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

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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.

  • Paul Forfia, MD, dispels the myth that if a patients pulmonary vascular resistance is greater than 1000 dynscm -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 patients pulmonary vascular resistance is greater than 1000 dynscm-5, he or she cannot be a candidate for pulmonary thromboendarterectomy (PTE, also known as PEA) surgery.
    Click to Play Video

  • 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

  • Victor F. Tapson, MD, dispels the myth that PTE (also known as PEA) surgery cant 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 cant 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 Dr Paul Forfia. I'm an associate professor of medicine and the director of the pulmonary vascular disease, right heart failure, and pulmonary thromboendarterectomy program at Temple University Hospital in Philadelphia.

I'd like to welcome you today to CTEPH.com. In this video, we're going to discuss how venous thromboembolism can be a potential precursor to CTEPH.

But first, a quick reminder of what we're talking about when we talk about CTEPH. CTEPH, or Chronic Thromboembolic Pulmonary Hypertension, is defined by a mean pulmonary arterial pressure of at least twenty-five millimeters of mercury and a 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 (such as the main, lobar, segmental, and subsegmental arteries) after at least three months of effective anticoagulation.

Acute pulmonary embolism is associated with the development of CTEPH. And we know that almost all cases of acute PE originate from thrombi in the legs or pelvis, that is, from deep venous thrombosis. Often the first manifestation of a DVT or an acute PE is death: In 2010 in the US, there were over forty-two thousand deaths associated with venous thromboembolism. And although the true incidence of CTEPH in the US is unknown, among survivors of acute PE, CTEPH can be a serious complication.

There are several risk factors that contribute to venous thromboembolism. I don't want to read them all off of this slide, but I do want to call your attention to some of the acquired risk factors. Together, the acquired risk factors in the middle column here account for over 75% of all incident VTEs that occur in the community. If we look at those circled in red, you'll see some very common characteristics, characteristics we see in our patients every day. One other thing to recall about VTE is that it can be chronic with episodic recurrence: about 30% of patients with VTE have a recurrence within 10 years.

So how do we go from a deep venous thrombosis to CTEPH? According to the "embolic hypothesis," proposed by Marc Humbert, a single or recurrent PE arises from a DVT as the initiating event. The acute PE fails to resolve completely and may organize in the pulmonary artery, blocking blood flow, which in turn elevates pulmonary arterial pressure, resulting in a progressive increase in the pulmonary vascular resistance. This pulmonary hypertensive state, in the presence of a chronic occlusive thromboembolic condition, is CTEPH.

Knowing how a DVT can lead to a PE and how a PE can lead to CTEPH, we're led to ask the question of how big a problem this is. Estimates of the proportion of patients who have acute PE that develops into CTEPH vary, but among the best data available were reported about 10 years ago by Pengo and colleagues.

In a prospective study of 314 patients with acute PE, 3.8% of patients developed CTEPH within 2 years of their first PE. Conversely, after 2 years post-PE, no additional cases of CTEPH emerged as a product of those acute PE. So that 2-year window after an acute PE would appear to be the most likely period during which about 4 out of every 100 acute PE patients will go on to develop CTEPH.

The next obvious question is, "Are there ways to predict which acute PE patients will go on to develop CTEPH?" The answer is not really, but there are risk factors that contribute to the development of CTEPH after acute PE, and those are shown here: Recurrent, unprovoked, or idiopathic PE; large perfusion defects evident when the PE was first detected; young age; high--that is, greater than fifty millimeters of mercury--pulmonary artery systolic pressure; and persistent pulmonary hypertension six months after an acute PE.

As we consider the relationship between DVT, acute PE, and CTEPH, we don't want to overstate its predominance. Not all patients who have CTEPH have a history of acute PE. There is no clear consensus about how many CTEPH patients there are, the most recent data available from a European CTEPH registry suggests that at least one quarter of CTEPH patients have no such history.

I mention this to make the point that the possibility of CTEPH in a patient with symptoms and CTEPH risk factors should not be summarily dismissed simply because of the absence of a history of PE.

I'll close by reviewing guidance from the ACCF and the AHA made in a joint statement on pulmonary hypertension. Alhough CTEPH is rare, we can expect 3 to 4 patients out of every 100 who have had an acute PE to develop CTEPH. Data suggest that the window in which this is most likely to occur is 2 years following the first acute PE.

For patients who show the risk factors I outlined and symptoms of pulmonary hypertension, screening for the presence of CTEPH is warranted. The recommended test for CTEPH screening is the ventilation/perfusion scan, and a V/Q scan should be ordered for any acute PE patient who displays symptoms of pulmonary hypertension three months after the event.

A negative V/Q scan excludes chronic thromboembolism. If your patient has a positive V/Q scan suggestive of CTEPH, order a pulmonary angiogram to help confirm the diagnosis.

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.