Chronic thromboembolic pulmonary hypertension: an underdiagnosed entity?

ABSTRACT

Chronic thromboembolic pulmonary hypertension (CTEPH) is the only cause of pulmonary hypertension for which there is a potential cure, which is in the form of pulmonary endarterectomy. There is a strong link between pulmonary embolism (PE) and the development of CTEPH. Although CTEPH was initially believed to be a rare complication, this belief has been reconsidered following several studies suggesting that up to 8.8% of patients develop CTEPH within the 2 years after PE. However, considering the incidence of PE, there is a significant discrepancy in the number of patients who are diagnosed, referred, and treated for CTEPH. Potential reasons for this include its often vague clinical presentation, the variable association of CTEPH with PE, and discrepancies when interpreting imaging studies. Underdiagnosis of CTEPH is preventing patients from accessing potentially curative therapy. Increased awareness about this condition is an important initial step to improving diagnostic rates and treatment.