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CTEPH Diagnosis

CTEPH diagnosis is challenging.

  • CTEPH’s common symptoms—progressive dyspnea on exertion, rapid exhaustion, fatigue—and its clinical course can be indistinguishable from other forms of severe PH.15
    • If symptoms, signs, and history suggest PH, patients should be urgently referred for further assessments.
  • Patients with CTEPH can present without a history of PE, but studies vary widely in their estimates of what proportion such patients represent.8,15
    • In 2004, Lang reported that of 142 consecutive CTEPH patients, 90 (63%) had no history of acute PE.16
    • Conversely, data reported in 2011 from a European registry, which included 679 patients, showed that 74.8% of CTEPH patients did have a history of acute PE.8
  • CTEPH can progress quickly, though some patients may experience a “honeymoon period” of some months to several years between their acute PE and clinical signs of CTEPH.15
    • Signs of right-heart failure occur late in the course of the disease.15

Using “SCAR” for diagnosis and treatment of CTEPH.

The broad approach to diagnosis and treatment of CTEPH can be described by a simple mnemonic, “SCAR”3:

  • Echocardiography is used in the initial assessment of suspected PH.18
  • According to the Fifth World Symposium on Pulmonary Hypertension, held in 2013 in Nice, France, the V/Q scan is the preferred and recommended screening test for CTEPH.3
    • A diagnosis of CTEPH may be confirmed by the presence of a mismatched wedge-shaped perfusion defect.18
    • Diagnosis of CTEPH can be supported by characteristic findings during multislice CT angiography, including a mosaic perfusion pattern, dilatation of proximal pulmonary arteries and right heart chambers, and the presence of vascular stenosis or obstruction.18
  • Right heart catheterization is used for hemodynamic evaluation to confirm the presence of PH and to provide prognostic information.18
  • Referral to specialized centers for testing to define anatomical locations and extent of obstructions and to quantify the degree of PH is essential.1
  • The sequence of these tests may vary according to the factors suggestive of PH.19

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The V/Q scan is the preferred and recommended screening test for CTEPH.

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

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