Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease observed in a small proportion of patients after acute pulmonary embolism (PE). CTEPH has a high morbidity and mortality rate, related to the PH severity, and a poor prognosis, which mirrors the right ventricular dysfunction involvement. Pulmonary endarterectomy (PEA) reduces pulmonary vascular resistance, making it the treatment of choice and should be offered to operable CTEPH patients, as significant symptomatic and prognostic improvement has been observed. Moreover, these patients may also benefit from the advances made in surgical techniques and pulmonary hypertension-specific medication. However, not all patients are eligible for PEA surgery, as some have either distal pulmonary vascular obstruction and/or significant comorbidities. Therefore, surgical candidates should be carefully selected by an interprofessional team in expert centers. This review aims at making an overview of the risk factors and latest developments in diagnostic tools and treatment options for CTEPH.
Highlights
Pulmonary hypertension secondary to chronic thromboembolism (CTEPH), a potentially life-threatening condition, is within group 4 of the World Health Organization (WHO) clinical classification of pulmonary hypertension (PH), based on specific etiological, clinical, diagnostic, and treatment strategies, and takes the form of a distinct pulmonary vascular disease [1,2,3,4,5,6,7]
Mechanical obstruction of the elastic pulmonary arteries is associated with an increase in pulmonary vascular resistance (PVR), which leads to a pressure increase and ‘shear stress’ which, in turn, leads to arterial disease of the peripheral pulmonary vessels, similar to what can be observed in other forms of precapillary PH [3]
In the Chronic Thromboembolic Pulmonary Hypertension Soluble Guanylate CyclaseStimulator Trial 1 (CHEST-1) study, it was demonstrated that riociguat significantly improved the results of the 6-min walking distance test (6MWD) and the World Health Organization (WHO) functional class and decreased PVR and Nterminal pro-brain natriuretic peptide (NT-proBNP) levels in 261 patients at 16 weeks after initiation of treatment compared to placebo
Summary
Pulmonary hypertension secondary to chronic thromboembolism (CTEPH), a potentially life-threatening condition, is within group 4 of the WHO clinical classification of pulmonary hypertension (PH), based on specific etiological, clinical, diagnostic, and treatment strategies, and takes the form of a distinct pulmonary vascular disease [1,2,3,4,5,6,7]. It can be stated that CTEPH originates from a remodeling process of the vessel wall after embolic obstruction and is initiated and enhanced by the combination of impaired angiogenesis, reduced fibrinolysis, and endothelial dysfunction This process may be influenced by predisposing factors, such as infections, autoimmune diseases, chronic inflammatory diseases, hypothyroidism in hormone replacement therapies, oncological diseases, and plasma factors associated with thrombophilic diathesis, and it leads to the progressive development of PH and, subsequently, right heart failure [20,21,22,23,24,25,26]. Pulmonary endarterectomy (PEA) remains the current potentially curative treatment of choice, especially when the organized thrombi involve the main, lobar, or segmental arteries This is a complex surgical procedure involving the removal of obstructive thromboembolic material from the pulmonary vessels, a high percentage of patients have enhanced quality and life expectancy, and it may even be considered a definitive cure for many of them [27,28,29].
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