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Differentiating & Identifying CTEPH from PAH

CTEPH defined:

  • CTEPH is not simply a blood clot.
  • CTEPH is a distinct medical condition4 defined by a mean pulmonary arterial pressure ≥25 mmHg and pulmonary capillary wedge pressure ≤15 mmHg in the presence of multiple chronic/organized occlusive thrombi/emboli in the elastic pulmonary arteries (main, lobar, segmental, subsegmental) after at least three months of effective anticoagulation.5
  • Despite similar symptoms, CTEPH is not PAH. The chart chart below differentiates between CTEPH (WHO Group 4) and PAH (WHO Group 1). Perform a V/Q scan to distinguish potentially surgically curable CTEPH.3

Differences between CTEPH and PAH

CTEPH “is a disease with a mechanical component potentially amenable to surgery, and a variable degree of small vessel arteriopathy. Pulmonary thromboendarterectomy is a potential cure, and the treatment of choice for CTEPH; therefore, differentiation between CTEPH and PAH is paramount.” 20

  • Can medical therapy delay or replace pulmonary thromboendarterectomy? Watch this video to find out.

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Differentiation between CTEPH and PAH is paramount.

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.

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