先天性心脏病修复术后肺动脉高压患者基线临床特征分析  

Pulmonary arterial hypertension after operation for congenital heart disease： analysis of baseline clinical characteristics of 122 Chinese patients

作　　者：孙明利[1] 程春燕[1] 张锐[1] 徐希奇[2] 温亮[3] 孙凯[2] 蒋鑫[2] 王晓建[2] 荆志成[2] 

机构地区：[1]同济大学医学院附属上海市肺科医院心肺循环中心,上海200433 [2]中国医学科学院北京协和医学院阜外心血管病医院血栓性疾病诊治中心 [3]汉中市中心医院心血管内科

出　　处：《中华心血管病杂志》2014年第5期396-399,共4页Chinese Journal of Cardiology

摘　　要：目的 探讨先天性心脏病修复术后肺动脉高压（pulmonary arterial hypertension,PAH）患者的基线临床特征.方法 回顾性分析上海市肺科医院心肺血管中心2006年9月至2011年8月连续确诊的122例先天性心脏病修复术后PAH患者的基线临床资料.结果 122例先天性心脏病修复术后PAH患者中,女性82例（67.2％）,修补/封堵术时年龄中位数14.8（7.1,24.9）岁,术后诊断PAH年龄中位数24.8（16.6,32.2）岁,从修复术至诊断PAH时间间隔中位数7.3 （2.4,12.6）年.临床症状以劳力性呼吸困难（109/122,89.3％）为主,体征以肺动脉瓣区第二心音亢进（113/122,92.6％）为主.确诊时符合世界健康组织肺高压功能Ⅰ/Ⅱ级的患者稍多（65/122,53.3％）.6 min步行距离为（408.4±103.3） m.Borg呼吸困难评分为2.0（1.0,3.0）.肺动脉平均压（71.6±24.2）mmHg（1 mmHg =0.133 kPa）,肺血管阻力指数（29.1±16.0） Wood U· m2,心指数（2.7±0.9） L·min-1·m-2.靶向PAH药物治疗率90.2％（110/122）,以5型磷酸二酯酶抑制剂为主.结论 先天性心脏病修复术后,部分患者发生PAH.与欧美发达国家相比,本研究中先天性心脏病修复术后PAH患者手术年龄大,术后发生PAH时间短,诊断PAH年龄轻,靶向PAH药物治疗率相当,以5型磷酸二酯酶抑制剂为主.Objective To explore the baseline clinical characteristics of patients with pulmonary arterial hypertension after operation for congenital heart disease （AO-CHD-PAH） in China.Methods A total of 122 consecutive patients diagnosed as AO-CHD-PAH in Shanghai Pulmonary Hospital from September 1,2006 to August 31,2011 were retrospective analyzed.Results Sixty-seven percent patients were female.The median age at procedure and diagnosis were 14.8 and 24.8 years old,respectively.The median duration from procedure of CHD to diagnosis of PAH was 7.3 years.The most frequent initial symptom （109/122,89.3％） attributed to PAH was dyspnea on exertion.The frequent sign was accentuation of second heart sound on pulmonary valve area （113/122,92.6％）.Incidence of WHO functional PAH classes Ⅰ / Ⅱ was 53.3％ （65/122） and mean 6 minutes walk distance was limited to （408.4 ± 103.3） m.Borg dyspnea score was 2.0 （1.0,3.0）.Right heart catheterization demonstrated severe elevated mean pulmonary arterial pressure[（71.6 ±24.2） mmHg,1 mmHg =0.133 kPa] and pulmonary vascular resistance index [（29.1 ±16.0） Wood U·m2]in this patient cohort.Cardiac index was （2.7 ±0.9） L·min-1·m-2,90.2％ （110/122） patients received PAH-specific therapy.The majority of PAH-specific therapy was phosphodiesterase type 5 inhibitor,which is somehow different from PAH-specific therapy regimen of American-European developed countries.Conclusions PAH-specific therapy rate is satisfactory for pulmonary arterial hypertension after operation for congenital heart disease in China and phosphodiesterase type 5 inhibitors are predominant PAH-specific medication in China.

关 键 词：心脏缺损 先天性 高血压 肺性 疾病特征 

分 类 号：R654.2[医药卫生—外科学]