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What is CTEPH?

  • CTEPH is not simply a blood clot

  • CTEPH is a distinct medical condition defined by1,2:

    • Mean pulmonary arterial pressure ≥25 mmHg and pulmonary capillary wedge pressure ≤15 mmHg in the presence of multiple chronic/organized occlusive thrombi/emboli in the elastic pulmonary arteries (main, lobar, segmental, subsegmental) after at least three months of effective anticoagulation1,2

  • Despite similar symptoms, CTEPH is not PAH. Perform a ventilation/perfusion (V/Q) scan to distinguish potentially surgically curable CTEPH3

 

differentiating CTEPH and PAH graphic

 

Differentiating CTEPH from PAH is essential

CTEPH is potentially curable with surgery and has a variable degree of small vessel arteriopathy. Pulmonary thromboendarterectomy is a potential cure and the recommended treatment for CTEPH. Therefore, differentiation between CTEPH and PAH is paramount.7

 

Learn about echocardiography for CTEPH

References:

1. Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62(suppl 25):D34-D41. 2. Wilkens H, Lang I, Behr J, et al. Chronic thromboembolic pulmonary hypertension (CTEPH): updated recommendations of the Cologne Consensus Conference 2011. Int J Cardiol. 2011;154(Suppl 1):S54-S60. 3. Kim NH, Delcroix M, Jenkins DP, et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013;62(suppl D):D92-D99. 4. Humbert M. Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology. Eur Respir Rev. 2010;19:59-63. 5. Galiè N, Hoeper MM, Humbert M, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2009;30(20):2493-2537. 6. McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension. Circulation. 2009;119(116):2250-2294. 7. Keogh AM, Mayer E, Benza RL, et al. Interventional and surgical modalities of treatment in pulmonary hypertension. J Am Coll Cardiol. 2009;54(suppl 1):S67-S77.

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