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

A model for estimating the health economic impact of earlier diagnosis of chronic thromboembolic pulmonary hypertension

Background Diagnostic delay of chronic thromboembolic pulmonary hypertension (CTEPH) exceeds 1 year, contributing to higher mortality. Health economic consequences of late CTEPH diagnosis are unknown. We aimed to develop a model for quantifying the impact of diagnosing CTEPH earlier on survival, quality-adjusted life-years (QALYs) and healthcare costs. Material and methods A Markov model was developed to estimate lifelong outcomes, depending on the degree of delay. Data on survival and quality of life were obtained from published literature. Hospital costs were assessed from patient records (n=498) at the Amsterdam UMC – VUmc, which is a Dutch CTEPH referral center. Medication costs were ba…

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…

Chronic thromboembolic pulmonary hypertension from the perspective of patients with pulmonary embolism

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but feared long-term complication of acute pulmonary embolism (PE), although CTEPH may occur in patients with no history of symptomatic venous thromboembolism. It represents the most severe presentation of the so-called 'post-PE syndrome', a phenomenon of permanent functional limitations after PE caused by deconditioning after PE or ventilatory or circulatory impairment as a result of unresolved pulmonary artery thrombi. Because the post-PE syndrome may occur in up to 50% of PE survivors, and CTEPH tends to have an insidious and non-specific clinical presentation, CTEPH is often not diagnosed or diagnosed after a very long dela…