特发性肺动脉高压患者发生肺动脉高压危象的相关因素分析

Analysis of factors related to pulmonary hypertensive crisis in patients with idiopathic pulmonary arterial hypertension

目的：探讨特发性肺动脉高压（ IPAH）患者发生肺动脉高压危象（ PHC）的相关因素。方法回顾性分析2010年1月至2013年12月收住北京安贞医院小儿心脏科且确诊为IPAH的65例患者的临床资料，根据临床表现分为PHC易患组和非易患组，对可能与PHC相关的临床、超声心动图、心导管检查和急性肺血管扩张试验指标分别进行单因素和多因素分析。结果纳入的65例患者中，男32例，女33例；年龄10个月～47.3岁，平均（14.4±12.3）岁；有明显PHC表现者23例，其中18例为运动诱发。单因素分析显示PHC易患组世界卫生组织（ WHO）心功能Ⅲ～Ⅳ级的患者比例明显高于非易患组（60.9％比23.8％，P＝0.003），而PHC易患组存在卵圆孔未闭的患者比例显著低于非易患组（8.7％比45.2％，P＝0.003）。其中WHO心功能Ⅰ～Ⅱ级的患者中，PHC易患组急性肺血管扩张试验中肺动脉压下降值、试验阳性率等指标明显高于非易患组；WHO心功能Ⅲ～Ⅳ级的患者中，PHC易患组基础肺动脉压、右房平均压等指标明显高于非易患组。多因素分析显示WHO心功能Ⅲ～Ⅳ级（ OR＝23.45，95％CI：2.85～193.09）、肺血管扩张试验中肺动脉收缩压的下降值（OR＝1.12，95％CI：1.04～1.22）是PHC的独立危险因素，而卵圆孔未闭（OR＝0.01，95％CI：0.00～0.52）为PHC的保护因素。结论 IPAH患者发生PHC与WHO心功能分级、肺血管反应性、基础肺动脉压以及是否存在卵圆孔未闭有关。运动是IPAH患者发生PHC的主要诱因。

Objective To explore the risk and protective factors for pulmonary hypertensive crisis （ PHC） in patients with idiopathic pulmonary arterial hypertension （ IPAH ）.Methods A retrospective study was performed for 65 patients with a diagnosis of IPAH between January 2010 and December 2013.According to clinical manifestations , they were divided into two groups of susceptibility and non-susceptibility to PHC.Clinical and hemodynamic parameters were analyzed in univariate and multivariate manners.Results Among them,there were 32 males and 33 females with a mean age of （14.4±12.3） （10/12-47.3） years.Twenty-three patients had typical manifestations of PHC and 18 of them were induced by exercises.Univariate analysis revealed that the proportion of patients with World Health Organization （ WHO） functional class Ⅲ-Ⅳin PHC-susceptible group was significantly higher than PHC-nonsusceptible group （ 60.9% vs 23.8%, P =0.003 ） while the percentage of patent foramen ovale in PHC-susceptible group was significantly lower than PHC-nonsusceptible group （ 8.7% vs 45.2%, P=0.003 ）.In patients with WHO functional class Ⅰ-Ⅱ, hemodynamic variables including the decline of pulmonary arterial pressure and positive rate in vasoreactivity testing in PHC-susceptible group were significantly higher than PHC-nonsusceptible group.In patients with WHO functional class Ⅲ -Ⅳ, baseline pulmonary arterial pressure and mean right atrial pressure in PHC-susceptible group were significantly higher than those in PHC-nonsusceptible group.Multivariate Logistic regression analysis revealed that , for those with WHO functional class Ⅲ-Ⅳ （OR =23.45,95%CI： 2.85 -193.09） and the decline of systolic pulmonary arterial pressure in vasoreactivity testing （OR=1.12,95%CI：1.04-1.22） were independent risk factors for PHC in IPAH patients while patent foramen ovale （ OR =0.01, 95%CI： 0.00 -0.52 ） was a＆amp;nbsp;protective factor.Conclusion PHC in IPAH patients is correlated with WHO functional class ,pulmonary vascular reactivity,baseline pulmonary arterial pressure and patent foramen ovale.And exercise is the most common stimulus to PHC.