Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of pulmonary embolism (PE). It is characterized by fibro-thrombotic material obliterating large pulmonary arteries, resulting in increased pulmonary vascular resistance, progressive pulmonary hypertension, right ventricular failure and death if left untreated. The incidence of CTEPH after PE is about 3% and the disease is largely underdiagnosed. The absence of specific symptoms delays its diagnosis resulting in patients presenting at advanced stages with compromised haemodynamic profile and limited exercise capacity. Treatment is complex, involving high risk surgery, angioplasty and drugs.
There is a current need to understand why a subset of patients display persistent perfusion defects after an acute episode of PE and further develop CTEPH. Despite the presence of risk factors such as splenectomy, chronic inflammatory disorders, ventriculo-atrial shunts, infections, cancer or non-O blood group, the pathogenesis of CTEPH remains incompletely understood. Different hypotheses have been suggested such as i) dysregulated thrombolysis including elevated factor VIII and van Willebrand factor (vWF) or abnormalities in fibrinogen structure and function; ii) activation of pulmonary arterial smooth muscle and endothelial cells and involvement of progenitor cells; iii) a concept of inflammatory thrombosis, based on an elevated prevalence of inflammatory diseases, the presence of inflammatory cells within the fibro-thrombotic material obstructing proximal pulmonary and elevated circulating inflammatory mediators; and iv) defective angiogenesis by preventing proper thrombus resolution and its association with adverse outcome in CTEPH patients.
The aim will be to discuss the recently published aspects of the involvement of inflammatory thrombosis and defective angiogenesis in the pathogenesis of CTEPH.
Rozenn Quarck completed her PhD in biomedical sciences at the University Denis Diderot in Paris (France) in 1996.
Since 2015, she is holding a position as scientific research expert within the division of Respiratory Diseases at the University of Leuven and as clinical data manager within the Centre of Pulmonary Vascular Diseases at the University Hospitals of Leuven.
Her research interest is focused on the biology of the pulmonary circulation, pulmonary vascular diseases, physiopathology and genetics of pulmonary hypertension using in vitro and animal models.
She has published 40 peer-reviewed articles in the field of vascular biology and pulmonary hypertension.