Giusca Sorin, Jurcut Ruxandra, Coman Ioan Mircea, Ghiorghiu Ioana, Catrina Daniela, Popescu Bogdan A, Dima Laura, Ginghina Carmen
Cardiology Department, Institute for Emergencies in Cardiovascular Diseases C C Iliescu, Bucharest, Romania.
Echocardiography. 2013 Jan;30(1):17-26. doi: 10.1111/j.1540-8175.2012.01809.x. Epub 2012 Sep 18.
We followed patients with pulmonary arterial hypertension (PAH) receiving specific vasodilator therapy and tested for predictors of clinical outcome.
Thirty-two patients (mean age 39 ± 15 years, 22 women, diagnosed with pulmonary hypertension; PH): 29 with PAH and 3 patients with inoperable chronic thromboembolic PH received therapy with either bosentan, sildenafil, or both and were evaluated with clinical parameters, biomarkers (B-type natriuretic peptide values), and echocardiography before receiving specific medication and every 3 months thereafter. A right heart catheterization was performed at baseline. A composite endpoint of death, worsening of functional class, or the need of a second vasodilator agent was used to define the clinical nonresponders.
Patients were followed for 14 months (7.5-21). The endpoint was reached by 15 patients: four patients died (two idiopathic PAH and two PAH in context of Eisenmenger syndrome), seven patients showed 1 functional class worsening, and four patients needed to be switched to combination therapy. Patients who remained clinically stable or improved had at baseline a better cardiac output with a less remodeled right ventricle (RV) and better functioning RV (all P < 0.05). A RV fractional area change (RVFAC) lower than 25.7% and a RV global strain value higher than -13.4% predict with 87% sensitivity and 83% specificity (AUC 87.3%, P = 0.001) and 73% sensitivity and 91% specificity (AUC 84.2%, P = 0.003), respectively, patients who will deteriorate clinically under specific vasodilator therapy. A multivariate model showed RVFAC to be the only independent predictor of the endpoint with a HR of 0.87 (0.8-0.96), P = 0.007.
Over an average period of 1 year, almost half of patients showed signs of clinical deterioration despite specific vasodilator therapy. Parameters of right ventricular morphology and function had prognostic value in these patients.
我们对接受特定血管扩张剂治疗的肺动脉高压（PAH）患者进行了随访，并测试了临床结局的预测因素。
32例患者（平均年龄39±15岁，22例女性，诊断为肺动脉高压；PH）：29例PAH患者和3例无法手术的慢性血栓栓塞性PH患者接受波生坦、西地那非或两者联合治疗，并在接受特定药物治疗前及之后每3个月进行临床参数、生物标志物（B型利钠肽值）和超声心动图评估。在基线时进行右心导管检查。使用死亡、功能分级恶化或需要第二种血管扩张剂的复合终点来定义临床无反应者。
患者随访14个月（7.5 - 21个月）。15例患者达到终点：4例患者死亡（2例特发性PAH和2例艾森曼格综合征相关PAH），7例患者功能分级恶化1级，4例患者需要改用联合治疗。临床保持稳定或改善的患者在基线时心输出量更好，右心室（RV）重塑程度更低且功能更好（所有P < 0.05）。RV面积变化分数（RVFAC）低于25.7%和RV整体应变值高于 - 13.4%分别以87%的敏感性和83%的特异性（AUC 87.3%，P = 0.001）以及73%的敏感性和91%的特异性（AUC 84.2%，P = 0.003）预测在特定血管扩张剂治疗下临床会恶化的患者。多变量模型显示RVFAC是终点的唯一独立预测因素，HR为0.87（0.8 - 0.96），P = 0.007。
在平均1年的时间里，尽管接受了特定的血管扩张剂治疗，但几乎一半的患者出现了临床恶化迹象。右心室形态和功能参数对这些患者具有预后价值。
