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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

 

Differentiation
between CTEPH and PAH is paramount

Watch Raymond L. Benza, MD, of Allegheny General Hospital, Pittsburgh, work through the differential diagnosis of CTEPH, outlining diagnostic difficulties and a helpful mnemonic device for physicians

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.