-BNP, liver function tests, including transaminases, renal function tests, cardiac troponin, and D-dimer (for suspected pulmonary embolism).

Management

• For patients with acute cor pulmonale due to pulmonary embolism:
  • Manage medically by cautious volume loading with saline, vasopressors, and inotropes.
  • Use reperfusion treatment that consists of IV thrombolysis in high-risk patients (high-risk defined as persistent arterial hypotension or shock caused by overt right ventricular failure).
  • See also Pulmonary Embolism.
• For patients with acute cor pulmonale due to acute respiratory distress syndrome (ARDS):
  • Manage acute cor pulmonale with optimal management of ventilatory support, prone positioning, fluid status, and pharmacotherapy.
  • See also Acute Respiratory Distress Syndrome (ARDS).
• For patients with chronic cor pulmonale:
  • Refer patients with cor pulmonale and pulmonary hypertension to specialized centers.
  • Treatment is based on the etiology of the underlying lung disease.
  • Consider evaluating patients with end-stage cor pulmonale for lung and heart transplant.
  • Optimize management of underlying lung disease, right heart failure, and pulmonary hypertension.