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

Vichaya Arunthari*, Charles D Burger
Division of Pulmonary Medicine, Mayo Clinic Jacksonville, 4500 San Pablo Road, Jacksonville, FL 32224, USA

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© Arunthari and Burger; Licensee Bentham Open.

open-access license: This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

* Address correspondence to this author at the Division of Pulmonary Medicine, Mayo Clinic Jacksonville, 4500 San Pablo Road, Jacksonville, FL 32224, USA; Tel: 904-953-2000; Fax: 904-953-2082; E-mail:



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.