Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive and life-threatening disease characterized by high blood pressure in the lungs, caused by the presence of blood clots or thromboemboli in the pulmonary arteries.

CTEPH affects approximately 5% of patients who experience an acute pulmonary embolism. Despite being a serious disease, CTEPH remains underdiagnosed and undertreated, with only a few treatment options available until recently.

Conventional Treatment Options

The conventional treatment options for CTEPH include anticoagulation with warfarin, balloon pulmonary angioplasty, and pulmonary endarterectomy (PEA). Anticoagulation therapy is aimed at preventing the formation of new blood clots.

Balloon pulmonary angioplasty is a minimally invasive procedure that uses a balloon catheter to open up blocked pulmonary arteries. PEA is a surgical procedure that involves the removal of the blood clots from the pulmonary arteries.

Although these treatments can improve the symptoms of CTEPH and prevent disease progression, they are not always effective in all patients and some individuals are not eligible for PEA or angioplasty.

New Treatment Advances

Recent years have seen significant advancements in the treatment of CTEPH. The following are examples of some of the new treatment options that have emerged.

Riociguat

Riociguat is a medication that has been approved by the Food and Drug Administration for the treatment of CTEPH. It is a stimulator of soluble guanylate cyclase (sGC), an enzyme that is involved in the mechanism of pulmonary vascular relaxation.

Riociguat is taken orally and has been shown to improve exercise capacity, hemodynamics, and quality of life for patients with CTEPH.

Balloon Pulmonary Angioplasty (BPA)

Balloon pulmonary angioplasty (BPA) is a minimally invasive procedure that uses a balloon catheter to open up blocked pulmonary arteries.

While BPA has been used for the treatment of pulmonary arterial hypertension, it is now increasingly being used for the treatment of CTEPH. BPA has been shown to improve exercise capacity, hemodynamics, and quality of life in patients with CTEPH. It may also be an alternative to PEA in patients who are not eligible for surgery.

Epoprostenol

Epoprostenol is a vasodilator that is used for the treatment of pulmonary arterial hypertension.

Although it has not been approved specifically for the treatment of CTEPH, studies have shown that it may be effective in improving hemodynamics and exercise capacity in patients with CTEPH. Epoprostenol is administered through a continuous intravenous infusion and may require a central venous catheter.

Macitentan

Macitentan is an endothelin receptor antagonist that has been approved by the Food and Drug Administration for the treatment of pulmonary arterial hypertension.

Although it has not been approved for the treatment of CTEPH, clinical studies have shown that macitentan may be effective in improving exercise capacity and pulmonary vascular resistance in patients with CTEPH. Macitentan is taken orally and is generally well-tolerated.

Catheter-Directed Thrombolysis

Catheter-directed thrombolysis is a minimally invasive procedure that involves the use of a catheter to deliver clot-busting medication directly to the blood clot in the pulmonary artery.

The procedure is typically reserved for patients who have undergone PEA but still have residual or recurrent clots. Studies have shown that catheter-directed thrombolysis can improve pulmonary hemodynamics and functional capacity in these patients.

Ventricular Assist Devices

Ventricular assist devices (VADs) are mechanical pumps that are used to assist the heart in pumping blood.

Although VADs have traditionally been used for the treatment of heart failure, they are now being used for the treatment of CTEPH in select patients who are not eligible for PEA or angioplasty. VADs can improve hemodynamics and functional capacity and may be used as a bridge to transplantation or as destination therapy.

Stem Cell Therapy

Stem cell therapy involves the use of stem cells to regenerate damaged tissues.

Although stem cell therapy has not been approved for the treatment of CTEPH, studies have shown that it may be effective in promoting pulmonary vascular regeneration and reducing pulmonary arterial hypertension in animal models of pulmonary hypertension. Stem cell therapy may offer a potential future treatment option for patients with CTEPH.

Talk to Your Doctor

When considering treatment options for CTEPH, it is important to consult with a healthcare professional who specializes in the treatment of pulmonary hypertension.

Each patient’s condition is unique, and treatment options will depend on the severity of the disease, the patient’s overall health, and other individual factors. New treatments for CTEPH continue to emerge, and it is important to stay informed about the latest advances in order to make informed decisions about treatment options.