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Short of Breath with a History of Pulmonary Embolism?

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Why You May Need Treatment From Specialized CTEPH Experts

Posted by Anjali Vaidya, MD, FACC, FASE, FACP

Shortness of breath can be a symptom of many conditions that affect the heart or lungs. But, it’s an especially alarming symptom for my patients who previously had a blood clot in their lungs, called a pulmonary embolism (PE). For these patients, shortness of breath can be a symptom of a serious condition called chronic thromboembolic pulmonary hypertension, or CTEPH for short. Without treatment, CTEPH can lead to heart failure and even death.

Fortunately, CTEPH can often be cured with specialized surgery, which is available only at a few medical centers in the entire U.S. The Temple Heart & Vascular Institute in Philadelphia is one of the nation’s leaders.

I recommend that patients who have had a pulmonary embolism and who now experience shortness of breath be evaluated immediately at a Pulmonary Hypertension Association Center of Comprehensive Care for CTEPH, such as the Temple Heart & Vascular Institute.

What causes CTEPH?

CTEPH can occur after a patient has had a pulmonary embolism. It can also develop when a patient has no known history of clotting problems.

As I’ve explained to my patients, the body often dissolves blood clots on its own, but for many people, these clots don’t go away completely. They stay in the lungs and lead to reactions in the blood vessels of the lungs, causing scar tissue to form that grows and lines the blood vessels. This is called chronic clot.

A chronic clot causes pressure to build up in the arteries of the lungs, which is a form of pulmonary hypertension. This puts a great deal of strain on the right side of the heart. It makes it harder for people to breathe and to get oxygen out of the air.

What are the symptoms of CTEPH?

Some people with CTEPH have mild symptoms, while others experience severe shortness of breath that limits their quality of life. I’ve had patients with CTEPH who are not able to walk a few feet across the room.

Other symptoms of CTEPH can include:

  • Lightheadedness
  • Fatigue
  • Chest pain
  • Racing pulse
  • Fluid build-up with the swelling of the legs or abdomen

It can be a devastating and even fatal condition.

Why is CTEPH so hard to diagnose?

CTEPH can be a difficult condition for some physicians to diagnose, especially if they don’t specialize in the diagnosis and management of all forms of pulmonary hypertension.

Awareness of CTEPH isn’t what it should be, even in the medical community. It was once thought that CTEPH was fairly rare. Now, the medical community knows more about CTEPH and realizes that the condition isn’t that rare — it just hasn’t been diagnosed correctly.

Cardiopulmonary (heart-lung) specialists use a variety of tools to ensure that we get a complete assessment. Most medical centers, even academic ones, don’t have the same expertise for diagnosing CTEPH. At the Temple Heart & Vascular Institute, we have a specialized team solely dedicated to the diagnosis and treatment of this condition.

CTEPH testing can include:

  • Echocardiogram — an ultrasound of the heart that estimates pulmonary pressure and shows any abnormalities in the right side of the heart
  • Right-heart catheterization — provides a direct measurement of pressures and flows in the heart and lungs
  • Ventilation-perfusion lung scan — provides a picture of air and blood circulation in the lungs, and can also reveal areas of the lung not receiving blood flow due to a clot
  • CT scan — can show blood vessels throughout the lungs and identify evidence of chronic clots
  • Pulmonary angiogram — can pinpoint the location of a clot and help the medical team plan for treatment

No matter what specific tests are used in the diagnostic process, an expert, multidisciplinary team is needed. Many patients have other, complicated medical conditions, so it’s important to have all their specialists involved in the care plan that’s developed.

What are my treatment options?

CTEPH can often be cured with surgery. Pulmonary thromboendarterectomy (PTE) involves removing the chronic clot from the lung. This is a highly specialized procedure that’s more involved than removing an acute pulmonary embolism.

Temple is home to the largest PTE program on the East Coast, completing more than 300 PTE surgeries since the program’s inception in 2013 — second most in the U.S. over that time period.

Since January 2020, Temple has also had a 1.5% mortality rate for patients who undergo PTE, which is among the best outcomes in the nation. This is a remarkable feat as the program cares for both a high volume of patients, and those with complex cases, from all over the country.

Advanced treatment for CTEPH
Learn more about this underdiagnosed condition and PTE surgery — an advanced treatment for CTEPH.
Learn more about this underdiagnosed condition and PTE surgery — an advanced treatment for CTEPH.

Surgery, however, is not an option for all patients. It’s best for people who:

  • Have clots that the surgeon can easily access in large and medium-sized pulmonary arteries
  • Don’t have other medical conditions that increase their surgical risk

Patients who do not qualify for surgery may benefit from medications or balloon pulmonary angioplasty (BPA) to open up the areas of blocked pulmonary arteries. Some patients receive all 3 treatments, but it depends on the individual case. We offer all of these therapies at Temple.

Expert CTEPH care

The Temple Heart & Vascular Institute is one of the few programs in the country to have a team of physicians and nurses dedicated to CTEPH. The Pulmonary Hypertension Association has accredited the Temple Heart & Vascular Institute as a Center of Comprehensive Care for CTEPH. It’s a designation that we’re incredibly proud of.

Get the immediate help you need

The Temple Cardiology CTEPH team is available for immediate evaluations and second opinions. To schedule a virtual or in-office visit with one of our CTEPH specialists, call 800-TEMPLE-MED (800-836-7536) or request an appointment.

Helpful Resources

Looking for more information?

Anjali Vaidya, MD, FACC, FASE, FACP

Dr. Vaidya is nationally respected for her expertise in all forms of pulmonary hypertension and right heart failure, including chronic thromboembolic pulmonary hypertension (CTEPH). She is highly skilled in the use of echocardiography to assess, monitor, and offer individually tailored therapy to patients with advanced forms of pulmonary hypertension. Dr. Vaidya is also Co-Director of Temple's Pulmonary Hypertension, Right Heart Failure and CTEPH Program, and Associate Professor of Medicine at the Lewis Katz School of Medicine at Temple University.

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