摘要目的 调查海拔2000 m以上地区先天性心脏病(CHD)并发肺动脉高压(PAH)的发生率及相关因素.方法 回顾性分析了我院2007年1月至2009年12月间收治的不同海拔高度的1178例CHD患者中肺动脉高压发病情况,分析了CHD-PAH与年龄、海拔高度、病种及与民族间的关系.结果 CHD患者共1178例年龄1～69岁.伴有PAH者688例(58.4％),不伴PAH者490.(41.6％).男性436例,其中239例有PAH(54.8％),女性742例,449例有PAH(60.5％),差异无统计学意义(x2=3.667,P=0.058).海拔＜2500 m平均肺动脉收缩压(PASP)为(58±18) mm Hg,海拔2500～3500 m为(63±17)mm Hg,海拔＞3500 m为(64±19) mm Hg.随着海拔的升高CHD-PAH的比例也增加(分别为55.2％、57.2％和68.7％),差异有统计学意义(x2=10.507,P=0.05).CHD-PAH在各年龄段的构成因病种的不同而不同,各民族间CHD-PAH发生率差异无统计学意义(x2=5.404,P=0.248).结论 高原地区CHD-PAH者明显高于平原地区,低氧环境促进了CHD-PAH的发生和发展.

abstractsObjective To explore the incidence of congenital heart disease (CHD) complicated with pulmonary arterial hypertension (PAH) at an altitude of over 2000 meters above sea level.Methods We retrospectively analyzed the profiles of 688 PAH patients selected from 1178 CHD patients at our hospital during January 2007 to December 2009.The risk factors of CHD-PAH were analyzed by age,altitude,etiologies and nationalities.Results Among 1178 CHD patients,688 (58.4％ ) suffered PAH.And 239 PAH cases(54.8％ )were found in 436 male patients and 449 PAH cases (60.5％) in 742 female patients (x2=3.667,P=0.058 ).The difference was not significant.The incidences of PAH in CHD patients varied at different altitudes ( x2 =10.507,P=0.005 ).And 55.2％,57.2％ and 68.7％ were found in ＜2500 m,2500-3500 m and＞3500 m groups respectively.And the ＞ 3500 m group was statistically higher than those of the ＜ 2500 m and 2500 - 3500 m groups ( x2=9.924,P =0.005 ) ( x2 =8.007,P =0.005 ).The mean pulmonary arterial systolic pressure (PASP) of ＜2500 m group was (58 ± 18) mm Hg,2500-3500 m was (63 ± 17 ) mm Hg and ＞ 3500 m was (64 ± 19 )mm Hg.There were significant differences among them.In age composition of CHD-PAH,the highest ratio appeared before the age of 18 years old for PDA (patent ductus arteriosus)-PAH and VSD (ventricular septal defect)-PAH while that of ASD-PAH at the ages of 30-39 years old.There were not statistical differences among all nationalities( x2 =5.404,P =0.248).Conclusion The incidence of CHD-PAH at a high altitude is significantly higher than those at a low altitude.Hypoxia may promote the occurrence and development of PAH in CHD patients.