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. 2010 Nov-Dec;17(6):301–334. doi: 10.1155/2010/704258

TABLE 5.

Risk factors for the development of chronic thromboembolic pulmonary hypertension (CTEPH)

Individual risk factors Author (reference), year
Risk factors present at the time of acute pulmonary embolism diagnosis
 Younger age Pengo et al (22), 2004; Becattini et al (24), 2006
 Previous pulmonary embolism Pengo et al (22), 2004
 Idiopathic pulmonary embolism (absence of either transient or permanent risk factors*) Pengo et al (22), 2004
 Large perfusion defects Pengo et al (22), 2004; Becattini et al (24), 2006
 Recurrent pulmonary embolism Pengo et al (22), 2004
Risks factors observed at the time of CTEPH diagnosis
 Demographic
  Female sex in Japanese population Tanabe et al (48), 2005; Nakamura et al (47), 2002
  Non-O blood group Bonderman et al (32), 2009
 Thrombophilias
  Antiphospholipid antibodies Bonderman et al (32), 2009; Wolf et al (46), 2000; Tanabe et al (48), 2005;
 Nakamura et al (47), 2002; Colorio et al (52), 2001; Bonderman et al (67), 2007;
 Jais et al (55), 2005, Martinuzzo et al (56), 1998
  Factor V Leiden mutation Lang et al (50), 1996, Wolf et al (46), 2000
  Hemoglobin abnormalities Bonderman et al (32), 2009; Bonderman et al (72), 2005
  Increased factor VIII level Wong et al (51), 2010; Bonderman et al (54), 2003
  Increased plasma lipoprotein (a) level Ignatescu et al (59), 1998
 Disease associations
  Splenectomy Condliffe et al (31), 2009; Bonderman et al (32), 2009; Suntharalingam et al (45), 2007;
 Bonderman et al (67), 2007; Jais et al (55), 2005; Jais et al (68), 2003;
 Chou et al (69), 2001; Cappellini et al (70), 2000; Stewart et al (71), 1996;
 Bonderman et al (72), 2005
  Ventriculoatrial shunt Bonderman et al (32), 2009; Bonderman et al (72), 2005
  Thyroid replacement therapy Bonderman et al (32), 2009
  Inflammatory disorder (eg, osteomyelitis, inflammatory bowel disease) Bonderman et al (72), 2005
*

Permanent risk factor defined as constitutive thrombophilia (deficiency of antithrombin, protein C or protein S; mutation in factor V Leiden or prothrombin gene; and the presence of lupus anticoagulants), active cancer, immobilization from chronic medical illness, or two or more first-degree relatives with venous thromboembolism.

This is controversial: the prevalence of factor V Leiden mutations was higher than in patients with idiopathic pulmonary arterial hypertension or healthy subjects (46). However, in another study (50), the prevalence of factor V Leiden in CTEPH was found to be comparable with the general population