Chronic Thromboembolic Pulmonary Hypertension: Results From A Prospective, Observational European Registry

Interventional and surgical modalities of treatment for pulmonary arterial hypertension

AbstractBeyond medical therapy, different interventional and surgical approaches exist for treatment of pulmonary arterial hypertension (PAH). Atrial septostomy has been applied in patients with lack of response to medical therapy in the absence of other surgical treatment options. With growing experience, procedure-related death rates have been reduced to 5.4%, and the most suitable patient group has been identified among patients with a mean right atrial pressure between 10 and 20 mm Hg. Pulmonary endarterectomy is the accepted form of treatment for patients with chronic thromboembolic pulmonary hypertension. Establishing the diagnosis and the classification of the type of lesions by pulm…

Determinants of diagnostic delay in chronic thromboembolic pulmonary hypertension: results from the European CTEPH Registry.

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterised by chronic thrombi in the pulmonary arterial bed, causing pulmonary hypertension [1–3]. CTEPH is diagnosed in ∼3% of patients who survive a symptomatic acute pulmonary embolism (PE) [4]. While the surgical removal of chronic fibrotic thrombotic vascular occlusions by pulmonary endarterectomy (PEA) may cure most patients with CTEPH by normalising pulmonary artery hemodynamics and improving symptoms, patients who remain not operated or do not undergo balloon pulmonary angioplasty have severe functional limitations, and poor quality of life and survival [5, 6]. Since the natural course of CTEPH involves progressive remodell…

Sex-specific differences in chronic thromboembolic pulmonary hypertension. Results from the European CTEPH registry

BACKGROUND: Women are more susceptible than men to several forms of pulmonary hypertension, but have better survival. Sparse data are available on chronic thromboembolic pulmonary hypertension (CTEPH). METHODS: We investigated sex-specific differences in the clinical presentation of CTEPH, performance of pulmonary endarterectomy (PEA), and survival. RESULTS: Women constituted one-half of the study population of the European CTEPH registry (N = 679) and were characterized by a lower prevalence of some cardiovascular risk factors, including prior acute coronary syndrome, smoking habit, and chronic obstructive pulmonary disease, but more prevalent obesity, cancer, and thyroid diseases. The med…

P2540Sex-specific differences in the clinical presentation, surgical complications, and course of chronic thromboembolic pulmonary hypertension

Abstract Background Women are more susceptible to develop several forms of pulmonary hypertension, but they may have better survival rates than men. Sparse data are available concerning sex-specific differences in chronic thromboembolic pulmonary hypertension (CTEPH). Purpose and methods We investigated sex-specific differences in the clinical presentation of CTEPH, functional parameters, exposure to pulmonary endarterectomy (PEA), and survival. Results Women constituted half of the study population (N=679 treatment-naïve patients from the European CTEPH registry) and were characterized by a lower prevalence of some cardiovascular risk factors (e.g. prior acute coronary syndrome, smoking ha…

Riociguat treatment in patients with pulmonary arterial hypertension: Final safety data from the EXPERT registry

OBJECTIVE: The 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 pulmonary hypertension following Phase 3 randomized trials. The EXPosurE Registry RiociguaT in patients with pulmonary hypertension (EXPERT) study was designed to monitor the long-term safety of riociguat in clinical practice. METHODS: EXPERT was an international, multicenter, prospective, uncontrolled, non-interventional cohort study of patients treated with riociguat. Patients were followed for at least 1 year and up to 4 years from enrollment or until 30 days after stopping ri…