What are the treatment options for chronic pulmonary embolism (CPE)?

From the Guidelines

Treatment for chronic pulmonary embolism, also known as chronic thromboembolic pulmonary hypertension (CTEPH), primarily involves lifelong anticoagulation therapy and consideration for pulmonary thromboendarterectomy (PTE) surgery in eligible patients, as recommended by the 2015 ESC/ERS guidelines 1. The standard anticoagulation regimen includes warfarin with a target INR of 2.0-3.0, or direct oral anticoagulants (DOACs) such as rivaroxaban 20mg daily, apixaban 5mg twice daily, or edoxaban 60mg daily. Some key points to consider in the treatment of CTEPH include:

• Lifelong anticoagulation is recommended in all patients with CTEPH, as stated in the 2015 ESC/ERS guidelines 1
• Surgical pulmonary endarterectomy is the recommended treatment for patients with CTEPH, as suggested by the 2009 European Respiratory Journal guidelines 1 and the 2016 Chest guideline and expert panel report 1
• Riociguat is recommended in symptomatic patients who have been classified as having persistent/recurrent CTEPH after surgical treatment or inoperable CTEPH by a CTEPH team, as recommended by the 2015 ESC/ERS guidelines 1
• Balloon pulmonary angioplasty may be considered for inoperable patients with accessible lesions, as suggested by the 2015 ESC/ERS guidelines 1 Supportive care includes diuretics for fluid overload, oxygen therapy for hypoxemia, and pulmonary rehabilitation to improve exercise tolerance. Early evaluation at a specialized CTEPH center is crucial as surgical intervention can be curative in appropriate candidates, while untreated CTEPH leads to progressive right heart failure, as highlighted in the 2016 Chest guideline and expert panel report 1. The disease results from incomplete resolution of pulmonary emboli that organize into fibrous tissue, causing vessel obstruction and pulmonary hypertension, which makes specialized assessment essential for optimal outcomes. It is essential to note that the selection of patients for surgery should be based on the extent and location of the organized thrombi, on the degree of PH, and on the presence of co-morbidities, as stated in the 2009 European Respiratory Journal guidelines 1. In patients who are not candidates for surgery, medical therapy with riociguat or other PAH-specific drugs may be considered, as suggested by the 2015 ESC/ERS guidelines 1 and the 2009 European Respiratory Journal guidelines 1.

From the FDA Drug Label

For patients with a first episode of DVT or PE secondary to a transient (reversible) risk factor, treatment with warfarin for 3 months is recommended For patients with a first episode of idiopathic DVT or PE, warfarin is recommended for at least 6 to 12 months. For patients with two or more episodes of documented DVT or PE, indefinite treatment with warfarin is suggested

The treatment for chronic pulmonary embolism with warfarin is as follows:

• For a first episode of idiopathic pulmonary embolism (PE), treatment with warfarin is recommended for at least 6 to 12 months.
• For patients with two or more episodes of documented DVT or PE, indefinite treatment with warfarin is suggested. The dose of warfarin should be adjusted to maintain a target INR of 2.5 (INR range, 2.0 to 3.0) for all treatment durations 2.

From the Research

Treatment Options for Chronic Pulmonary Embolism

• Pulmonary endarterectomy is considered the gold standard treatment for chronic thromboembolic pulmonary hypertension (CTEPH) 3, 4, 5, 6
• Balloon pulmonary angioplasty is also a recommended treatment option for CTEPH, especially for patients with inoperable disease or as a bridge to pulmonary endarterectomy 3, 6
• Targeted medical therapy, including riociguat and treprostinil, is approved for patients with inoperable CTEPH 3, 6
• Medical treatment with pulmonary arterial hypertension (PAH)-targeted therapies, such as endothelin receptor antagonists, phosphodiesterase inhibitors, and prostacyclin analogues, may be considered for patients with inoperable disease or as a bridge to pulmonary endarterectomy 7

Evaluation and Referral

• Patients with suspected CTEPH should be referred to a specialist center for right-heart catheterization and pulmonary angiography 4, 5
• Evaluation of patients with CTEPH in experienced centers is mandatory to determine the best course of treatment 3
• Diagnostic work-up for CTEPH should include ventilation-perfusion scintigraphy, CT angiography, and pulmonary angiography 4, 5

Anticoagulation and Prevention

• Anticoagulation is recommended to protect against recurrence of pulmonary embolism, which has a high mortality rate 5
• The recommended duration of anticoagulation for patients with reversible PE risk factors is 3 months, while extended duration of anticoagulation is preferred for patients with idiopathic PE or persistent risk factors 5