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chronic thromboembolic pulmonary hypertension (CTEPH)

Etiology: - pulmonary embolism (history of pulmonary embolism) 50% Pathology: - characterized by recurrent, small pulmonary emboli over an extended period of time leading to pulmonary hypertension & impaired gas exchange - obstruction of medium-size to larger pulmonary arteries [1] - right heart failure & tricuspic regurgitation due to pulmonary hypertension [1] - absence of left heart pressure overload (congestive heart failure) Clinical manifestations: - persistent or progressive dyspnea, especially dyspnea on exertion Special laboratory: - pulmonary artery catheterization if CTEPH is confirmed by VQ scan - pulmonary artery pressures & VQ scan* - mean pulmonary artery pressure > 20 mm Hg - compatible imaging by VQ scan [1] * VQ scanning superior to pulmonary angiography Radiology: - ventilation-perfusion scan (VQ scan) showing VQ mismatch - method of choice in the evaluation of chronic thromboembolic pulmonary hypertension [1,2] - CT angiography to localize obstructing thrombus/thrombi if pulmonary endarterectomy is being considered Management: - long term anticoagulation - pulmonary endarterectomy may be indicated if proximal thromboembolism & functional impairment is severe - medical therapy for CTEPH is riociguat [1] - bosentan is third-line therapy after pulmonary endarterectomy & riociguat - infusion of epoprostenol & other parenteral prostacyclin analogues in patients taking oral therapy with disease progression after determining pulmonary endarterectomy not an option [1] - calcium channel blockers are not used as a primary treatment modality for CTEPH [1] - see pulmonary embolism

Related

chronic pulmonary embolism

General

pulmonary hypertension pulmonary embolism (PE)

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2012, 2015, 2018, 2021. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022, 2023
  2. Fedullo P, Kerr KM, Kim NH, Auger WR. Chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med. 2011 Jun 15;183(12):1605-13. PMID: 21330453
  3. Kim NH, Delcroix M, Jenkins DP et al Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D92-9 PMID: 24355646
  4. Edward JA, Mandras S. An Update on the Management of Chronic Thromboembolic Pulmonary Hypertension. Curr Probl Cardiol. 2017 Jan;42(1):7-38. Review. PMID: 27989311
  5. Piazza G, Goldhaber SZ. Chronic thromboembolic pulmonary hypertension N Engl J Med. 2011 Jan 27;364(4):351-60. PMID: 21268727 Review
  6. Mahmud E, Madani MM, Kim NH et al Chronic Thromboembolic Pulmonary Hypertension: Evolving Therapeutic Approaches for Operable and Inoperable Disease. J Am Coll Cardiol. 2018 May 29;71(21):2468-2486 PMID: 29793636 Free article