Chronic thromboembolic pulmonary hypertension (CTEPH) is viewed as a long-term complication of acute pulmonary embolism. Although its physiopathology remains poorly understood, the hypothesis relies on the fibrotic transformation of thrombi in pulmonary arteries leading to nonhomogeneous vascular obstructions. Together with an overflow arteriopathy in the nonobstructed vascular bed, this causes an increase in pulmonary artery pressure and, finally, right heart failure. Recently, increased awareness of the condition and more active screening have yielded an updated incidence and prevalence of about 2–6 and 26–38 cases/million adults, respectively. Another concept has emerged in recent years, describing patients with post-pulmonary symptoms but without fulfilling the hemodynamic definition of pulmonary hypertension at rest, despite the new threshold established at the 2018 Nice PH conference. This condition has been named Chronic Thromboembolic Disease (CTEPD) or Post Pulmonary Embolism Syndrome, and its management is poorly defined.
Some unanswered questions this Research Topic aims to address are:
• How many patients at the time of acute PE diagnosis have already established CTEPH and how could it be diagnosed?
• Why does a significant number of patients with CTEPH have no history of thromboembolic disease, and why this proportion varies according to geography?
• Has nowadays CT scan pulmonary angiography a sufficient sensitivity and negative predictive value to rule out CTEPH diagnosis?
• Does the risk stratification, as defined to the PAH group1, applies also to CTEPH?
• What is the value of Cardiopulmonary Exercise Testing (CPET) and right heart cath on exercise in the stratification of CTEPD and CTEPH?
• Has DLCO any diagnostic or prognostic value in the management of CTEPH?
• How can we choose between anti-vitamin K and direct anticoagulant?
• How can we compare the degree of vascular obstruction on chest imaging with the hemodynamic impairment? What are the implications for the treatment strategy?
• How can we manage patients with simultaneous comorbidities such as left heart failure with preserved ejection fraction or severe COPD?
• What is the accurate profile and prognostic of patients that are denied surgery?
• Do BPA results differ in patients treated after surgery from naïve patients?
The scope of this Research Topic includes pathophysiology, epidemiology of CTEPH, diagnostic strategy, imaging performance, and risk stratification. While certain clinical risk factors are known to be associated with the development of CTEPH, the molecular mechanisms involved in the defective resolution of the clots remain largely unknown. Specific biological interactions between the endothelial cells and mediators of fibrinolysis are likely involved. A better comprehension of these mechanisms might open perspectives on disease prevention, a topic completely unknown at present. On the therapeutic side, surgical PEA for eligible patients is the gold standard. The place and efficacy of balloon pulmonary angioplasty have been recently assessed. However, for patients that are ineligible for these approaches, or for those incompletely improved by surgery or invasive radiology the medical management remains poorly defined. In particular, we lack evidence for combined therapy, including parenteral prostacyclins, in contrast to group 1 PAH. In this context, real-life experience from registries and large databases is needed. Lastly, the debate about the respective advantage of antivitamin K versus NOAC continues. Articles that may be eligible in this topic are:
• Pathological aspects of CTEPH including molecular and biochemical mechanisms
• Clinical prospective studies
• Clinical retrospective studies, either epidemiological, diagnostic, or therapeutic
• Data from registries
• Post hoc analysis from previous (large enough) clinical studies
• Meta-analyses
• Case series if highlighting a specific and novel aspect of the disease.
• Comparisons of diagnostic tools (eg. echocardiography and cardiac MRI)
• Systematic reviews
Chronic thromboembolic pulmonary hypertension (CTEPH) is viewed as a long-term complication of acute pulmonary embolism. Although its physiopathology remains poorly understood, the hypothesis relies on the fibrotic transformation of thrombi in pulmonary arteries leading to nonhomogeneous vascular obstructions. Together with an overflow arteriopathy in the nonobstructed vascular bed, this causes an increase in pulmonary artery pressure and, finally, right heart failure. Recently, increased awareness of the condition and more active screening have yielded an updated incidence and prevalence of about 2–6 and 26–38 cases/million adults, respectively. Another concept has emerged in recent years, describing patients with post-pulmonary symptoms but without fulfilling the hemodynamic definition of pulmonary hypertension at rest, despite the new threshold established at the 2018 Nice PH conference. This condition has been named Chronic Thromboembolic Disease (CTEPD) or Post Pulmonary Embolism Syndrome, and its management is poorly defined.
Some unanswered questions this Research Topic aims to address are:
• How many patients at the time of acute PE diagnosis have already established CTEPH and how could it be diagnosed?
• Why does a significant number of patients with CTEPH have no history of thromboembolic disease, and why this proportion varies according to geography?
• Has nowadays CT scan pulmonary angiography a sufficient sensitivity and negative predictive value to rule out CTEPH diagnosis?
• Does the risk stratification, as defined to the PAH group1, applies also to CTEPH?
• What is the value of Cardiopulmonary Exercise Testing (CPET) and right heart cath on exercise in the stratification of CTEPD and CTEPH?
• Has DLCO any diagnostic or prognostic value in the management of CTEPH?
• How can we choose between anti-vitamin K and direct anticoagulant?
• How can we compare the degree of vascular obstruction on chest imaging with the hemodynamic impairment? What are the implications for the treatment strategy?
• How can we manage patients with simultaneous comorbidities such as left heart failure with preserved ejection fraction or severe COPD?
• What is the accurate profile and prognostic of patients that are denied surgery?
• Do BPA results differ in patients treated after surgery from naïve patients?
The scope of this Research Topic includes pathophysiology, epidemiology of CTEPH, diagnostic strategy, imaging performance, and risk stratification. While certain clinical risk factors are known to be associated with the development of CTEPH, the molecular mechanisms involved in the defective resolution of the clots remain largely unknown. Specific biological interactions between the endothelial cells and mediators of fibrinolysis are likely involved. A better comprehension of these mechanisms might open perspectives on disease prevention, a topic completely unknown at present. On the therapeutic side, surgical PEA for eligible patients is the gold standard. The place and efficacy of balloon pulmonary angioplasty have been recently assessed. However, for patients that are ineligible for these approaches, or for those incompletely improved by surgery or invasive radiology the medical management remains poorly defined. In particular, we lack evidence for combined therapy, including parenteral prostacyclins, in contrast to group 1 PAH. In this context, real-life experience from registries and large databases is needed. Lastly, the debate about the respective advantage of antivitamin K versus NOAC continues. Articles that may be eligible in this topic are:
• Pathological aspects of CTEPH including molecular and biochemical mechanisms
• Clinical prospective studies
• Clinical retrospective studies, either epidemiological, diagnostic, or therapeutic
• Data from registries
• Post hoc analysis from previous (large enough) clinical studies
• Meta-analyses
• Case series if highlighting a specific and novel aspect of the disease.
• Comparisons of diagnostic tools (eg. echocardiography and cardiac MRI)
• Systematic reviews