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CTEPH epidemiology, SIGNS, symptoms, 
and pathophysiology

CTEPH is a form of PH

CTEPH is a form of pulmonary hypertension (PH), categorized by the WHO as Group 4 PH.1

 

CTEPH is defined as mean pulmonary arterial pressure ≥25 mm Hg and pulmonary capillary wedge pressure ≤15 mm Hg 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 anticoagulation.2

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CTEPH is a rare disease

Previous estimates approximate that nearly 600,000 people in the United States have an acute pulmonary embolism (PE) each year and between 500–2,500 new cases of CTEPH are diagnosed yearly.3

 

A diagnosis of CTEPH is often not correctly made in patients with PH, because they have no overt history of PE.4 As many as 1 out of every 25 patients previously treated for PE (>3 months of anticoagulation) could develop CTEPH.2,3,5*

*Based on a study with 223 patients in which 3.8% were diagnosed with CTEPH within 2 years of their first episode of pulmonary embolism with or without prior deep-vein thrombosis (95% CI, 1.1 to 6.5). CTEPH did not develop after two years in any of the 132 remaining patients with more than 2 years of follow-up.

 

 

As many as 1 out of 25 pulmonary embolism (PE) patients previously treated with ≥3 months of anticoagulation therapy could develop CTEPH2-4*

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*Based on a study with 223 patients in which 3.8% were diagnosed with CTEPH within 2 years of their first episode of pulmonary embolism with or without prior deep-vein thrombosis (95% CI, 1.1 to 6.5). CTEPH did not develop after two years in any of the 132 remaining patients with more than 2 years of follow-up.
Learn more about the epidemiology of CTEPH

References:

1. Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53:1801913. 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. Tapson VF and Humbert M. Incidence and prevalence of chronic thromboembolic pulmonary hypertension: from acute to chronic pulmonary embolism. Proc Am Thorac Soc. 2006;3:564-567. 4. Piazza G and Goldhaber SZ. Chronic thromboembolic pulmonary hypertension. N Engl J Med. 2011;364:351-360. 5. Pengo V, Lensing AWA, Prins MH, et al. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med. 2004;350(22):2257-2264.

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