Scognamiglio Giancarlo, Kempny Aleksander, Price Laura C, Alonso-Gonzalez Rafael, Marino Philip, Swan Lorna, D' Alto Michele, Hooper James, Gatzoulis Michael A, Dimopoulos Konstantinos, Wort Stephen J
Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK Department of Cardiology, Second University of Naples, Monaldi Hospital, Naples, Italy.
Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton Hospital, London, UK NIHR Cardiovascular Biomedical Research Unit, Royal Brompton Hospital and National Heart and Lung Institute, Imperial College London, London, UK Division of Adult Congenital and Valvular Heart Disease, Department of Cardiovascular Medicine, University Hospital Muenster, Muenster, Germany.
Heart. 2014 Sep;100(17):1335-41. doi: 10.1136/heartjnl-2014-305494. Epub 2014 May 1.
To assess the relationship of C-reactive protein (CRP) to clinical outcome and mortality in adults with pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD-PAH).
Approximately 5-10% of adults with congenital heart disease (ACHD) develop PAH, which in turn is associated with substantial morbidity and mortality. Although CRP is known to predict outcome in idiopathic PAH, little is known regarding its prognostic value in CHD-PAH.
We obtained and analysed 1936 CRP values in a total of 225 adults with CHD-PAH (median age at study entry 34.0 years (27.0-41.7); 32.9% male, 35% with Down syndrome), performed over a 12-year period. High CRP values related to infection or blood transfusions were excluded from the analysis.
During a median follow-up of 4.8 years (1149 patients-years), 50 patients died. The median CRP concentration on the last assessment was 5.0 mg/L (IQR 2.0-10.0), higher in deceased patients compared with survivors (11.5 mg/L (6.0-23.0) vs 4.0 mg/L (1.5-8.0), p<0.0001). Following univariate Cox regression analysis, CRP emerged as a strong predictor of mortality (HR 1.18; 95% CI 1.11 to 1.26, p<0.0001) and remained significant after adjustment for age, presence of Down syndrome and advanced PAH therapy. Survival-receiver-operator characteristic analysis identified an optimal cut-off value of 10 mg/L. Patients with CRP >10 mg/L had more than a threefold increased risk of death (HR 3.63, 95% CI 2.07 to 6.38, p<0.0001).
Serum CRP is a simple but powerful marker of mortality in CHD-PAH patients and should be incorporated in the risk stratification and routine assessment of these patients.
评估C反应蛋白（CRP）与先天性心脏病相关性肺动脉高压（CHD-PAH）成年患者临床结局及死亡率之间的关系。
约5%-10%的先天性心脏病成年患者（ACHD）会发生PAH，进而导致显著的发病率和死亡率。虽然已知CRP可预测特发性PAH的结局，但对于其在CHD-PAH中的预后价值却知之甚少。
我们获取并分析了12年间共225例CHD-PAH成年患者（研究入组时的中位年龄为34.0岁（27.0-41.7）；男性占32.9%，35%患有唐氏综合征）的1936个CRP值。分析中排除了与感染或输血相关的高CRP值。
在中位随访4.8年（1149患者-年）期间，50例患者死亡。末次评估时CRP的中位浓度为5.0mg/L（四分位间距2.0-10.0），死亡患者的CRP浓度高于存活患者（11.5mg/L（6.0-23.0） vs 4.0mg/L（1.5-8.0），p<0.0001）。单因素Cox回归分析显示，CRP是死亡率的强预测指标（风险比1.18；95%置信区间1.11至1.26，p<0.0001），在对年龄、唐氏综合征的存在情况及晚期PAH治疗进行校正后仍具有显著性。生存-接受者操作特征分析确定最佳临界值为10mg/L。CRP>10mg/L的患者死亡风险增加超过三倍（风险比3.63，95%置信区间2.07至6.38，p<0.0001）。
血清CRP是CHD-PAH患者死亡率的一个简单但有力的标志物，应纳入这些患者的风险分层和常规评估中。
