Scottish Pulmonary Vascular Unit, Golden Jubilee National Hospital, Agamemnon Street, Glasgow, UK.
Am J Respir Crit Care Med. 2012 Oct 15;186(8):790-6. doi: 10.1164/rccm.201203-0383OC. Epub 2012 Jul 12.
Incident pulmonary arterial hypertension was underrepresented in most pulmonary hypertension registries and may have a different disease profile to prevalent disease.
To determine the characteristics and outcome of a purely incident, treatment-naive cohort of idiopathic, heritable, and anorexigen-associated pulmonary arterial hypertension and to determine the changes in presentations and survival over the past decade in the United Kingdom and Ireland.
All consecutive newly diagnosed patients from 2001 to 2009 were identified prospectively.
A total of 482 patients (93% idiopathic, 5% heritable, and 2% anorexigen-associated pulmonary arterial hypertension) were diagnosed, giving rise to an estimated incidence of 1.1 cases per million per year and prevalence of 6.6 cases per million in 2009. Younger patients (age ≤ 50 yrs) had shorter duration of symptoms, fewer comorbidities, better functional and exercise capacity, higher percent diffusing capacity of carbon monoxide, more severe hemodynamic impairment, but better survival compared with older patients. In comparison with the earlier cohorts, patients diagnosed in 2007-2009 were older, more obese, had lower percent diffusing capacity of carbon monoxide,(,) and more comorbidities, but better survival. Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) equation, REVEAL risk score, and Pulmonary Hypertension Connection Registry survival equation accurately predicted survival of our incident cohort at 1 year.
This study highlights the influence of age on phenotypes of incident pulmonary arterial hypertension and has shown the changes in demographics and epidemiology over the past decade in a national setting. The results suggest that there may be two subtypes of patients: the younger subtype with more severe hemodynamic impairment but better survival, compared with the older subtype who has more comorbidities.
在大多数肺动脉高压登记处，偶发性肺动脉高压代表性不足，其疾病特征可能与流行疾病不同。
确定特发性、遗传性和食欲抑制剂相关性肺动脉高压的纯偶发性、未经治疗的新发病例队列的特征和结局，并确定过去十年在英国和爱尔兰的临床表现和生存率的变化。
前瞻性地确定 2001 年至 2009 年期间所有新诊断的连续患者。
共诊断出 482 例患者（93%为特发性，5%为遗传性，2%为食欲抑制剂相关性肺动脉高压），估计每年每百万人口中有 1.1 例发病，2009 年每百万人口中有 6.6 例发病。年轻患者（年龄≤50 岁）的症状持续时间较短，合并症较少，功能和运动能力较好，一氧化碳弥散量百分比较高，血流动力学损害更严重，但生存时间较长。与早期队列相比，2007-2009 年诊断的患者年龄较大，更肥胖，一氧化碳弥散量百分比较低，合并症更多，但生存时间更长。登记评估早期和长期肺动脉高压疾病管理（REVEAL）方程、REVEAL 风险评分和肺动脉高压连接登记处生存方程准确预测了我们的偶发性队列 1 年的生存率。
本研究强调了年龄对偶发性肺动脉高压表型的影响，并展示了过去十年在国家范围内的人口统计学和流行病学变化。结果表明，可能存在两种患者亚型：年轻亚型血流动力学损害更严重但生存时间更长，而年龄较大的亚型合并症更多。
