Predictors of poor outcome in patients with pulmonary arterial hypertension: A single center study.
Predictors of poor outcome in patients with pulmonary arterial hypertension: A single center study.
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DOI：
10.1371/journal.pone.0193245
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发表时间：
2018
期刊：
影响因子：
3.7
通讯作者：
Raczak G
中科院分区：
文献类型：
--
作者：
Stepnowska E;Lewicka E;Dąbrowska-Kugacka A;Daniłowicz-Szymanowicz L;Zagożdżon P;Kamiński R;Lewicka-Potocka Z;Miękus P;Kozłowski D;Potocki W;Raczak G
肺动脉高压（PAH）是一种罕见的疾病，尽管实施了针对PAH的特定治疗，但预后不良。该研究的目的是确定根据波兰国家卫生基金计划治疗的一个中心的患者预后不良的预测因素。47例确诊为PAH的连续患者（30例女性;年龄39±17岁）入组本研究。常规访视时进行临床评估、实验室测量、心电图、超声心动图、6分钟步行试验、24小时霍尔特监测、心肺运动试验和微伏T波电交替试验。8例患者在2.6±1.7年随访期间死亡。以脑钠肽（BNP）浓度≥330 pg/mL作为区分死亡的指标（灵敏度88%，特异性92%，ROC曲线下面积[AUC] 0.92）;胆红素浓度≥1.2 mg/dL（敏感性88%，特异性81%，AUC 0.85）;右心房面积≥21 cm 2（灵敏度86%，特异性69%，AUC 0.84），心尖四腔视图中的右心室（RV）尺寸≥47 mm（敏感性86%，特异性86%，AUC 0.85），右室与左室舒张期内径比值≥1.5（敏感性83%，特异性84%，AUC 0.85）。在多变量分析中，死亡率的独立预测因素是BNP（p = 0.04）和胆红素水平（p = 0.03）较高，右心房面积较高（p = 0.02）和三尖瓣环平面收缩期偏移较低（p = 0.03）。在接受特定PAH导向治疗的PAH患者中，右心房扩大、右心室收缩功能受损以及BNP和胆红素浓度升高与死亡风险增加相关。
Pulmonary arterial hypertension (PAH) is a rare disorder with unfavorable prognosis despite implementation of specific PAH-oriented therapy. The aim of the study was to define predictors of poor prognosis in patients from one center treated according to the Polish National Health Fund program. Forty-seven consecutive patients (30 women; aged 39±17 years) with PAH diagnosis were enrolled to the study. Clinical assessment, laboratory measurements, electrocardiogram, echocardiography, 6-minute walk test, 24-hour Holter monitoring, cardiopulmonary exercise test and microvolt T-wave alternans test were performed during routine visits. Eight patients died during 2.6±1.7 years follow-up. Parametrs which differentiated patients who died were brain natriuretic peptide (BNP) concentration ≥330 pg/mL (sensitivity 88%, specificity 92%, area under the ROC curve [AUC] 0.92); bilirubin concentration ≥1.2 mg/dL (sensitivity 88%, specificity 81%, AUC 0.85); right atrial area ≥21 cm2 (sensitivity 86%, specificity 69%, AUC 0.84), right ventricular (RV) dimension in the apical 4-chamber view ≥47 mm (sensitivity 86%, specificity 86%, AUC 0.85) and RV to left ventricular diastolic diameter ratio ≥1.5 (sensitivity 83%, specificity 84%; AUC 0.85). In multivariate analysis, independent predictors of mortality were higher BNP (p = 0.04) and bilirubin level (p = 0.03), higher right atrial area (p = 0.02) and lower tricuspid annular plane systolic excursion (p = 0.03). In PAH patients treated with specific PAH-oriented therapy right atrial enlargement, impaired right ventricular systolic function, as well as increased BNP and bilirubin concentration was associated with an increased mortality risk.
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10.1016/j.echo.2006.01.014
发表时间：
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影响因子：
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