From the Guidelines
Lifelong anticoagulation is recommended in all patients with chronic thromboembolic pulmonary hypertension (CTEPH), and surgical pulmonary endarterectomy (PEA) is the treatment of choice for eligible patients. The treatment for chronic pulmonary embolism, also known as CTEPH, involves a multifaceted approach to manage the underlying thrombotic disease and resulting pulmonary hypertension. According to the 2015 ESC/ERS guidelines 1, lifelong anticoagulation is essential for all CTEPH patients, and the assessment of operability and decisions regarding other treatment strategies should be made by a multidisciplinary team of experts.
Treatment Options
- Surgical PEA in deep hypothermia circulatory arrest is recommended for patients with CTEPH 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 including at least one experienced PEA surgeon 1.
- Off-label use of drugs approved for PAH may be considered in symptomatic patients who have been classified as having inoperable CTEPH by a CTEPH team including at least one experienced PEA surgeon 1.
- Interventional balloon pulmonary angioplasty (BPA) may be considered in patients who are technically non-operable or carry an unfavourable risk:benefit ratio for PEA 1.
Medical Therapy
- Anticoagulation therapy is crucial, and the choice of anticoagulant may depend on individual patient factors.
- Riociguat, a soluble guanylate cyclase stimulator, is specifically approved for CTEPH and improves exercise capacity and pulmonary vascular resistance.
- Other medications, such as phosphodiesterase-5 inhibitors, endothelin receptor antagonists, or prostacyclins, may be used in selected cases under the guidance of a pulmonary hypertension specialist.
Monitoring and Follow-up
- Regular follow-up with pulmonary hypertension specialists is essential to monitor disease progression and adjust treatment as needed.
- Periodic echocardiograms and exercise capacity assessments are crucial to evaluate the effectiveness of treatment and guide further management.
From the FDA Drug Label
The dosage and administration of warfarin sodium tablets must be individualized for each patient according to the particular patient’s PT/INR response to the drug. 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:
- For a first episode of idiopathic pulmonary embolism (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 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.
- Balloon pulmonary angioplasty is a recommended interventional therapy for patients with appropriate target lesions 3, 6.
- Targeted medical therapy, including riociguat and treprostinil, is approved for inoperable patients 3, 7.
- Anticoagulation is essential for preventing recurrence of pulmonary embolism and is recommended for all patients with CTEPH 4, 7.
Patient Evaluation and Management
- Evaluation and treatment of patients with CTEPH should be done in experienced centers 3, 4, 5.
- A multidisciplinary team approach is recommended for treatment decision making 6.
- Patients should be assessed for operability, and those deemed inoperable may benefit from medical therapy or balloon pulmonary angioplasty 7, 6.
- Lung transplantation may be an option for patients who are not candidates for pulmonary endarterectomy 4.
Diagnostic Tests
- V˙/Q˙ scintigraphy is the best screening test for CTEPH 5.
- CT scan imaging and other advanced imaging methods can aid in disease detection and confirmation 5.
- Pulmonary angiography and right heart catheterization are required for confirmation and treatment planning 5.
- Echocardiography can provide an initial indication of pulmonary hypertension 4.