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Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Program

Lifesaving, Cutting-Edge Care for CTEPH

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a rare, but debilitating and disabling, disease that is often missed or undertreated. Characterized by high blood pressure in your lungs’ arteries, CTEPH is caused by blood clots and can happen to anyone. Chronic thromboembolic pulmonary hypertension can be cured, and Rochester Regional Health is home to the only medical center in Upstate and Western New York that provides comprehensive evaluation and treatment of CTEPH. Schedule a consultation today.

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What is CTEPH?

CTEPH is commonly described as high blood pressure in the arteries in your lungs due to blockages in those arteries. The right-sided chamber of your heart exerts pressure to move blood through your lungs, and high blood pressure in your lungs can cause enlargement of your heart chamber and heart failure. Many people get CTEPH after a pulmonary embolism (PE), which is a blood clot in your lung.

Each year in the United States, there are about 5,000 cases, and about 1 - 5% of people who have had a PE will get CTEPH.

CTEPH Risk Factors

The risk factors for CTEPH include:

  • A blood clotting disorder
  • History of cancer
  • History of clots in your veins
  • Large blood clots obstructing lung circulation
  • Obesity and/or inactive lifestyle
  • Repeated episodes of clots in your pulmonary vessels
  • Surgically removed spleen

Some risk factors can be combatted by maintaining a healthy weight and avoiding smoking. If you are on prescribed blood thinners, take them as directed. And, if you notice fatigue or shortness of breath that is increasing, please talk with your primary care physician.

CTEPH Symptoms

Diagnosing CTEPH can be difficult because the symptoms resemble those of other diseases. Often, the symptoms appear slowly, and may include:

  • Bluish fingers or toes (called cyanosis)
  • Decreased appetite and/or bloated belly
  • Fainting
  • Fatigue
  • Pain or pounding in your chest
  • Shortness of breath, especially during exercise
  • Swelling in your legs (called edema)
  • Weakness

In some rare cases, CTEPH can cause you to cough up blood. If you are experiencing any of the symptoms above, please schedule a consultation with our chronic thromboembolic pulmonary hypertension experts.

CTEPH Diagnosis and Treatment

  • Ventilation-Perfusion Scan (V/Q Scan)

    A V/Q scan is a non-invasive test that involves an intravenous injectable and an inhaled radiotracer. This test is nearly 100% accurate in identifying the presence or absence of an obstructive clot in your lungs.

  • Computer Tomography Angiography (CT)

    CTs are non-invasive imaging tests used to see the severity of your disease. Our radiologists will reconstruct a three-dimensional picture of your pulmonic circulation to better see the location and size of your clot.

  • Right-Heart Catherization and Pulmonary Angiography

    This invasive imaging test is performed by our skilled interventional cardiologists and pulmonary hypertension experts in a cardiac catheterization laboratory. During this procedure, a very delicate catheter will be delivered to your main pulmonary vessel. We use this test to evaluate the severity of your disease and determine how effective or feasible a surgical approach may be.

Treating CTEPH

Before your treatment route is decided, you will meet with a cardiologist that specializes in pulmonary hypertension. During this visit, you will learn more about your disease, and any diagnostic procedures you may receive and have the opportunity to ask questions or voice concerns.

We understand you have a busy life and will consolidate your tests and schedule them in one day to limit your trips to Rochester General Hospital.

Surgery

If our experts decide that surgery is right for you, you will meet with our CTEPH surgeon. Our team will help you find a surgery day that works for you, but you can expect a hospital stay of 7-14 days. Most likely, you’ll be discharged to cardiac rehabilitation to get stronger and recover from surgery before you head home.

Long-term, your care will be co-managed by your local primary cardiologist or pulmonologist and our pulmonary hypertension experts.

Pulmonary Thromboendarterectomy (PTE) Surgery

PTE involves your surgeon removing the blockages in your pulmonary arteries (in the left and right lungs) using specialized tools. This is the gold standard and most effective treatment for many patients with CTEPH and is also used to treat patients with chronic pulmonary embolism.

During this surgery, your surgeon will utilize a heart-lung machine and circulatory arrest where the function of your heart and lungs are taken over by a machine. This highly specialized procedure is performed by a team of experts including a cardiothoracic surgeon, cardiac anesthesiologists, and perfusionists.

There are three major reasons for PTE surgery:

  1. The hemodynamic goal: to prevent or improve right ventricular compromise caused by pulmonary hypertension
  2. Respiratory goal: to make breathing and oxygenation of the blood more efficient
  3. Prophylactic goal: to prevent progressive right ventricular dysfunction, a retrograde extension of the clot, and secondary changes in the remaining vessels

PTE will not only eradicate blockages in the pulmonary arteries, but it will also reduce or resolve pulmonary hypertension, improve functional status, relieve symptoms, prevent worsening heart failure, and improve your survival.

Alternative Treatment Options

If it's determined that PTE surgery is not right for you, there are other treatment options available. Our CTEPH team will discuss and explore these non-surgical options with you.

Percutaneous Balloon Pulmonary Angioplasty

This procedure begins with a small puncture and uses catheters with small balloons at the end to break the scars in your arteries. It is typically done more than once and may be done in someone who has already undergone PTE surgery.

Percutaneous balloon pulmonary angioplasty has been shown to improve breathing and blood flow.

Double Lung Transplant

Lung transplantation may be an option if you are not a candidate for PTE surgery or balloon angioplasty. This procedure is a surgical option that replaces your lungs with the donor’s lungs.

Medical Management

While there are very few medications that have demonstrated benefits for patients with CTEPH who cannot have surgery, your provider will use your health and goals to explore medication options.

Why Rochester Regional Health?

CTEPH is curable with the help of PTE, a unique and complex open-heart surgery. Very few hospitals nationwide offer lifesaving surgery like PTE, but Rochester General Hospital has state-of-the-art facilities equipped with the latest and greatest in modern technology. But, most importantly, we have skilled professionals who have dedicated their lives to the field. We work as a team to discuss each patient at a multidisciplinary conference to ensure we’re giving the best possible care.

Our highly trained and experienced physicians, physician assistants, nurse practitioners, and nurses will take the time to answer all your questions and address your concerns. Every step of the way, we’ll walk you through diagnostic procedures, lay out your treatment options, and help you choose the strategy that best fits you as an individual and unique patient.

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CTEPH Care

You can receive exemplary CTEPH care at Rochester General Hospital in Rochester, NY. Accessible and centrally located, it is equipped with cutting-edge technology and is home to experienced specialists of all kinds.

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Schedule a CTEPH Consultation

While disabling and debilitating, chronic thromboembolic pulmonary hypertension is curable. If you are experiencing any of the symptoms above or are at a higher risk for CTEPH, please call (585) 442-5320 or contact us via the form below.
Contact the CTEPH Team