Chronic thromboembolic pulmonary hypertension: time for research in pathophysiology to catch up with developments in treatment

The modern treatment era in chronic thromboembolic disease has seen significant advances in both surgical and medical treatment. One such treatment, the pulmonary endarterectomy (where established chronic organized thrombus is removed), has dramatically affected morbidity and mortality. These advances have outstripped basic research into the causes and pathophysiology of disease, which remain largely poorly understood. In this review, we will set out to explain some of the historical reasons for this, including the difficulties inherent in human studies and the lack of good animal models. We will review some of the recent advances in pathophysiology from registries and translational research, and we will summarize the treatment options, with some discussion of very recently published work, including medical and surgical treatments, both traditional and more experimental work in non-invasive techniques.