Abstract

In the 2009 European Guidelines on pulmonary hypertension one section covers aspects of pathophysiology, diagnosis and treatment of chronic thromboembolic pulmonary hypertension (CTEPH). The practical implementation of the guidelines for this disease is of crucial importance, because CTEPH is a form of pulmonary hypertension which can be surgically cured. It is, however, frequently diagnosed late in the course of disease and often treated not correctly. In the European Guidelines CTEPH is addressed relatively briefly, although it is a common form of PH which is often overlooked. Any patient with unexplained PH should be evaluated for the presence of CTEPH. A ventilation/perfusion lung scan is recommended as the first step to exclude CTEPH. If the ventilation/perfusion lung scan or multislice CT angiography reveals perfusions defects suggesting the diagnosis of CTEPH, the patient should be referred to a centre with expertise in the medical and surgical management of these patients. After diagnosis of CTEPH the case has to be reviewed by an experienced surgeon in a PEA centre for assessment of operability. The recommendations of the European guidelines are summarized in the current manuscript with additional comments regarding diagnosis and treatment according to most recent evidence.

© Georg Thieme Verlag KG Stuttgart · New York.