Adult Congenital Heart Disease Centre and, National Centre for Pulmonary Hypertension, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK.
Heart. 2012 May;98(9):736-42. doi: 10.1136/heartjnl-2011-301522. Epub 2012 Mar 7.
To assess the relationship between elevated levels of B-type natriuretic peptide (BNP) and outcome in patients with Eisenmenger syndrome.
Retrospective study.
Tertiary centre for adult congenital heart disease.
All patients with Eisenmenger syndrome (n=181, age 36.9±12.1 years, 31% with Down syndrome) in whom BNP concentrations were measured as part of routine clinical care were included.
The study end point was all cause mortality.
During a median follow-up period of 3.3 years, 20 patients (7 with Down syndrome) died. Higher BNP concentrations were predictive of all cause mortality on univariate analysis in patients with or without Down syndrome. On multivariable Cox proportional hazard analysis, BNP predicted survival independently of renal function, Down syndrome, or 6 min walk test distance (p=0.004). Temporal increases in BNP concentration were also found to predict mortality. Treatment with disease targeting therapies was associated with a significant reduction in BNP concentrations.
BNP concentrations predict outcome in contemporary Eisenmenger patients. Increases in BNP concentrations over time are also of prognostic significance. In addition, disease targeting therapies may help to reduce BNP concentrations in this population, while treatment-naïve patients have static or rising BNP concentrations.
评估 B 型利钠肽（BNP）水平升高与艾森曼格综合征患者结局的关系。
回顾性研究。
成人先天性心脏病的三级中心。
所有接受 BNP 浓度测量作为常规临床护理一部分的艾森曼格综合征患者（n=181，年龄 36.9±12.1 岁，31%伴有唐氏综合征）均被纳入研究。
研究终点为全因死亡率。
在中位随访 3.3 年期间，20 名患者（7 名伴有唐氏综合征）死亡。在伴有或不伴有唐氏综合征的患者中，BNP 浓度在单因素分析中可预测全因死亡率。多变量 Cox 比例风险分析显示，BNP 可独立于肾功能、唐氏综合征或 6 分钟步行试验距离预测生存率（p=0.004）。BNP 浓度的时间性升高也被发现与死亡率相关。针对疾病的治疗与 BNP 浓度的显著降低相关。
BNP 浓度可预测当代艾森曼格综合征患者的结局。随着时间的推移，BNP 浓度的增加也具有预后意义。此外，针对疾病的治疗可能有助于降低该人群中的 BNP 浓度，而未接受治疗的患者的 BNP 浓度则保持稳定或升高。
