Pulmonary Department, Heart Institute, University of São Paulo Medical School, São Paulo, Brazil.
J Am Coll Cardiol. 2010 Aug 24;56(9):715-20. doi: 10.1016/j.jacc.2010.03.065.
The objective of this study was to evaluate the natural history of untreated schistosomiasis-associated pulmonary arterial hypertension (Sch-PAH) patients as compared to idiopathic pulmonary arterial hypertension (IPAH) with respect to hemodynamics recorded at presentation and 36 months survival.
Schistosomiasis (Sch) is one of the most prevalent chronic infectious diseases in the world. Nevertheless data regarding one of its most severe clinical complications, pulmonary arterial hypertension (PAH), is scarce.
We retrospectively analyzed case notes of all consecutive patients diagnosed of Sch-PAH and IPAH referred to the Heart Institute in São Paulo, Brazil, between 2004 and 2008. None of the Sch-PAH received PAH specific treatment whereas all IPAH patients did.
Sch-PH patients (n = 54) had less severe pulmonary hypertension as evidenced by lower levels of pulmonary vascular resistance (11.3 +/- 11.3 W vs. 16.7 +/- 10.6 W; p = 0.002) and mean pulmonary artery pressure (56.7 +/- 18.7 mm Hg vs. 64.6 +/- 17.4 mm Hg; p = 0.01) and higher cardiac output (4.62 +/- 1.5 l/min vs. 3.87 +/- 1.5 l/min; p = 0.009) at presentation than IPAH patients (n = 95). None of the Sch-PAH patients demonstrated a positive response to acute vasodilator testing, whereas 16.2% of IPAH patients did (p = 0.015). Survival rates at 1, 2, and 3 years were 95.1%, 95.1%, and 85.9% and 95%, 86%, and 82%, for Sch-PAH and IPAH, respectively (p = 0.49). Both groups had a higher survival rate when compared to IPAH survival as estimated by the NIH equation (71%, 61%, and 52%, respectively).
Sch-PAH has a more benign clinical course than IPAH despite a lack of demonstrable acute vasoreactivity at hemodynamic evaluation.
本研究旨在评估未经治疗的血吸虫病相关肺动脉高压（Sch-PAH）患者与特发性肺动脉高压（IPAH）患者在就诊时的血流动力学记录和 36 个月生存率方面的自然病史。
血吸虫病（Sch）是世界上最普遍的慢性传染病之一。然而，关于其最严重的临床并发症之一，肺动脉高压（PAH）的数据却很少。
我们回顾性分析了 2004 年至 2008 年期间在巴西圣保罗心脏研究所连续诊断为 Sch-PAH 和 IPAH 的所有患者的病历。没有一名 Sch-PAH 患者接受 PAH 特异性治疗，而所有 IPAH 患者均接受了治疗。
Sch-PH 患者（n=54）的肺动脉高压程度较轻，表现为较低的肺血管阻力（11.3+/-11.3 W 与 16.7+/-10.6 W；p=0.002）和平均肺动脉压（56.7+/-18.7 mm Hg 与 64.6+/-17.4 mm Hg；p=0.01）和较高的心输出量（4.62+/-1.5 l/min 与 3.87+/-1.5 l/min；p=0.009）。没有一名 Sch-PAH 患者对急性血管扩张剂测试有阳性反应，而 IPAH 患者中有 16.2%（p=0.015）有阳性反应。Sch-PAH 和 IPAH 患者的 1、2 和 3 年生存率分别为 95.1%、95.1%和 85.9%和 95%、86%和 82%（p=0.49）。与 NIH 方程估计的 IPAH 生存率相比，两组的生存率都更高（分别为 71%、61%和 52%）。
尽管在血流动力学评估中未显示出明显的急性血管反应性，但 Sch-PAH 的临床病程比 IPAH 更为良性。
