Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

A condition where blood clots block blood flow in the arteries of the lungs, causing a buildup of pressure in the arteries.

CT Pulmonary Angiography (CTPA) is a type of scan that uses X-rays to create detailed images of the arteries in the lungs.

Digital Subtraction Angiography (DSA) is a type of imaging test that involves injecting dye into the arteries and taking X-rays to visualize the blood vessels.

A narrowing or retraction of the arteries in the lungs is a sign of CTEPH.

Very small blood clots in the peripheral arteries of the lungs are difficult to see on CTPA.

CTEPH is a condition where blood clots form in the pulmonary arteries, leading to high blood pressure in the lungs.

PVR stands for Pulmonary Vascular Resistance. It measures the resistance blood faces when flowing through the lungs.

Patients with similar severity of CTEPH on imaging can have different blood pressures in their lungs.

CTEPH without high lung blood pressure is not necessarily an early stage of the disease.

The clots in CTEPH can occur in different locations, either close to the main arteries or in smaller branches.

CTEPH can involve damage to the small blood vessels in the lungs, beyond the main blockages.

Lobar arteries are the larger arteries in the lungs, while subsegmental arteries are smaller branches.

Different imaging techniques, like ultrasound and optical coherence tomography, are used to visualize the clots and damaged vessels.

Advances in treating CTEPH have significantly improved survival rates. Over 90% of patients, both operable and inoperable, are now surviving for 3 years.

A multidisciplinary approach targets all aspects of CTEPH, including the underlying blood clots, high blood pressure, and right ventricular dysfunction.

Systolic pulmonary arterial pressure (sPAP) exceeding 60 mm Hg, along with right ventricular dysfunction or hypertrophy, are strong indicators of CTEPH.

Factors like antiphospholipid syndrome, certain cancers, splenectomy, implanted devices, inflammatory bowel disease, and thyroid hormone replacement therapy can increase the risk of CTEPH.

Despite the effectiveness of treatment, some patients are hesitant to seek specialized care due to the potential for invasive procedures, travel distance, or personal reluctance.

This paper summarizes the successful treatment approaches for CTEPH, aiming to encourage patients to seek timely and comprehensive care.

By highlighting the positive outcomes of multidisciplinary treatment, this paper seeks to address reluctance and encourage patients to seek specialized care for CTEPH.

A type of pulmonary hypertension where blood flow is restricted in the small blood vessels in both obstructed and non-obstructed areas of the lungs.

A technique during cardiac catheterization where the pulmonary artery is temporarily blocked to assess blood flow and pressure in the lungs.

The degree of resistance to blood flow in the pulmonary arteries.

A condition where blood clots form in the lungs, often after a deep vein thrombosis (DVT).

A rare condition in which the small blood vessels in the lungs become narrow and thickened, leading to high blood pressure in the lungs.

The process of change in the structure and function of pulmonary arteries, often leading to narrowing and thickening.

The difference between the pressure measured in the pulmonary artery and the pressure in capillaries.

A measurement of the force of blood against the walls of the arteries, specifically in the lungs.

When blood flow is blocked in the lungs, the body tries to compensate by creating new pathways for blood to reach the lungs. This is called systemic collateral circulation.

Microvasculopathy in CTEPH is caused by high blood flow through the newly formed pathways and high pressure in the pulmonary arteries, damaging the tiny blood vessels.

The microvasculopathy in CTEPH is explained by the shift of blood flow from the blocked areas to the new pathways, leading to high flow and pressure.

While imaging helps diagnose CTEPH, it cannot directly show the tiny vessel damage. However, measuring blood pressure in the lungs (mPAP), resistance (PVR), and other indicators can suggest microvasculopathy.

The newly created blood pathways in CTEPH can be responsible for coughing up blood (hemoptysis).

These new blood pathways are important for keeping the lung tissue healthy by preventing tissue death or infarction.

A procedure that widens narrowed pulmonary artery branches using balloons, improving blood flow to the lungs.

The pressure in the pulmonary arteries is measured in Wood units. A PVR greater than 320 dyn·s·cm–⁵ (4 Wood units) suggests the need for BPA.

A staged approach is preferred, treating a limited number of pulmonary segments per session to minimize the risk of damaging healthy lung tissue.

The most common complication is pulmonary haemorrhage, which can range from minor bleeding to severe lung injury. Even with experience, it's a risk.

The goal of BPA is to treat all accessible narrowed or blocked areas in the pulmonary arteries to improve blood flow.