儿童重度特发性肺动脉高压晕厥相关危险因素分析

Risk factors for syncope in children with severe idiopathic pulmonary arterial hypertension

目的探讨儿童重度特发性肺动脉高压(IPAH)患儿发生晕厥的相关危险因素。方法回顾性分析2011年5月至2021年10月在北京安贞医院小儿心脏中心确诊为重度IPAH的44例患儿(年龄<18岁)的临床资料,根据患儿是否出现晕厥分为晕厥组和非晕厥组,比较两组患儿的临床特征、超声心动图、实验室检查、右心导管检查及急性肺血管扩张试验等指标,组间比较采用t检验、Mann-Whitney U检验或χ^(2)检验,组间差异有统计学意义的指标采用Logistic回归计算OR,分析其相关性。结果44例患儿中男16例、女28例;IPAH发病年龄(7.2±3.9)岁;非晕厥组患儿20例,晕厥组患儿24例(55%),晕厥发作次数1~11次,其中18例(75%)为运动诱发。晕厥组纽约心脏病协会(NYHA)心功能Ⅲ~Ⅳ级的患儿比例[67%(16/24)比25%(5/20),χ^(2)=7.59,P=0.006]、脑钠肽[(251±39)比(61±40)pg/L,t=-2.18,P=0.035]、肺动脉内径/主动脉内径比值(1.6±0.4比1.4±0.2,t=-2.25,P=0.030)、肺血管阻力指数[(22±11)比(16±7)WU/m^(2),t=-2.13,P=0.039]明显高于非晕厥组,晕厥组存在卵圆孔未闭的患儿比例[4%(1/24)比45%(9/20),χ^(2)=10.36,P=0.001]、左心室射血分数(LVEF)[(68±5)%比(72±8)%,t=2.23,P=0.031]、急性肺血管扩张试验阳性例数[8%(2/24)比35%(7/20),χ^(2)=4.77,P=0.029]明显低于非晕厥组。Logistic回归分析显示NYHA心功能Ⅲ~Ⅳ级(OR=6.787,95%CI 1.445~31.880)、肺血管阻力指数(OR=1.247,95%CI 1.020~1.525)、脑钠肽(OR=1.036,95%CI 1.007~1.066)是IPAH患儿晕厥发生的独立危险因素,卵圆孔未闭(OR=0.010,95%CI 0.000~0.586)为IPAH患儿晕厥发生的保护性因素。结论NYHA心功能分级、肺血管阻力指数、脑钠肽是IPAH患儿发生晕厥的危险因素,卵圆孔未闭是保护性因素;运动是IPAH患儿发生晕厥的主要诱因。

Objective To explore the risk factors for syncope in children with severe idiopathic pulmonary arterial hypertension(IPAH).Methods Forty-four patients(age<18 years)with IPAH admitted to the Department of Pediatric Cardiology,Beijing Anzhen Hospital between May 2011 and October 2021 were retrospectively included.Patients were devided into syncope group and non-syncope group.Clinical manifestation and hemodynamic parameters including echocardiography,blood tests,right heart catheterization and acute pulmonary vascular expansion test were compared between two groups.Comparisons between groups were performed with unpaired Student t test,or Mann-Whitney U test or chi-square test.Logistic regression was used to calculate the odds ratio(OR)for parameters with statistically significant differences between groups and analyze the statistical correlation.Results Among the 44 patients,16 were males,the onset age was(7.2±3.9)years.Twenty-four(55%)children presented with 1 to 11 times of episodes of syncope,and 18 cases of whom induced by physical activity.Syncope group had a larger proportion of New York Heart Association(NYHA)heart function classⅢ-Ⅳ(67%(16/24)vs.25%(5/20),χ^(2)=7.59,P=0.006),higher brain natriuretic peptide(BNP)value((251±39)vs.(61±40)pg/L,t=-2.18,P=0.035),higher pulmonary-to-aorta diameter ratio(1.6±0.4 vs.1.4±0.2,t=-2.25,P=0.030)and larger pulmonary vascular resistance index((22±11)vs.(16±7)WU/m^(2),t=-2.13,P=0.039)compared with non-syncope group.The proportion of patent foramen ovale(4%(1/24)vs.45%(9/20),χ^(2)=10.36,P=0.001),left ventricular ejection fraction(LVEF)((68±5)%vs.(72±8)%,t=2.23,P=0.031)and the positive rate of acute pulmonary vascular expansion test(8%(2/24)vs.35%(7/20),χ^(2)=4.77,P=0.029)of syncope group were significantly lower than those of non-syncope group.Multiple Logistic regression analysis showed that NYHA heart functionⅢ-Ⅳ(OR=6.787,95%CI 1.445-31.880),pulmonary vascular resistance index(OR=1.247,95%CI 1.020-1.525)and BNP(OR=1.036,95%CI 1.007-1.066)were independent risk factors for syncope.The patent foramen ovale(OR=0.010,95%CI 0.000-0.586)was a protective factor for syncope.Conclusions NYHA cardiac function grade,pulmonary vascular resistance index and BNP are independent risk factors for syncope.Patent foramen ovale is a protective factor for syncope.Exercise is the main inducement of syncope in children with IPAH.