From the Guidelines
Chronic thromboembolic pulmonary hypertension (CTEPH) should be primarily treated with pulmonary endarterectomy (PEA) surgery for eligible patients, as it can be curative by removing the obstructive material, and lifelong anticoagulation is recommended for all patients with CTEPH. The management of CTEPH requires a multidisciplinary team approach, including experts in pulmonary hypertension and surgical techniques.
- The diagnosis of CTEPH is based on the presence of pre-capillary pulmonary hypertension (PH) in patients with multiple chronic/organized occlusive thrombi/emboli in the elastic pulmonary arteries, as stated in the guidelines 1.
- Surgical PEA in deep hypothermia circulatory arrest is recommended for patients with CTEPH, and the assessment of operability and decisions regarding other treatment strategies should be made by a multidisciplinary team of experts 1.
- For patients who are not surgical candidates, balloon pulmonary angioplasty (BPA) may be considered as an option to mechanically open narrowed vessels, and medical therapy includes riociguat (Adempas), which is the only FDA-approved medication for inoperable CTEPH, typically started at 1 mg three times daily and titrated up to 2.5 mg three times daily as tolerated 1.
- Lifelong anticoagulation with warfarin (target INR 2.0-3.0) or direct oral anticoagulants is essential to prevent recurrent clots, as recommended in the guidelines 1.
- CTEPH requires management at specialized centers with expertise in pulmonary hypertension and surgical techniques, and early diagnosis is critical, as untreated CTEPH leads to progressive right heart failure, as stated in the guidelines 1.
- The condition develops in approximately 3-4% of patients after acute pulmonary embolism, making follow-up important for patients with persistent shortness of breath after PE treatment, and screening for CTEPH in asymptomatic survivors of PE is currently not recommended 1.
From the Research
Definition and Treatment of CTEPH
- Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare form of pulmonary hypertension characterized by precapillary pulmonary hemodynamic parameters with chronic thrombotic occlusion of the pulmonary vasculature 2.
- The treatment of CTEPH includes pulmonary thromboendarectomy (PTE), which remains the recommended treatment for patients with surgically accessible disease 2, 3.
- For patients with inoperable CTEPH, medical therapies such as riociguat, endothelin receptor blockers, and treprostinil may be used 2, 4.
Riociguat as a Treatment Option
- Riociguat is a soluble guanylate cyclase (sGC) stimulator that directly stimulates sGC independently of nitric oxide (NO) and increases sGC sensitivity for NO 5.
- Riociguat has been shown to improve hemodynamics, right ventricular function, quality of life, and functional capacity in patients with CTEPH 2.
- Switching from a phosphodiesterase 5 inhibitor (PDE5i) to riociguat may be a safe and effective treatment strategy for patients with CTEPH who have an inadequate clinical response to PDE5i 5, 4, 6.
Diagnostic Work-up and Treatment Algorithm
- The diagnostic work-up for CTEPH should include ventilation-perfusion scintigraphy, CT angiography, and right-heart catheterization 3.
- The treatment algorithm for CTEPH involves PTE as the first-line treatment for surgically accessible disease, and medical therapies such as riociguat for inoperable disease 2, 3.
- Balloon pulmonary angioplasty (BPA) may be a well-tolerated and effective adjunct to pharmacological treatment for patients with inoperable subsegmental disease 2.