脾切除术后肺动脉高压的临床特征和靶向药物的疗效  

Clinical features of post-splenectomy pulmonary hypertension and effects of its target therapy

作　　者：黄丽[1] 杨涛[1] 李雯[1] 王晓彤[1] 熊长明 倪新海[1] 顾晴[1] 何建国[1] Huang Li;Yang Tao;Li Wen;Wang Xiaotong;Xiong Changming;Ni Xinhai;Gu Qing;He Jianguo(State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China)

机构地区：[1]中国医学科学院北京协和医学院国家心血管病中心阜外医院心血管疾病国家重点实验室,100037

出　　处：《中华医学杂志》2018年第22期1761-1765,共5页National Medical Journal of China

基　　金：国家重点研发计划（2016YFC1304400）

摘　　要：目的总结脾切除术后肺动脉高压的临床特征和靶向药物的疗效。方法系统整理2006年10月至2017年3月阜外医院住院收治的18例脾切除术后肺动脉高压患者的基本情况、临床表现、辅助检查和靶向药物治疗等信息，并进行分析。结果18例患者女11例，男7例，年龄（41±11）岁，脾切除术至诊断肺动脉高压间隔为（11±6）年，最常见症状为气短（14／18例）和胸闷（12／18例）,世界卫生组织（WHO）功能分级Ⅲ-Ⅳ者9例，合并肺栓塞2例。依据脾切除术病因分为肝硬化门脉高压组10例和非肝硬化门脉高压组8例。肝硬化门脉高压组患者伴慢性肝病表现，不合并肺栓塞，心输出量和心指数显著高于非肝硬化门脉高压组患者（P=0．007和P=0．011），肺血管阻力显著低于非肝硬化门脉高压组（P=0．013），两组肺动脉平均压和WHO功能分级无显著差别（P=0．448和P=0．379）。8例患者接受靶向药物治疗3个月后，3例WHO功能分级改善（P=0．102），超声心动图测得的肺动脉收缩压从（84±10）降至（71±12）mmHg（1mmHg=0．133kPa）（P=0．005），右室舒张末内径／左室舒张末内径从（0．78±0．17）降至（0．62±0．16）（P=0．010），血浆氨基酸末端脑钠素前体（NT-proBNP）呈下降趋势。结论脾切除术后肺动脉高压的临床特征因脾切除病因而异，肝硬化门脉高压是脾切除术的常见病因，此类肺动脉高压患者的血流动力学指标较好。初步提示靶向药物治疗脾切除术后肺动脉高压有效。Objective To analyze the clinical features of post-splenectomy pulmonary hypertension and effects of its target therapy, and improve the diagnosis and treatment of the disease. Methods Clinical data of 18 patients with post-splenectomy pulmonary hypertension admitted to Fuwai Hospital from October 2006 to March 2017 were systematically reviewed and analyzed. Results Among the 18 patients with a mean age of （41 ± 11 ） years old, 11 were women and 7 were men. The interval between splenectomy and the diagnosis of pulmonary hypertension was （ 11 ± 6） years. Shortness of breath （14/18） and chest distress （12/18） were the most common clinical manifestations. Nine patients were in World Health Organization functional class （WHO-FC） Ⅲ - Ⅳ, and two patients were combined with pulmonary embolism. According to the cause of splenectomy, the pulmonary hypertension patients were divided into hepatocirrhosis portal hypertension group （10/18） and non-hepatocirrhosis portal hypertension group （8/18）. In hepatocirrhosis portal hypertension group, patients were combined with chronic liver abnormalities presentation, and no pulmonary embolism was found in these patients. Cardiac output and cardiac index were significantly higher （P = O. 007 and O. 011, respectively ） and pulmonary vascular resistance was significantly lower （P = O. 013 ） in hepatocirrhosis portal hypertension group as compared with non-hepatocirrhosis portal hypertension group. There was no difference in WHO-FC and mean pulmonary arterial pressure between two groups （ P = O. 448 and 0. 379, respectively ） . After 3 months of target treatment in 8 patients, WHO-FC of 3 patients improved, systolic pulmonary artery measured by ultrasonic cardiogram was significantly decreased [ （ 84±10 ） vs （ 71 ± 12 ） mmHg （ 1 mmHg = 0. 133 kPa） , P = 0. 0051 and right ventricular end diastolic diameter/left ventricular end diastolic diameter was significantly reduced[ （0. 78 ±0. 17） vs （0. 62±0. 16）, P =0. 010] com

关 键 词：高血压 肺性 脾切除术 疾病特征 靶向治疗 

分 类 号：R544.1[医药卫生—心血管疾病]