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CTEPH Myths:
Learn common myths and realities about CTEPH and its treatment

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Myth: PEA Is Still Experimental

Richard Channick, MD, dispels the myth that pulmonary thromboendarterectomy (PTE, also known as PEA) is still an experimental technique in CTEPH.

  • Richard Channick, MD, dispels the myth that pulmonary thromboendarterectomy (PTE, also known as PEA) is still an experimental technique in CTEPH.

    Richard Channick, MD, dispels the myth that pulmonary thromboendarterectomy (PTE, also known as PEA) is still an experimental technique in CTEPH.
    Click to Play Video

  • Nick H. Kim, MD, dispels the myth that very ill patients cannot be considered for pulmonary thromboendarterectomy surgery, the only potentially curative treatment for CTEPH.

    Nick H. Kim, MD, dispels the myth that very ill patients cannot be considered for pulmonary thromboendarterectomy surgery, the only potentially curative treatment for CTEPH.
    Click to Play Video

  • Paul Forfia, MD, dispels the myth that the ventilation/perfusion scan is contraindicated in patients who have pulmonary hypertension, arguing that it is an essential screening tool in CTEPH diagnosis.

    Paul Forfia, MD, dispels the myth that the ventilation/perfusion scan is contraindicated in patients who have pulmonary hypertension, arguing that it is an essential screening tool in CTEPH diagnosis.
    Click to Play Video

  • William Auger, MD, dispels the myth that pulmonary thromboendarterectomy surgery has a high mortality rate, pointing out that experienced centers have been able to achieve mortality rates of <5%.

    William Auger, MD, dispels the myth that pulmonary thromboendarterectomy surgery has a high mortality rate, pointing out that experienced centers have been able to achieve mortality rates of <5%.
    Click to Play Video

  • Paul Forfia, MD, dispels the myth that if a patient’s pulmonary vascular resistance is greater than 1000 dyn∙s∙cm −5, he or she cannot be a candidate for pulmonary thromboendarterectomy (PTE, also known as PEA) surgery.

    Paul Forfia, MD, dispels the myth that if a patient’s pulmonary vascular resistance is greater than 1000 dyn∙s∙cm−5, he or she cannot be a candidate for pulmonary thromboendarterectomy (PTE, also known as PEA) surgery.
    Click to Play Video

  • Victor F. Tapson, MD, dispels the myth that PTE (also known as PEA) surgery can’t be done in obese patients, pointing out that obesity does not by itself disqualify a CTEPH patient as a candidate for PTE surgery.

    Victor F. Tapson, MD, dispels the myth that PTE (also known as PEA) surgery can’t be done in obese patients, pointing out that obesity does not by itself disqualify a CTEPH patient as a candidate for PTE surgery.
    Click to Play Video

  • Ivan Robbins, MD, dispels the myth that a ventilation/perfusion scan showing perfusion defects is sufficient to diagnose CTEPH. Definitive diagnosis requires RHC and pulmonary angiography.

    Ivan Robbins, MD, dispels the myth that a ventilation/perfusion scan showing perfusion defects is sufficient to diagnose CTEPH. Definitive diagnosis requires RHC and pulmonary angiography.
    Click to Play Video

  • Richard Channick, MD, dispels the myth that medical therapy can be used to delay or replace PTE surgery, urging that CTEPH patients with operable disease be referred for PTE surgery without delay.

    Richard Channick, MD, dispels the myth that medical therapy can be used to delay or replace PTE surgery, urging that CTEPH patients with operable disease be referred for PTE surgery without delay.
    Click to Play Video

  • William Auger, MD, dispels the myth that pulmonary thromboendarterectomy (PTE, also known as PEA) cannot be performed in older patients.

    William Auger, MD, dispels the myth that pulmonary thromboendarterectomy (PTE, also known as PEA) cannot be performed in older patients.
    Click to Play Video

  • Victor F. Tapson, MD, dispels the myth that a patient who recovers from an acute pulmonary embolism cannot go on to develop CTEPH.

    Victor F. Tapson, MD, dispels the myth that a patient who recovers from an acute pulmonary embolism cannot go on to develop CTEPH.
    Click to Play Video

  • Nick H. Kim, MD, dispels the myth that computerized tomographic pulmonary angiography has become so sophisticated that it can be used as a screen instead of a V/Q scan to rule out CTEPH.

    Nick H. Kim, MD, dispels the myth that computerized tomographic pulmonary angiography has become so sophisticated that it can be used as a screen instead of a V/Q scan to rule out CTEPH.
    Click to Play Video

  • Ivan Robbins, MD, dispels the myth that once PH is confirmed by RHC, there is no need to perform a V/Q scan, arguing that a V/Q scan remains essential to screen for CTEPH.

    Ivan Robbins, MD, dispels the myth that once PH is confirmed by RHC, there is no need to perform a V/Q scan, arguing that a V/Q scan remains essential to screen for CTEPH.
    Click to Play Video

  • Myth: PEA Is Still Experimental

    Richard Channick, MD, dispels the myth that pulmonary thromboendarterectomy (PTE, also known as PEA) is still an experimental technique in CTEPH.
    Click to Play Video

  • Myth: Very Ill Patients Cannot Be Considered for PEA Surgery

    Nick H. Kim, MD, dispels the myth that very ill patients cannot be considered for pulmonary thromboendarterectomy surgery, the only potentially curative treatment for CTEPH.
    Click to Play Video

  • Myth: A V/Q Scan is Contraindicated in Patients with PH

    Paul Forfia, MD, dispels the myth that the ventilation/perfusion scan is contraindicated in patients who have pulmonary hypertension, arguing that it is an essential screening tool in CTEPH diagnosis.
    Click to Play Video

  • Myth: PEA Surgery Has a High Mortality Rate

    William Auger, MD, dispels the myth that pulmonary thromboendarterectomy surgery has a high mortality rate, pointing out that experienced centers have been able to achieve mortality rates of <5%.
    Click to Play Video

  • Myth: If PVR Is Too High, You Can’t Do PEA

    Paul Forfia, MD, dispels the myth that if a patient’s pulmonary vascular resistance is greater than 1000 dyn∙s∙cm−5, he or she cannot be a candidate for pulmonary thromboendarterectomy (PTE, also known as PEA) surgery.
    Click to Play Video

  • Myth: PEA Surgery Can’t Be Done in Obese Patients

    Victor F. Tapson, MD, dispels the myth that PTE (also known as PEA) surgery can’t be done in obese patients, pointing out that obesity does not by itself disqualify a CTEPH patient as a candidate for PTE surgery.
    Click to Play Video

  • Myth: A V/Q Scan Is Enough to Make a Definitive Diagnosis

    Ivan Robbins, MD, dispels the myth that a ventilation/perfusion scan showing perfusion defects is sufficient to diagnose CTEPH. Definitive diagnosis requires RHC and pulmonary angiography.
    Click to Play Video

  • Myth: I Can Use Medical Therapy to Delay or Replace PEA Surgery

    Richard Channick, MD, dispels the myth that medical therapy can be used to delay or replace PTE surgery, urging that CTEPH patients with operable disease be referred for PTE surgery without delay.
    Click to Play Video

  • Myth: PEA Cannot Be Performed in Older Patients

    William Auger, MD, dispels the myth that pulmonary thromboendarterectomy (PTE, also known as PEA) cannot be performed in older patients.
    Click to Play Video

  • Myth: Recovery From Acute PE Excludes CTEPH

    Victor F. Tapson, MD, dispels the myth that a patient who recovers from an acute pulmonary embolism cannot go on to develop CTEPH.
    Click to Play Video

  • Myth: CT Pulmonary Angiography Can Be Used to Rule Out CTEPH

    Nick H. Kim, MD, dispels the myth that computerized tomographic pulmonary angiography has become so sophisticated that it can be used as a screen instead of a V/Q scan to rule out CTEPH.
    Click to Play Video

  • Myth: If Signs Point to PH, There Is No Need for a V/Q Scan

    Ivan Robbins, MD, dispels the myth that once PH is confirmed by RHC, there is no need to perform a V/Q scan, arguing that a V/Q scan remains essential to screen for CTEPH.
    Click to Play Video

Hi, I'm Richard Channick, Director of the Pulmonary Hypertension and Thromboendarterectomy Program at Massachusetts General Hospital in Boston, Massachusetts. I want to thank you for visiting CTEPH.com.

In this video, I'll be discussing pulmonary thromboendarterectomy--which is commonly known as PEA or PTE--the only potentially curative treatment for CTEPH.

To begin, let's be clear what we mean when we say CTEPH. Chronic Thromboembolic Pulmonary Hypertension, or CTEPH, is defined as mean pulmonary arterial pressure of at least twenty-five millimeters of mercury and pulmonary capillary wedge pressure of no more than fifteen millimeters of mercury in the presence of multiple, chronic, or organized occlusive thrombi or emboli in the elastic pulmonary arteries (which are the main, lobar, segmental, or subsegmental arteries) after at least three months of effective anticoagulation.

Screening and diagnosis are covered in another of CTEPH.com's video, but it's worth mentioning here that the preferred and recommended method to screen PH patients for the presence of CTEPH is the ventilation/perfusion scan (or V/Q scan). The V/Q scan is highly sensitive for CTEPH, and a negative V/Q scan effectively rules out the presence of CTEPH.

CTEPH is unique among the five groups of pulmonary hypertension in that it is potentially curable--but only with pulmonary thromboendarterectomy. Because there is a potential cure for CTEPH, every patient who is diagnosed with CTEPH should be assessed for operability. Patients who are viable candidates for surgery should be referred for PEA without any delay. There is insufficient evidence to support trying medical therapy first. Patients who undergo PEA surgery should understand they will need to receive lifelong anticoagulation unless contraindicated.

I want to now say a few words about how the operability assessment is performed. Pulmonary angiography is still considered the gold standard modality for confirmation of CTEPH and evaluation of operability, and computerized tomography or CT scanning with pulmonary angiography can provide additional information about the lesions as a complement to PA gram.

Operability assessment is a subjective exercise, and should be performed by an experienced CTEPH team.

Now there's no definitive way to predict who is most likely to benefit from PEA surgery. Studies suggest, though, that concordance or agreement between preoperative pulmonary vascular resistance, also known as PVR, and anatomic disease, and having a preoperative PVR less than about 1,000 to 1,200 dyn•s•cm-5 have been associated with better outcomes.

PEA surgery is a complex process: It requires a median sternotomy to approach both lungs and cardiopulmonary bypass. It involves cooling the patient to about 20°C, leading to short-term circulatory arrest and removing the hardened thromboembolic material during those periods of circulatory arrest.

This surgical technique has been proven at centers around the world. It allows for potentially complete endarterectomy, and does not cause significant impairment of cognitive function in the vast majority of patients.

In fact, most patients who undergo a PEA will experience lasting symptomatic and hemodynamic improvements.

To support the safety of the procedure, I want to show you some data from 4 studies. The green table shows mortality rates reported by 27 centers stratified by the number of PEAs performed each year, a measure of surgical experience. As you can see, even in centers that perform as few as one to ten PEA surgeries, almost 93% of patients survive to discharge. Not surprisingly, as experience goes up, rates of in-hospital deaths and deaths within one year of the procedure decrease.

The blue table documents the PEA history of a single center, the University of California at San Diego. UCSD began doing PEAs in the 1970s, and since then, mortality rates have dropped sharply, such that none of their last 260 PEAs as reported in this study resulted in death.

The red table makes the point that even in patients of advanced age--those who are seventy or more years old--PEA surgery can be safe, with no statistically significant difference in mortality between those 70 and over versus those younger than 70.

Survival rates are only one outcome--albeit a very important one. It's well established that successful PEA surgery improves other outcomes, too. From the same registry that provided the data on improvement of mortality rates with experience, we can see that in addition to a one-year mortality rate of only seven percent, surviving patients had substantially decreased median PVR, substantially increased six-minute walking distance, and improvement in WHO functional class from having marked or severe physical limitation to having mild or no physical limitation.

A long-term study published in early 2014 supports these findings. Here, 110 patients--mean age 56 (plus or minus fourteen years)--who had a PEA between 1994 and 2010 were followed, in many cases for more than 10 years. On average, PVR decreased from a preoperative level of 770 dynes down to 369 dynes immediately following PEA surgery. One year later, PVR had fallen further to only 280 dynes.

As reported in this study, one-year survival is comparable to the European registry, but importantly, this study shows that at 5 years, 85% of patients were still alive, and at 10 years after PEA surgery, 61% of patients were still alive.

I'll close with a few key takeaways. First, every patient who has CTEPH must be evaluated as a potential candidate for PEA surgery. Though PEA surgeries are complex, there are about 300 performed each year in the US, and the procedure is proven.

Because PEA surgery is potentially curative, it should not be delayed in viable candidates.

And last, know that there is a growing number of expert PEA centers geographically spread throughout the US. You can access a list of PEA expert centers by visiting PHA Association dot org.

I sincerely thank you for visiting CTEPH.com and watching this educational video. Please bookmark this site and check back frequently, as we'll continue to update CTEPH.com with new content.