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McGoon M.D.,Mayo Medical School | Benza R.L.,Allegheny General Hospital | Escribano-Subias P.,A+ Network | Jiang X.,Chinese Academy of Sciences | And 8 more authors.
Journal of the American College of Cardiology | Year: 2013

Registries of patients with pulmonary arterial hypertension (PAH) have been instrumental in characterizing the presentation and natural history of the disease and provide a basis for prognostication. Since the initial accumulation of data conducted in the 1980s, subsequent registry databases have yielded information about the demographic factors, treatment, and survival of patients and have permitted comparisons between populations in different eras and environments. Inclusion of patients with all subtypes of PAH has also allowed comparisons of these subpopulations. We describe herein the basic methodology by which PAH registries have been conducted, review key insights provided by registries, summarize issues related to interpretation and comparison of the results, and discuss the utility of data to predict survival outcomes. Potential sources of bias, particularly related to the inclusion of incident and/or prevalent patients and missing data, are addressed. A fundamental observation of current registries is that survival in the modern treatment era has improved compared with that observed previously and that outcomes among PAH subpopulations vary substantially. Continuing systematic clinical surveillance of PAH will be important as treatment evolves and as understanding of mechanisms advance. Considerations for future directions of registry studies include enrollment of a broader population of patients with pulmonary hypertension of all clinical types and severity and continued globalization and collaboration of registry databases. © 2013 by the American College of Cardiology Foundation. Published by Elsevier Inc.

Peacock A.J.,Scottish Pulmonary Vascular Unit | Noordegraaf A.V.,VU University Amsterdam
European Respiratory Review | Year: 2013

Cardiac magnetic resonance imaging (CMRI) provides accurate information about right ventricular (RV) mass, RV volumes and other markers of RV function. CMRI is proving to be a particularly useful tool in pulmonary arterial hypertension (PAH), as measures of RV function have been shown to be prognostic of long-term outcomes in this disease. Changes in RV function can also provide important information about a patient's disease course and response to treatment. As CMRI is noninvasive it can be used to regularly monitor patients with PAH, which is an important advantage over invasive right heart catheterisation. This review will explore the use of CMRI in the context of existing monitoring tools for PAH and will explore the forthcoming developments that are likely to be important in the future monitoring of patients with PAH. © ERS 2013.

Peacock A.,Scottish Pulmonary Vascular Unit | Keogh A.,St Vincentas Hospital
Current Opinion in Pulmonary Medicine | Year: 2010

Purpose of review: Design and selection of endpoints used in clinical trials is a complex and compelling topic; the diverse needs of clinicians, patients and regulatory authorities represent a challenge in devising the most relevant, meaningful yet practical measures. In trials of therapies for pulmonary arterial hypertension (PAH), a variety of endpoints have been used, including assessments of exercise capacity (6-min walk distance [6MWD]), functional class, hemodynamics, and time to clinical worsening. Most have relied upon 6MWD as the primary endpoint. Recent findings: Accumulating experience and expertise suggests that the relevance of the 6-min walk test as a stand-alone measure of efficacy is now a topic for consideration. Furthermore, 6MWD tends to correlate poorly with other endpoints and, most importantly, there is no linear relationship between 6MWD and morbidity/mortality. Although no linear relationship has been identified with other endpoints, time to clinical worsening may be considered a better endpoint for assessing the effectiveness of PAH therapies, in particular the impact of treatment on disease progression, because it is very relevant to the clinical outcome of patients. Treatment of mildly symptomatic patients, for example, has demonstrated a clear delay in time to clinical worsening and the endpoint might be better suited than others to demonstrate the efficacy of combination therapy. Summary: As the field of PAH develops and progresses, time to clinical worsening may be the best way currently of distinguishing between the increasing number of treatment options available. A clear definition must, therefore, be established, with adjudication by an expert panel; the needs of clinicians, patients, and regulators should be balanced when selecting the most appropriate endpoints. © 2010 Wolters Kluwer Health. Lippincott Williams & Wilkins.

Grunig E.,University of Heidelberg | Peacock A.J.,Scottish Pulmonary Vascular Unit
European Respiratory Review | Year: 2015

Noninvasive imaging of the heart plays an important role in the diagnosis and management of pulmonary hypertension (PH), and several well-established techniques are available for assessing performance of the right ventricle, the key determinant of patient survival. While right heart catheterisation is mandatory for establishing a diagnosis of PH, echocardiography is the most important screening tool for early detection of PH. Cardiac magnetic resonance imaging (CMRI) is also a reliable and practical tool that can be used as part of the diagnostic work-up. Echocardiography can measure a range of haemodynamic and anatomical variables (e.g. pericardial effusion and pulmonary artery pressure), whereas CMRI provides complementary information to echocardiography via high-resolution, three-dimensional imaging. Together with echocardiography and CMRI, techniques such as high-resolution computed tomography and positron emission tomography may also be valuable for screening, monitoring and follow-up assessments of patients with PH, but their clinical relevance has yet to be established. Technological advances have produced new variants of echocardiography, CMRI and positron emission tomography, and these permit closer examination of myocardial architecture, motion and deformation. Integrating these new tools into clinical practice in the future may lead to more precise noninvasive determination of diagnosis, risk and prognosis for PH. © ERS 2015.

Peacock A.,Scottish Pulmonary Vascular Unit
European Respiratory Review | Year: 2013

2011 to 2012 has seen an explosion in published research in the field of pulmonary vascular disease, especially pulmonary hypertension. In conjunction with this research has been an explosion in clinical interest in treating pulmonary hypertension. This is possible because we now have three different generic classes of drug therapy: endothelin receptor antagonists, phosphodiesterase-5 inhibitors and prostacyclins. Clearly, however, we need to be careful that we are treating the correct disease with the correct drug and measuring the correct response. Herein, I will review the papers published over the last year that offer the most insight into the pathobiology, but also those that give us the clinical information we need in epidemiology, treatment and end-points so that we can treat this devastating group of disease. © ERS 2013.

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