From the Guidelines
Treatment for Group 4 Pulmonary Hypertension (Chronic Thromboembolic Pulmonary Hypertension, CTEPH) The primary treatment for CTEPH is pulmonary endarterectomy (PEA) surgery, which is a potentially curative option, as it can significantly improve hemodynamics and symptoms in selected patients 1, 2.
Anticoagulation Therapy
All patients with CTEPH should receive life-long anticoagulation with vitamin K antagonists, such as warfarin, with a target international normalized ratio (INR) of 2.0-3.0, to prevent further blood clots from forming 1, 3, 4.
Medical Therapy
Riociguat, a soluble guanylate cyclase stimulator, may be prescribed for patients with inoperable CTEPH or persistent/recurrent PH after PEA, to improve exercise capacity and WHO functional class 3, 4, 5.
Surgical Intervention
PEA surgery is typically performed in patients with more severe disease and is usually done at a specialized center. The decision to undergo PEA surgery should be made by a multidisciplinary team of experts, including at least one experienced PEA surgeon 1, 2, 4.
Other Treatment Options
- Balloon pulmonary angioplasty (BPA) may be considered in patients who are technically non-operable or carry an unfavourable risk-benefit ratio for PEA 4, 5.
- Oxygen therapy and diuretics, such as furosemide, may be used to manage symptoms and fluid buildup 3, 5.
- Lung transplantation may be considered in advanced cases that are not suited for PEA 2.
It is essential to work closely with a healthcare provider to determine the best course of treatment and to monitor the condition regularly.
From the Research
Treatment Options for Group 4 Pulmonary Hypertension (CTEPH)
The treatment for Chronic Thromboembolic Pulmonary Hypertension (CTEPH), classified as World Health Organization (WHO) group 4 pulmonary hypertension, includes:
- Surgery: Pulmonary thromboendarterectomy is the gold standard treatment and offers better improvements in clinical and hemodynamic parameters compared with medical therapy 6, 7.
- Medical therapy: Pharmacological agents such as phosphodiesterase Type 5 inhibitors, endothelin receptor antagonists, soluble guanylate cyclase stimulators, and prostacyclin are used to treat inoperable CTEPH 8, 9, 10.
- Balloon pulmonary angioplasty: This is an interventional procedure that uses telescoping catheters to mechanically disrupt chronic clot material and relieve pulmonary vascular obstruction, and is a viable option for inoperable CTEPH 7, 10.
Considerations for Treatment
The management of CTEPH requires a multidisciplinary team, operability assessment, and an experienced surgical center 6, 7. The choice of treatment depends on the individual patient's condition, with surgery being the preferred option for operable patients, and medical therapy and balloon pulmonary angioplasty being considered for inoperable patients 6, 7, 8, 9, 10.
Emerging Therapies
Recent developments have made balloon pulmonary angioplasty a viable option for inoperable CTEPH, and riociguat, a soluble guanylate cyclase stimulator, has been approved for the treatment of inoperable CTEPH, demonstrating improvements in functional capabilities and pulmonary hemodynamic end points 10.