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Our specialists are experts at diagnosing, evaluating, and managing chronic thromboembolic pulmonary hypertension (CTEPH), in which fibrotic scar tissue forms on the inside of a lung artery after pulmonary embolism. We are one of the only centers in the United States to offer pulmonary thromboendarterectomy. This surgical procedure, the gold standard for treatment, is highly effective at stopping its progression.

Diagnosing and Evaluating CTEPH

Upon referral to our program and before your first appointment, the CTEPH team reviews all your medical records and tests. Our specialists may order a variety of additional tests to diagnose and evaluate the condition. This helps us create a treatment plan and determine whether you are a candidate for pulmonary thromboendarterectomy. To do this, you may be given tests such as the following:

  • a CT scan, or computed tomography, performed with a special protocol and reviewed by radiologists to identify scar tissue inside of the arteries in the lungs
  • a ventilation and perfusion (V/Q) scan, a nuclear medicine imaging test that identifies problems between the air and blood flow in the lungs
  • echocardiography, a noninvasive ultrasound that provides information about the structure and function of the heart and pulmonary artery pressure
  • a right heart catheter study, in which a catheter is inserted into a vein in the neck or groin to measure the pressures in the chambers of the heart and the pulmonary artery; this is the most accurate way to confirm a CTEPH diagnosis

If you are a candidate for surgery, your team may recommend more tests, such as blood tests, pulmonary or coronary angiograms, an X-ray of the arteries in the lungs or heart taken with contrast dye, ultrasounds of the veins and arteries, lung function tests, and exercise tests.

Advanced Treatment Options for CTEPH

CTEPH is a manageable condition, and there are treatments that can potentially stop its progression or even resolve it.

Pulmonary Thromboendarterectomy

Pulmonary thromboendarterectomy is a highly effective treatment for CTEPH. For this procedure, the surgeon makes an incision through the sternum, or breastbone, and then places the patient on a heart–lung bypass machine and systematically cools the body to preserve organ function. The surgeon opens the affected pulmonary artery and carefully removes the scar tissue from the inner layer of the blood vessel. Both lungs are explored, and all the major blood vessels are cleaned of scar tissue. Briefly stopping the heart–lung machine reduces bleeding and allows for the most accurate removal of scar tissue.

After the procedure, patients recover in the intensive care unit for several days and for 10 to 14 days in the hospital. Your care team may recommend spending some time at the pulmonary or cardiac rehabilitation program at Rusk Rehabilitation to build up your strength and stamina before going home.

Balloon Pulmonary Angioplasty

Our interventional cardiologists at NYU Langone Heart may manage CTEPH with a balloon pulmonary angioplasty, which is less invasive than surgery. For this procedure, a doctor threads a catheter into a lung artery and inflates a balloon to open the blood vessel, improving blood flow. This approach may be recommended for people who are not candidates for surgery.

Pulmonary Hypertension Medication

Another option for managing CTEPH is to prescribe pulmonary hypertension medications to reduce the pressure inside the pulmonary arteries and to improve the function of the right side of the heart. People who cannot have surgery or an angioplasty or people who have residual pulmonary hypertension after these interventions may benefit from taking these medications.