Carrington Melinda, Murphy Niamh F, Strange Geoff, Peacock Andrew, McMurray John J V, Stewart Simon
Baker Heart Research Institute, Melbourne, Victoria, Australia.
Int J Cardiol. 2008 Feb 29;124(2):183-7. doi: 10.1016/j.ijcard.2006.12.045. Epub 2007 Apr 11.
Although pulmonary arterial hypertension (PAH) is widely accepted as deadly, if not a rare disease, its prognostic impact beyond reports from specialist centres is unknown.
Using the unique Scottish Morbidity Record Scheme and linked survival data, we tracked the survival of all Scottish adults aged < or =65 years admitted for the first time during the period of 1986 to 2001 with a probable diagnosis of Idiopathic PAH and a PAH related to connective tissue disorders (Connective PAH) and congenital abnormalities (Congenital PAH) - the three most common forms of PAH.
Overall, 374 Scottish men and women were discharged from the hospital with incident PAH during the period 1986 to 2001. On an unadjusted basis, Congenital PAH (40-45%) was associated with the lowest case fatality at 5 years in both men and women. In both sexes, Idiopathic PAH and Connective PAH were associated with high initial one-year case fatality (20-30%) with a steady accumulation of fatal events in the four years thereafter (60-75% case fatality at 5 years). Overall, the adjusted risk of dying within one year in the period 1986 to 1989 was 2.22-fold greater (OR 95% CI, 1.27 to 3.85) than in 1998 to 2001 (P<0.001). The greatest falls in one year case fatality were seen in those with Connective PAH (18-fold increased risk of dying in 1986 to 1989 versus 1998 to 2001: P=0.013). Similarly, women (adjusted OR 1.38, 95% CI 1.16 to 1.63: P<0.001) and the most deprived individuals (OR 2.38, 95% CI 1.17 to 4.82: P<0.05) were at greater risk of dying within 5 years. Alternatively, those patients discharged in 1997 were less likely to die during this period compared to their 1986 counterparts, although this difference did not quite reach statistical significance (OR 0.45, 95% CI 0.22 to 1.06: P=0.056).
This population-based study has confirmed the deadly impact of the three most common forms of PAH. Overall, there are encouraging trends in relation to one and five year adjusted survival rates; particularly in relation to PAH related to connective tissue disorders.
尽管肺动脉高压（PAH）即便不算罕见疾病，但被广泛认为是致命的，然而其在专科中心报告之外的预后影响尚不清楚。
利用独特的苏格兰发病率记录计划及相关生存数据，我们追踪了1986年至2001年期间首次入院的所有年龄小于或等于65岁的苏格兰成年人，这些人可能被诊断为特发性PAH、与结缔组织疾病相关的PAH（结缔组织PAH）以及先天性异常相关的PAH（先天性PAH）——PAH的三种最常见形式。
总体而言，1986年至2001年期间，374名苏格兰男性和女性因新发PAH出院。未经调整的情况下，先天性PAH（40 - 45%）在男性和女性中5年病死率最低。在两性中，特发性PAH和结缔组织PAH在最初一年病死率较高（20 - 30%），此后四年致命事件稳步累积（5年病死率60 - 75%）。总体而言，1986年至1989年期间一年内死亡的调整风险比1998年至2001年高2.22倍（OR 95% CI，1.27至3.85）（P<0.001）。结缔组织PAH患者一年内病死率下降幅度最大（1986年至1989年死亡风险是1998年至2001年的18倍：P = 0.013）。同样，女性（调整后OR 1.38，95% CI 1.16至1.63：P<0.001）和最贫困个体（OR 2.38，95% CI 1.17至4.82：P<0.05）在5年内死亡风险更高。另外，1997年出院的患者在此期间死亡可能性低于1986年出院的患者，尽管这种差异未达到统计学显著性（OR 0.45，95% CI 0.22至1.06：P = 0.056）。
这项基于人群的研究证实了PAH三种最常见形式的致命影响。总体而言，在一年和五年调整生存率方面有令人鼓舞的趋势；特别是与结缔组织疾病相关的PAH。
