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CTEPH Basics

Nick H. Kim, MD, of UCSD School of Medicine, provides a background understanding of chronic thromboembolic pulmonary hypertension, or CTEPH, including epidemiology, risk factors, and symptoms.

  • Nick H. Kim, MD, of UCSD School of Medicine, provides a background understanding of chronic thromboembolic pulmonary hypertension, or CTEPH, including epidemiology, risk factors, and symptoms.

    Nick H. Kim, MD, of UCSD School of Medicine, provides a background understanding of chronic thromboembolic pulmonary hypertension, or CTEPH, including epidemiology, risk factors, and symptoms.
    Click to Play Video

  • Learn more about the stages of progression of an acute clot to CTEPH, sometimes mistakenly referred to as "chronic clot" or "chronic PE".

    Learn more about the stages of progression of an acute clot to CTEPH, sometimes mistakenly referred to as "chronic clot" or "chronic PE".
    Click to Play Video

  • Victor F. Tapson, MD, of Cedars-Sinai Medical Center, Los Angeles, looks at how CTEPH develops, how it manifests, and several important considerations in distinguishing CTEPH from PAH.

    Victor F. Tapson, MD, of Cedars-Sinai Medical Center, Los Angeles, looks at how CTEPH develops, how it manifests, and several important considerations in distinguishing CTEPH from PAH.
    Click to Play Video

  • Raymond L. Benza, MD, of Allegheny General Hospital, Pittsburgh, works through the differential diagnosis of CTEPH, outlining diagnostic difficulties and a helpful mnemonic device to guide clinicians.

    Raymond L. Benza, MD, of Allegheny General Hospital, Pittsburgh, works through the differential diagnosis of CTEPH, outlining diagnostic difficulties and a helpful mnemonic device to guide clinicians.
    Click to Play Video

  • William R. Auger, MD, of UCSD School of Medicine, discusses the use of V/Q scanning and right heart catheterization in diagnosing chronic thromboembolic pulmonary hypertension, or CTEPH.

    William R. Auger, MD, of UCSD School of Medicine, discusses the use of V/Q scanning and right heart catheterization in diagnosing chronic thromboembolic pulmonary hypertension, or CTEPH.
    Click to Play Video

  • Paul Forfia, MD, of Temple University Hospital, Philadelphia, discusses how venous thromboembolism can be a potential precursor to CTEPH.

    Paul Forfia, MD, of Temple University Hospital, Philadelphia, discusses how venous thromboembolism can be a potential precursor to CTEPH.
    Click to Play Video

  • Richard Channick, MD, of Massachusetts General Hospital reviews pulmonary thromboendarterectomy—which is commonly known as PTE or PEA—the only potentially curative treatment for CTEPH.

    Richard Channick, MD, of Massachusetts General Hospital reviews pulmonary thromboendarterectomy—which is commonly known as PTE or PEA—the only potentially curative treatment for CTEPH.
    Click to Play Video

  • Raymond L. Benza, MD, of Allegheny Health Network, Pittsburgh, reviews the respective roles of ventilation/perfusion scans and computed tomographic pulmonary angiography in CTEPH.

    Raymond L. Benza, MD, of Allegheny Health Network, Pittsburgh, reviews the respective roles of ventilation/perfusion scans and computed tomographic pulmonary angiography in CTEPH.
    Click to Play Video

  • In this video, Dr Michael Madani, of the PTE Program at UCSD, shows key steps—including extraction of thromboembolic material—in performing PTE surgery (also known as PEA).

    In this video, Dr Michael Madani, of the PTE Program at UCSD, shows key steps—including extraction of thromboembolic material—in performing PTE surgery (also known as PEA).
    Click to Play Video

  • Dr Bill Auger of the PTE Program at UCSD reviews important considerations in evaluating CTEPH patients’ eligibility for potentially curative PTE surgery (also known as PEA).

    Dr Bill Auger of the PTE Program at UCSD reviews important considerations in evaluating CTEPH patients’ eligibility for potentially curative PTE surgery (also known as PEA).
    Click to Play Video

  • Ivan Robbins, MD, of Vanderbilt University Medical Center, Nashville, presents a case illustrating the importance of the ventilation/perfusion scan as a screen for CTEPH.

    Ivan Robbins, MD, of Vanderbilt University Medical Center, Nashville, presents a case illustrating the importance of the ventilation/perfusion scan as a screen for CTEPH.
    Click to Play Video

  • CTEPH Basics

    Nick H. Kim, MD, of UCSD School of Medicine, provides a background understanding of chronic thromboembolic pulmonary hypertension, or CTEPH, including epidemiology, risk factors, and symptoms.
    Click to Play Video

  • The Pathophysiology of CTEPH

    Victor F. Tapson, MD, of Cedars-Sinai Medical Center, Los Angeles, looks at how CTEPH develops, how it manifests, and several important considerations in distinguishing CTEPH from PAH.
    Click to Play Video

  • Differential Diagnosis of CTEPH

    Raymond L. Benza, MD, of Allegheny General Hospital, Pittsburgh, works through the differential diagnosis of CTEPH, outlining diagnostic difficulties and a helpful mnemonic device to guide clinicians.
    Click to Play Video

  • V/Q Scanning and RHC in CTEPH

    William R. Auger, MD, of UCSD School of Medicine, discusses the use of V/Q scanning and right heart catheterization in diagnosing chronic thromboembolic pulmonary hypertension, or CTEPH.
    Click to Play Video

  • Potential Precursors to CTEPH

    Paul Forfia, MD, of Temple University Hospital, Philadelphia, discusses how venous thromboembolism can be a potential precursor to CTEPH.
    Click to Play Video

  • PTE/PEA Surgery

    Richard Channick, MD, of Massachusetts General Hospital reviews pulmonary thromboendarterectomy—which is commonly known as PTE or PEA—the only potentially curative treatment for CTEPH.
    Click to Play Video

  • V/Q scan versus CTPA

    Raymond L. Benza, MD, of Allegheny Health Network, Pittsburgh, reviews the respective roles of ventilation/perfusion scans and computed tomographic pulmonary angiography in CTEPH.
    Click to Play Video

  • Key Steps in Performing PTE Surgery

    In this video, Dr Michael Madani, of the PTE Program at UCSD, shows key steps—including extraction of thromboembolic material—in performing PTE surgery (also known as PEA).
    Click to Play Video

  • Important Operability Assessment Considerations

    Dr Bill Auger of the PTE Program at UCSD reviews important considerations in evaluating CTEPH patients’ eligibility for potentially curative PTE surgery (also known as PEA).
    Click to Play Video

  • Reassessing a Misdiagnosis

    Ivan Robbins, MD, of Vanderbilt University Medical Center, Nashville, presents a case illustrating the importance of the ventilation/perfusion scan as a 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.