CTEPH is a form of pulmonary hypertension (PH), categorized by the WHO as Group 4 PH.4
CTEPH is defined as 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 anticoagulation.5
Adapted from Simonneau et al.4
CTEPH is a rare disease.
Each year, in the US, about 600,000 persons have an acute PE, and it is estimated that there are between 500 and 2500 new cases of CTEPH diagnosed each year.2 A diagnosis of CTEPH is often not correctly made in patients with PH, because they have no overt history of PE.1 In fact, as many as 1 out of every 25 previously treated PE patients (>3 months of anticoagulation5) could develop CTEPH.2,7*
*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.
Uncover common myths and realities concerning surgery for CTEPH in this video.
CTEPH is the only form of PH that is potentially curable by surgery.
Nick H. Kim, MD, of UCSD School of Medicine, provides a background understanding of chronic thromboembolic pulmonary hypertension, or CTEPH, including epidemiology, risk factors, and symptoms.