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

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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.

  • 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

  • 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

  • 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 Nick Kim. I’m an Associate Clinical Professor of Medicine and Director of Pulmonary Vascular Medicine at the University of California, San Diego.

Chronic Thromboembolic Pulmonary Hypertension, or CTEPH, is defined as mean pulmonary artery 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 (the main, lobar, segmental, subsegmental vessels) after at least three months of effective anticoagulation.

CTEPH is classified by the World Health Organization as Group Four PH.

The other WHO Groups of PH include, Group one, pulmonary arterial hypertension; Group two, which is PH owing to left heart disease; Group three, PH stemming from lung diseases and/or hypoxia; and Group five, which is PH with unclear or multifactorial mechanisms.

By any measure, CTEPH is a rare disease, with an estimated 500 to 2,500 new diagnoses in the United States each year. But CTEPH may be more prevalent than previously thought.

There is an apparent link between acute pulmonary embolism and the development of CTEPH. A European registry that published results in 2011 demonstrated that almost three quarters of CTEPH patients presented with previous acute PE. Conversely, observational studies suggest that as many as zero point five-seven percent to three point eight percent of patients may go on to develop CTEPH within two years following a first acute PE.

Each year, in the US, about 600,000 persons have an acute pulmonary embolism. Applying even the lower range of the estimate of development of CTEPH from acute PE suggests the actual number of new CTEPH cases each year may be substantially greater than the number of diagnosed cases. Its exact prevalence is unknown.

One must also appreciate that although the link between acute PE and CTEPH appears strong, not every patient who has CTEPH has a history of acute PE: As many as thirty percent of patients with CTEPH do not have a history of overt PE. Further, because postembolism observational studies do not include patients who have no thromboembolic history, the true incidence of CTEPH may be underestimated.

Many patients do not have a history of PE, therefore it’s essential to perform a ventilation/perfusion scan to exclude CTEPH when working up a patient with suspected PH.

So if a patient has CTEPH, what signs might a clinician see pointing to this condition?

There are no pathognomonic symptoms of CTEPH, but the most commonly reported include exercise intolerance, fatigue, and dyspnea. Subsequently, patients may report chest discomfort, syncope, hemoptysis, light-headedness, or peripheral leg edema.

In part because these symptoms are indistinguishable from other forms of PH, and because they can be mistaken for symptoms of respiratory disease, like asthma and COPD, CTEPH is underdiagnosed and often misdiagnosed as another disease.

Since CTEPH, unlike any other form of PH, can be cured through pulmonary endarterectomy or PEA (also known as pulmonary thromboendarterectomy, or PTE), it is imperative to differentiate CTEPH from other PH diagnoses.

So we’ve already discussed history of acute PE as a risk factor for CTEPH, but are there others? Indeed, there are PE-related risk factors and also PE-independent risk factors.

Among PE-related risk factors, we count recurrent, unprovoked, or idiopathic PE; large perfusion defects at detection of PE; young age at detection of PE; high—that is, greater than fifty millimeters of mercury—pulmonary artery systolic pressure; and persistent PH six months after an acute PE.

Some chronic medical conditions—including splenectomy; infections related to cardiac devices, chronic inflammatory disorders; antiphospholipid syndrome; thyroid replacement therapy; and cancers—can also be related to development of CTEPH.

And finally, there are genetic and thrombotic factors that increase the risk of developing CTEPH.

To sum up, there are a few key takeaways I want to leave with you.

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 after at least three months of effective anticoagulation.

Although most patients who have CTEPH have a history of acute PE, a sizable proportion, perhaps upward of thirty percent, have no such history.

CTEPH is potentially curable only with PEA surgery, and it is the only form of PH that may be curable, so it is essential to exclude CTEPH in any patient who is suspected of having pulmonary hypertension.

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.