Background: Chronic thromboembolic pulmonary hypertension (CTEPH) results from inadequate recanalization of the pulmonary circulation after pulmonary thromboembolism. Its 2-year prevalence is 1-4% . If untreated, patients with CTEPH have a mean life expectancy of less than three years. Fortunately, a number of effective treatments are now available.
Methods: This review is based on a selective search of PubMed for pertinent articles published from 1980 to 2014.
Results: The gold-standard test for the exclusion of CTEPH is perfusion scintigraphy: the predictive value of a negative test is nearly 100% . On the other hand, confirmation of a positive diagnosis for treatment planning requires right-heart catheterization and pulmonary angiography. The treatment of first choice for CTEPH is surgical pulmonary endarterectomy (PEA), with which about 70% of patients can be cured. The perioperative mortality of this procedure in experienced centers is now 2-4% . Thirty to 50% of all patients with CTEPH are considered inoperable; for these patients, and for patients with persistent pulmonary hypertension after PEA, the drug riociguat was introduced in Germany in 2014 (the first drug specifically introduced for the treatment of CTEPH). There is also a new interventional treatment option for inoperable patients-pulmonary balloon angioplasty, which is currently being performed in a small number of centers.
Conclusion: The timely diagnosis of CTEPH, followed by referral to a specialized center, is now more important than ever, because treatment options are now available for nearly all of the forms in which this disease can manifest itself.