Utility of D-Dimer in the Diagnosis of Patients with Chronic Thromboembolic Pulmonary Hypertension

The Open Respiratory Medicine Journal 05 June 2009 RESEARCH ARTICLE DOI: 10.2174/1874306400903010085



Chronic thromboembolic pulmonary hypertension (CTEPH) is an important cause of severe pulmonary hypertension (PH). D-dimer, a degradation product of fibrin, has been used as a marker for various diseases. In patients with idiopathic pulmonary arterial hypertension there is evidence to suggest that D-dimer levels are associated with disease severity; however, data regarding D-dimer in patients with CTEPH are lacking.


To assess the significance of D-dimer in patients with CTEPH.

Patients and Methods:

Retrospective chart review of 618 patients seen at our PH clinic from 1991 to June 2008. Data collection focused on patients diagnosed with CTEPH, D-dimer levels, demographics, clinical, and hemodynamics. We compared D-dimer levels in CTEPH patients or World Health Organization (WHO) diagnostic group 4 with PH patients in WHO group 1.


Thirty-four patients with confirmed CTEPH were identified, of these 19 had D-dimer levels and 7 were positive. Of the 234 patients in WHO group 1 excluding patients with portopulmonary hypertension (n = 54) and pulmonary venoocclusive disease (n = 2) 97 had D-dimer levels and 52 were positive. We found an estimated sensitivity of the D-dimer test in diagnosing CTEPH was 37% while the estimated specificity was 46%. The positive predictive value and negative predictive value were 12% and 79% respectively.


D-dimer is an insensitive and nonspecific test for the diagnosis of CTEPH. Despite a high negative predictive value D-dimer alone cannot be used to rule out CTEPH in patients with PH.

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