Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are two of the key subgroups of pulmonary hypertension. They are characterised by different risk factors. PAH can be associated with mutations in the gene encoding bone morphogenetic protein receptor type II (BMPR2), HIV infection, congenital heart disease, connective tissue disease (such as systemic...

Pulmonary hypertension (PH) is a severe haemodynamic disorder in which the pulmonary artery pressure is persistently elevated, leading to right-sided heart failure and death. Recently, chronic myeloproliferative diseases associated with pulmonary hypertension were included in the group 5 category, corresponding to PH for which the aetiology is unclear and/or multifactorial. In this review we wi...

The historical perspective Chronic thromboembolic pulmonary hypertension (CTEPH) is a unique form of pulmonary hypertension (PH) arising from the obstruction of pulmonary arterial vessels by organised thromboembolic material [1]. Much like other forms of PH, CTEPH has historically proven to be a challenging clinical entity in that it is frequently underdiagnosed and undertreated [1–3]. This lac...

D uring the last 20 yrs, a tremendous amount of scientific discoveries have greatly improved our knowledge on the pathophysiology of pulmonary hypertension, and expanded our armamentarium of important novel medications that have substantially improved survival and the quality of life of patients suffering from this devastating disorder. Although most investigations have focused on pulmonary art...

Chronic thromboembolic pulmonary hypertension (CTEPH) is a disabling condition that is being increasingly recognised. It is unique as a cause of pulmonary hypertension in that it is surgically curable. We wish to highlight the importance of recognition and early referral of any patient who may have CTEPH even in the absence of resting pulmonary hypertension as excellent results can be achieved ...

Pulmonary endarterectomy (PEA) is used to treat chronic thromboembolic pulmonary hypertension (CTEPH) patients, and it can effectively remove organized thrombotic materials proliferative intima as well improve hemodynamics. It has been reported that the levels of several inflammatory factors were altered in peri-operative period PEA. Even though their specific role remains unknown, this could h...

ObjectiveThe soluble guanylate cyclase stimulator riociguat is approved for the treatment of adult patients with pulmonary arterial hypertension (PAH) and inoperable or persistent/recurrent chronic thromboembolic (CTEPH) following Phase 3 randomized trials. The EXPosurE Registry RiociguaT in (EXPERT) study was designed to monitor long-term safety clinical practice.MethodsEXPERT an international...