Chronic thromboembolic pulmonary hypertension after an acute pulmonary embolism: fundamental concepts of diagnosis and review of current treatment options

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe and underdiagnosed disorder that leads to right ventricular failure and, subsequently, to death. The article discusses the fundamental concepts of monitoring patients after a pulmonary embolism (PE) in order to timely detect CTEPH. The pathogenetic processes, risk factors and diagnostic criteria of this complication are described. The role of early diagnosis of CTEPH, which may be important in relation to further outcomes, is emphasized. A modern strategy for monitoring patients after PE is presented in detail, where echocardiography plays a key role. The stepwise diagnostic algorithm for CTEPH includes a comprehensive analysis of the patient’s condition: assessment of clinical status, identification of N-terminal pro-brain natriuretic peptide levels, cardiopulmonary exercise testing, imaging diagnostic procedures (ventilation/perfusion lung scanning, pulmonary angiography) and, finally, right heart catheterization. The article provides an overview of modern treatment options. CTEPH is a unique type of pulmonary hypertension because it is potentially curable with surgery — pulmonary thromboendarterectomy. For patients with inoperable or persistent/ recurrent CTEPH, medication therapy is recommended. Currently, the only drug with a high class of evidence for this group of patients is riociguat. A positive effect on exercise tolerance, functional class, and hemodynamic parameters has been shown in the CHEST-1,2 studies. The favorable safety profile of the drug was also demonstrated in the long-term follow-up in routine clinical practice (EXPERT registry). The presence of various options in the management of patients and development of a multimodal therapy makes it possible to provide high-quality care to patients with CTEPH, and, namely, fast and accurate diagnosis plays a key role in timely treatment.