Prognostication in Pulmonary Arterial Hypertension and Use of Current Risk Prediction Models

Advances in Pulmonary Hypertension

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关键词: Cardiology;    Rheumatology;    Pediatrics;    Psychosocial Issues;    Lifestyle Issues;    Social Work;    WHO Group 1- Pulmonary Arterial Hypertension;    WHO Group 4;    Surgical Interventions;    symptoms;    Gender;    PH;    Research;    Exercise;    Cardiopulmonary rehabilitation;    Idiopathic (Primary pulmonary hypertension);    Associated Diseases;    Connective Tissue Diseases;    HIV Infection;    Congenital Heart Disease;    Chronic thromboembolic pulmonary hypertension;    Chronic renal failure;    nitric oxide;    Anticoagulation;    Diuretics;    Non-invasive estimate of PVR;    Pulmonary vascular resistance;    Cardiac Output;    exercise hemodynamic testing;    6-Minute Walk Test;    Transplantation;    thromboendarterectomy;    Preserved ejection fraction;    endothelial dysfunction;    Micro Particles;    tricuspid regurgitation;    Functional Class;    Testosterone;    Drug development;    clinical trial endpoints;    REVEAL;    CME;    Classification;    Epidemiology;    Screening;   

DOI  :  

学科分类：医学（综合）

来源: Pulmonary Hypertension Association

【 摘 要 】

Since the time of the National Institutes of Health (NIH) registry conducted in the 1980s of incident pulmonary arterial hypertension (PAH) cases reporting a median survival of 2.8 years after diagnosis,1 significant therapeutic advances in the field, increased awareness and diagnosis, and changing demographics of PAH patients in the contemporary era have facilitated the development of better prognostic tools for predicting survival. One-year survival in the NIH registry before modern therapies existed was a sobering 67%, compared to the 93% 1-year survival estimate of incident PAH cases from US REVEAL (Registry to Evaluate Early and Long-term PAH Disease Management) from 2006-2009 (Figure 1).2 The French PAH registry, which enrolled patients over a 1-year period from 2002-2003, estimated a 1- and 3-year survival of 82.9% and 58.2% respectively.3 The NIH registry is no longer relevant for discussion in the current era, but it serves as an important reference for the natural history of untreated PAH patients. Despite superior survival compared with the NIH registry, overall patient outcomes remain poor, and measurement of most prognostic factors still occurs at the time of initial PAH diagnosis or enrollment into clinical trials, when referral and treatment delays may have substantially affected disease progression.4 We now appreciate that treatment of PAH patients requires an individualized approach based on disease severity and burden of risk factors to improve patient outcomes.5 Clinical experts are increasingly utilizing risk prediction models for prognosticating pulmonary hypertension (PH) groups and the individual patient both at time of diagnosis and in a serial fashion. With serial risk prediction, individual disease trajectories could be mapped and targeted with timely treatment interventions to avoid future morbid and mortal events. Additionally, the field now desires to prioritize treatment goals associated with long-term outcomes rather than rely on short-term functional changes (ie, 6-minute walk distance or 6MWD) that may not meaningfully translate into improved survival.

【 授权许可】

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