Strange Geoff, Lau Edmund M, Giannoulatou Eleni, Corrigan Carolyn, Kotlyar Eugene, Kermeen Fiona, Williams Trevor, Celermajer David S, Dwyer Nathan, Whitford Helen, Wrobel Jeremy P, Feenstra John, Lavender Melanie, Whyte Kenneth, Collins Nicholas, Steele Peter, Proudman Susanna, Thakkar Vivek, Keating Dominic, Keogh Anne
School of Medicine, University of Notre Dame, Perth, WA, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia; Pulmonary Hypertension Society of Australia and New Zealand, Sydney, NSW, Australia.
Sydney Medical School, University of Sydney and Royal Prince Alfred Hospital, Sydney, NSW, Australia.
Heart Lung Circ. 2018 Nov;27(11):1368-1375. doi: 10.1016/j.hlc.2017.08.018. Epub 2017 Sep 20.
Epidemiology and treatment strategies continue to evolve in pulmonary arterial hypertension (PAH). We sought to define the characteristics and survival of patients with idiopathic, heritable and drug-induced PAH in the current management era.
Consecutive cases of idiopathic, heritable and drug-induced PAH were prospectively enrolled into an Australian and New Zealand Registry.
Between January 2012 and December 2016, a total of 220 incident cases were enrolled (mean age 57.2±18.7years, female 69.5%) and followed for a median duration of 26 months (IQR17-39). Co-morbidities were common such as obesity (34.1%), systemic hypertension (30.5%), coronary artery disease (16.4%) and diabetes mellitus (19.5%). Initial combination therapy was used in 54 patients (dual, n=50; triple, n=4). Estimated survival rates at 1-year, 2-years and 3-years were 95.6% (CI 92.8-98.5%), 87.3% (CI 82.5-92.4%) and 77.0% (CI 70.3-84.3%), respectively. Multivariate analysis showed that male sex and lower 6-minute distance at diagnosis independently predicted worse survival, whereas obesity was associated with improved survival. Co-morbidities other than obesity did not impact survival. Initial dual oral combination therapy was associated with a trend towards better survival compared with initial oral monotherapy (adjusted HR=0.27, CI 0.06-1.18, p=0.082) CONCLUSIONS: The epidemiology and survival of patients with idiopathic PAH in Australia and New Zealand are similar to contemporary registries reported in Europe and North America. Male sex and poorer exercise capacity are predictive of mortality whereas obesity appears to exert a protective effect. Despite current therapies, PAH remains a life-threatening disease associated with significant early mortality.
肺动脉高压（PAH）的流行病学和治疗策略不断发展。我们试图明确在当前治疗时代特发性、遗传性和药物性PAH患者的特征及生存率。
将特发性、遗传性和药物性PAH的连续病例前瞻性纳入澳大利亚和新西兰注册研究。
2012年1月至2016年12月，共纳入220例新发病例（平均年龄57.2±18.7岁，女性占69.5%），中位随访时间为26个月（四分位间距17 - 39个月）。合并症常见，如肥胖（34.1%）、系统性高血压（30.5%）、冠状动脉疾病（16.4%）和糖尿病（19.5%）。54例患者采用初始联合治疗（双联治疗，n = 50；三联治疗，n = 4）。1年、2年和3年的估计生存率分别为95.6%（92.8 - 98.5%）、87.3%（82.5 - 92.4%）和77.0%（70.3 - 84.3%）。多因素分析显示，男性及诊断时6分钟步行距离较短独立预测生存率较差，而肥胖与生存率改善相关。除肥胖外的合并症不影响生存率。与初始口服单药治疗相比，初始双联口服联合治疗有生存改善的趋势（校正风险比=0.27，9 = 0.06 - 1.18，P = 0.082）。结论：澳大利亚和新西兰特发性PAH患者的流行病学和生存率与欧洲和北美的当代注册研究相似。男性及运动能力较差可预测死亡率，而肥胖似乎具有保护作用。尽管有当前的治疗方法，PAH仍然是一种与早期高死亡率相关的危及生命的疾病。
