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CTEPH reassessment for PAH patients

CTEPH is often misdiagnosed1

CTEPH is often misdiagnosed due to nonspecific symptoms and variable disease course.1


  • Although ventilation/perfusion (V/Q) scans are >96% sensitive for detecting CTEPH and can effectively rule out CTEPH when negative, they are underutilized when screening pulmonary hypertension (PH) patients2,3

  • A US registry of patients (N=786) who had been diagnosed with pulmonary arterial hypertension (PAH) showed that 43% had been diagnosed with PAH despite never having received a V/Q scan to screen for CTEPH4

    • In approximately one-third of the cases without a V/Q scan, the healthcare provider answered that it was not relevant when asked why a V/Q scan was not performed4


If a V/Q scan suggests CTEPH, the patient should be referred to a specialized center with expertise in the treatment of this condition.2



1. Tapson VF and Humbert M. Incidence and prevalence of chronic thromboembolic pulmonary hypertension: from acute to chronic pulmonary embolism. Proc Am Thorac Soc. 2006;3:564-567. 2. Kim NH, Delcroix M, Jenkins DP, et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013;62(suppl D):D92-D99. 3. Tunariu N, Gibbs SJR, Win Z, et al. Ventilation–perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of pulmonary hypertension. J Nucl Med. 2007;48(5):680-684. 4. McLaughlin VV, Langer A, Tan M, et al. Contemporary trends in the diagnosis and management of pulmonary arterial hypertension: an initiative to close the care gap. Chest. 2013;143(2):324-332. 5. Fedullo P, Kerr KM, Kim NH, Auger WR. Chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med. 2011;183(12):1605-1613.