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Epidemiology and risk factors for chronic thromboembolic pulmonary hypertension

Published:January 08, 2018DOI:https://doi.org/10.1016/j.thromres.2018.01.012
Epidemiology and risk factors for chronic thromboembolic pulmonary hypertension
Previous ArticleCurrent understanding of the pathophysiology of chronic thromboembolic pulmonary hypertension
Next ArticlePrevention and treatment of the chronic thromboembolic pulmonary hypertension
      Chronic thromboembolic pulmonary hypertension (CTEPH) as a secondary form of pulmonary hypertension is unique in its potential for remedial intervention. CTEPH is defined by a mean pulmonary artery pressure >25 mm Hg in the setting of thrombotic pulmonary vascular obstruction [
      • Kim N.H.
      • Delcroix M.
      • Jenkins D.P.
      • Channick R.
      • Dartevelle P.
      • Jansa P.
      • Lang I.
      • Madani M.M.
      • Ogino H.
      • Pengo V.
      • et al.
      Chronic thromboembolic pulmonary hypertension.
      J. Am. Coll. Cardiol. 2013; 62
      ]. The disease occurs after preceding massive or recurrent pulmonary embolism in the majority of patients but upwards of one-quarter of patients referred for surgical management have no document history of a prior acute venous thromboembolic event [
      • Pepke-Zaba J.
      • Delcroix M.
      • Lang I.
      • Mayer E.
      • Jansa P.
      • Ambroz D.
      • Treacy C.
      • D'Armini A.M.
      • Morsolini M.
      • Snijder R.
      • et al.
      Chronic thromboembolic pulmonary hypertension (cteph): results from an international prospective registry.
      Circulation. 2011; 124: 1973-1981
      ]. In this review we will discuss the epidemiology and risk factors for the progression from acute to chronic pulmonary embolism, and the development of CTEPH (Table 1).
      Table 1Selected risk factors and univariate odds ratios for CTEPH.
      Risk factor Comparator OR (95% CI) Ref.
      VA shunt IPAH 19.49 (2.47–2520) [
      • Bonderman D.
      • Wilkens H.
      • Wakounig S.
      • Schäfers H.J.
      • Jansa P.
      • Lindner J.
      • Simkova I.
      • Martischnig A.M.
      • Dudczak J.
      • Sadushi R.
      Risk factors for chronic thromboembolic pulmonary hypertension.
      Eur. Respir. J. 2009; 33: 325-331
      ]
      Splenectomy IPAH 22.09 (2.97–2824) [
      • Bonderman D.
      • Wilkens H.
      • Wakounig S.
      • Schäfers H.J.
      • Jansa P.
      • Lindner J.
      • Simkova I.
      • Martischnig A.M.
      • Dudczak J.
      • Sadushi R.
      Risk factors for chronic thromboembolic pulmonary hypertension.
      Eur. Respir. J. 2009; 33: 325-331
      ]
      Massive/submassive PE IPAH 13.03 (p = .004)
      Adjusted for age and sex.
      		 [
      • Lang I.M.
      • Simonneau G.
      • Pepke-Zaba J.W.
      • Mayer E.
      • Ambrož D.
      • Blanco I.
      • Torbicki A.
      • Mellemkjaer S.
      • Yaici A.
      • Delcroix M.
      Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension. A case-control study.
      Thromb. Haemost. 2013; 110: 83-91
      ]
      VTE history IPAH 49.01 (p < .001)
      Adjusted for age and sex.
      		 [
      • Lang I.M.
      • Simonneau G.
      • Pepke-Zaba J.W.
      • Mayer E.
      • Ambrož D.
      • Blanco I.
      • Torbicki A.
      • Mellemkjaer S.
      • Yaici A.
      • Delcroix M.
      Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension. A case-control study.
      Thromb. Haemost. 2013; 110: 83-91
      ]
      Recurrent VTE IPAH 45.02 (21.00–114.73) [
      • Bonderman D.
      • Wilkens H.
      • Wakounig S.
      • Schäfers H.J.
      • Jansa P.
      • Lindner J.
      • Simkova I.
      • Martischnig A.M.
      • Dudczak J.
      • Sadushi R.
      Risk factors for chronic thromboembolic pulmonary hypertension.
      Eur. Respir. J. 2009; 33: 325-331
      ]
      Thyroid replacement IPAH 5.41 (2.70–12.23) [
      • Bonderman D.
      • Wilkens H.
      • Wakounig S.
      • Schäfers H.J.
      • Jansa P.
      • Lindner J.
      • Simkova I.
      • Martischnig A.M.
      • Dudczak J.
      • Sadushi R.
      Risk factors for chronic thromboembolic pulmonary hypertension.
      Eur. Respir. J. 2009; 33: 325-331
      ]
      Hypothyroidism Resolved PE 4.3 (1.4–13.0) [
      • Klok F.A.
      • Dzikowska-Diduch O.
      • Kostrubiec M.
      • Vliegen H.W.
      • Pruszczyk P.
      • Hasenfuß G.
      • Huisman M.V.
      • Konstantinides S.
      • Lankeit M.
      Derivation of a clinical prediction score for chronic thromboembolic pulmonary hypertension after acute pulmonary embolism.
      J. Thromb. Haemost. 2016; 14: 121-128
      ]
      Prior VTE IPAH 19.36 (11.66–33.79) [
      • Bonderman D.
      • Wilkens H.
      • Wakounig S.
      • Schäfers H.J.
      • Jansa P.
      • Lindner J.
      • Simkova I.
      • Martischnig A.M.
      • Dudczak J.
      • Sadushi R.
      Risk factors for chronic thromboembolic pulmonary hypertension.
      Eur. Respir. J. 2009; 33: 325-331
      ]
      APS/lupus AC IPAH 3.28 (1.58–7.50) [
      • Bonderman D.
      • Wilkens H.
      • Wakounig S.
      • Schäfers H.J.
      • Jansa P.
      • Lindner J.
      • Simkova I.
      • Martischnig A.M.
      • Dudczak J.
      • Sadushi R.
      Risk factors for chronic thromboembolic pulmonary hypertension.
      Eur. Respir. J. 2009; 33: 325-331
      ]
      Non-blood group O IPAH 3.12 (p < .001)
      Adjusted for age and sex.
      		 [
      • Lang I.M.
      • Simonneau G.
      • Pepke-Zaba J.W.
      • Mayer E.
      • Ambrož D.
      • Blanco I.
      • Torbicki A.
      • Mellemkjaer S.
      • Yaici A.
      • Delcroix M.
      Factors associated with diagnosis and operability of chronic thromboembolic pulmonary hypertension. A case-control study.
      Thromb. Haemost. 2013; 110: 83-91
      ]
      Malignancy IPAH 1.99 (1.01–4.26) [
      • Bonderman D.
      • Wilkens H.
      • Wakounig S.
      • Schäfers H.J.
      • Jansa P.
      • Lindner J.
      • Simkova I.
      • Martischnig A.M.
      • Dudczak J.
      • Sadushi R.
      Risk factors for chronic thromboembolic pulmonary hypertension.
      Eur. Respir. J. 2009; 33: 325-331
      ]
      Unprovoked PE Resolved PE 20.0 (2.7–>100) [
      • Klok F.A.
      • Dzikowska-Diduch O.
      • Kostrubiec M.
      • Vliegen H.W.
      • Pruszczyk P.
      • Hasenfuß G.
      • Huisman M.V.
      • Konstantinides S.
      • Lankeit M.
      Derivation of a clinical prediction score for chronic thromboembolic pulmonary hypertension after acute pulmonary embolism.
      J. Thromb. Haemost. 2016; 14: 121-128
      ]
      RV dysfunction at diagnosis Resolved PE 4.1 (1.4–12.0) [
      • Klok F.A.
      • Dzikowska-Diduch O.
      • Kostrubiec M.
      • Vliegen H.W.
      • Pruszczyk P.
      • Hasenfuß G.
      • Huisman M.V.
      • Konstantinides S.
      • Lankeit M.
      Derivation of a clinical prediction score for chronic thromboembolic pulmonary hypertension after acute pulmonary embolism.
      J. Thromb. Haemost. 2016; 14: 121-128
      ]
      Symptoms >2 weeks prior to PE diagnosis Resolved PE 7.9 (3.3–19.0) [
      • Klok F.A.
      • Dzikowska-Diduch O.
      • Kostrubiec M.
      • Vliegen H.W.
      • Pruszczyk P.
      • Hasenfuß G.
      • Huisman M.V.
      • Konstantinides S.
      • Lankeit M.
      Derivation of a clinical prediction score for chronic thromboembolic pulmonary hypertension after acute pulmonary embolism.
      J. Thromb. Haemost. 2016; 14: 121-128
      ]
      Age >60 years Resolved PE 2.9 (1.2–7.2) [
      • Klok F.A.
      • Dzikowska-Diduch O.
      • Kostrubiec M.
      • Vliegen H.W.
      • Pruszczyk P.
      • Hasenfuß G.
      • Huisman M.V.
      • Konstantinides S.
      • Lankeit M.
      Derivation of a clinical prediction score for chronic thromboembolic pulmonary hypertension after acute pulmonary embolism.
      J. Thromb. Haemost. 2016; 14: 121-128
      ]
      a Adjusted for age and sex.
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      Article info

      Publication history

      Published online: January 08, 2018
      Accepted: January 7, 2018
      Received in revised form: January 5, 2018
      Received: May 3, 2017

      Identification

      DOI: https://doi.org/10.1016/j.thromres.2018.01.012

      Copyright

      © 2018 Elsevier Ltd. All rights reserved.

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