2017 年第 12 期 第 12 卷

先天性心脏病术后肺动脉高压与特发性肺动脉高压患者的临床特点与预后

Clinical characteristics and prognosis of pulmonary artery hypertension secondary to congenital heart disease and idiopathic pulmonary artery hypertension

作者：徐茁原李强强刘倩张陈张丹张红胜刘迎龙顾虹

英文作者：Xu Zhuoyuan Li Qiangqiang Liu Qian Zhang Chen Zhang Dan Zhang Hongsheng Liu Yinglong Gu Hong

单位：100029首都医科大学附属北京安贞医院小儿心脏中心北京市心肺血管疾病研究所（徐茁原、李强强、刘倩、张陈、张红胜、刘迎龙、顾虹）；100020北京，首都儿科研究所附属儿童医院小儿心内科（张丹）

英文单位：Pediatric Cardiac Center Beijing Anzhen Hospital Capital Medical University Beijing Institute of Heart Lung and Blood Vessel Diseases Beijing 100029 China(Xu ZY Li QQ Liu Q Zhang C Zhang HS Liu YL Gu H); Department of Pediatric Cardiology Children′s Hospital Capital Institute of Pediatrics Beijing 100020 China(Zhang D)

关键词：先天性心脏病；肺动脉高压；心脏修补术；特发性肺动脉高压

英文关键词：Congenitalheartdisease;Pulmonaryarteryhypertension;Cardiacdefectcorrection;Idiopathicpulmonaryarteryhypertension

• 摘要：

• 目的    分析先天性心脏病（CHD）术后肺动脉高压（PAH）与特发性肺动脉高压（IPAH）患者的临床表现、功能性指标、血液动力学特点及生存情况。方法    收集2010年1月至2016年12月于首都医科大学附属北京安贞医院小儿心脏中心住院的88例PAH患者的临床资料。超声心动图和/或行规范心导管术明确诊断PAH，包括60例IPAH及28例CHD术后PAH患者。回顾性分析这2种类型患者的临床特点和远期预后情况。结果    入组患者年龄0.8～50.2岁、平均（18±12）岁，中位随访时间为4.4年。超声估测及右心导管检查结果显示CHD术后PAH组与IPAH组患者肺动脉收缩压、肺动脉平均压及肺小动脉阻力指数差异均无统计学意义［（82±25）mmHg（1 mmHg=0.133 kPa）比（76±22）mmHg、（58±18）mmHg比（62±18）mmHg、（13±4）Wood/m2比（18±10）Wood/m2］（均P＞0.05）。CHD术后PAH组B型脑钠肽水平及右心室与左心室内径比值明显低于IPAH组［18.2(9.2,25.4)ng/L比681.0(53.0,728.0)ng/L、（0.53±0.18）比（0.87±0.41）］，差异均有统计学意义（均P<0.05）。IPAH患者在随访期间有16例（18.2%）死亡，36例（60.0%）出现相关并发症；CHD术后PAH患者无死亡，有5例（17.9%）出现并发症；CHD术后PAH组相应的并发症发生率均明显低于IPAH组，差异均有统计学意义（均P<0.05）。CHD术后PAH组生存率高于IPAH组［100.0%（28/28）比73.3%（44/60）］，差异有统计学意义（P<0.05）。结论    CHD术后PAH患者与IPAH患者血流动力学指标类似，但心功能更稳定，右心室扩张程度低，预后也更好。

• Objective    To analyze clinical manifestations, functional indexes, hemodynamic features and survival condition in patients with idiopathic pulmonary artery hypertension(IPAH) and pulmonary artery hypertension(PAH) secondary to congenital heart disease(CHD). Methods    Clinical data of 88 patients with PAH who were hospitalized in Pediatric Cardiac Center of Beijing Anzhen Hospital, Capital Medical University from January 2010 to December 2016 were retrospectively reviewed, including 60 cases of IPAH and 28 cases of PAH secondary to CHD after cardiac defect correction. Clinical features and long-term prognosis of patients were analyzed. Results    The average age of 88 patients was （18±12）years old (0.8-50.2 years old). The median follow-up time was 4.4 years. Pulmonary artery systolic pressure, pulmonary artery mean pressure and pulmonary arteriolar resistance index estimated by echocardiography and cardiac catheterization showed no significant differences between PAH secondary to CHD group and IPAH group［（82±25）mmHg vs （76±22）mmHg, （58±18）mmHg vs （62±18）mmHg, （13±4）Wood/m2 vs （18±10）Wood/m2］（P＞0.05）. The level of B-type natriuretic peptide and the ratio of right ventricular internal diameter/left ventricular internal diameter in PAH secondary to CHD group were significantly lower than those in IPAH group［18.2(9.2,25.4)ng/L vs 681.0(53.0,728.0)ng/L, （0.53±0.18） vs （0.87±0.41）］（P<0.05）. In IPAH group, 16 patients（18.2%） died during follow-up period; 36 patients(60.0%) had cardiac complications. In PAH secondary to CHD group, no patient died and 5 cases(17.9%) had cardiac complications. The postoperative complication rate in PAH secondary to CHD group was significantly lower than that in IPAH group(P<0.05). The survival rate in PAH secondary to CHD group was significantly higher than that in IPAH group［100.0%（28/28） vs 73.3%（44/60）］（P<0.05）. Conclusion    Patients with PAH secondary to CHD have similar hemodynamic features but better heart function and prognosis compared to patients with IPAH.