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Diagnosing and Treating CTEPH

What to Expect

The diagnosis of CTEPH involves using a lung ventilation-perfusion scan and an echocardiogram (an ultrasound of the heart) to look for the disease. If these tests are normal, then CTEPH is ruled out. However, if they are abnormal, more tests may be done to confirm the diagnosis and locate the blood clots.

How Is CTEPH Diagnosed?

A ventilation-perfusion (V/Q) scan is the next test often used to diagnose CTEPH. In this test, a radioactive protein is injected into your bloodstream to trace how blood circulates through the lungs. Blockages to blood flow in the lungs may suggest the presence of blood clots in the lungs.

To establish the diagnosis of chronic blood clots, and to accurately locate the clots, a pulmonary angiogram, CT angiography or magnetic resonance imaging (MRI) of the pulmonary arteries is necessary. For each study, contrast is injected into the pulmonary arteries so that blockages in these vessels can be identified. A physician familiar with CTEPH often in conjunction with a surgeon experienced in the procedure determines if these clots are in locations that allow for surgical removal. Ideally, patients should be evaluated by medical centers that specialize in the care and management of patients with pulmonary hypertension, and in particular, CTEPH.

Signs of strain on the heart caused by pulmonary hypertension may be measured using an ultrasound called an echocardiogram and a right heart catheterization.

How Is CTEPH Treated?

The definitive treatment for CTEPH is a delicate surgery called a pulmonary thromboendarterectomy (PTE), also referred to as a pulmonary endarterectomy. This surgery is done while the heart is stopped, and a heart-lung bypass machine does the work of the heart and the lungs. The surgeon carefully removes the clots from the pulmonary arteries. This surgery if highly specialized, and should be done by medical centers with CTEPH expertise. PTE surgery is curative in over 90 percent of patients.

For patients who are not good candidates for PTE, either due to chronic clots that are not easily accessible to the surgeon or who have other medical conditions which make PTE too risky, there are other options. A newer procedure called balloon pulmonary angioplasty uses a tiny balloon which is inflated inside the pulmonary artery to reduce the narrowing, and open the vessel. This procedure can reduce pulmonary artery pressure and help relieve right heart strain. There are also medications for inoperable CTEPH or residual pulmonary hypertension after PTE surgery.

    This content was developed in partnership with the CHEST Foundation, the philanthropic arm of the American College of Chest Physicians.

    Reviewed and approved by the American Lung Association Scientific and Medical Editorial Review Panel. Last reviewed December 13, 2016.

    Page Last Updated: March 13, 2018

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