目的:探讨右心发育不良综合征（HRHS）胎儿的心脏介入治疗及围手术期管理。方法:收集2018年7月至2019年6月青岛市妇女儿童医院行胎儿肺动脉瓣成形术（FPV）介入治疗的13例HRHS胎儿的围手术期临床资料进行回顾性分析，探讨FPV的术前评估、麻醉、手术治疗及结果、围手术期并发症以及HRHS胎儿的遗传学筛查结果。结果:（1）术前评估：13例HRHS胎儿的右心室相关指标，三尖瓣瓣环直径/二尖瓣瓣环直径比值、右心室长径/左心室长径比值、肺动脉瓣瓣环直径/主动脉瓣瓣环直径比值、三尖瓣流入时间/心动周期比值分别为0.81±0.04、0.56±0.14、0.69±0.06、0.35±0.03，与FPV的适应证（上述指标分别为≤0.83、≤0.64、≤0.75、<0.36）符合，可行FPV。（2）麻醉：采用舒芬太尼、丙泊酚联合七氟醚的静脉-吸入复合全身麻醉，13例HRHS胎儿的术前基线胎心率为（156±12）次/min，术中9例胎儿出现心动过缓，均予心内注射药物复苏治疗后恢复正常；孕妇术前血压与术中比较，收缩压［分别为（114.7±3.9）、（99.7±6.0） mmHg（1 mmHg=0.133 kPa）］和舒张压［分别为（78.7±5.6）、（63.8±3.2） mmHg］均显著下降（n P<0.01），但术前脉压差与术中脉压差［分别为（36.0±5.6）、（35.8±6.9） mmHg］比较无显著差异（n t=8.102，n P=0.951）。（3）手术治疗及结果：13例HRHS胎儿行FPV时的孕周为（27.3±0.8）周，均顺利完成FPV，手术时间为（23.2±1.0） min。10例继续妊娠的HRHS胎儿，术后6周评估右心室相关指标，三尖瓣瓣环直径/二尖瓣瓣环直径比值（n t=-2.513，n P=0.022）、右心室长径/左心室长径比值（n t=-3.373，n P=0.003）分别与术前比较，差异均有统计学意义。10例继续妊娠的HRHS胎儿均顺利分娩，出生后经早期干预后均存活。（4）围手术期并发症：13例孕妇中，FPV术后当日，3例出现恶心、呕吐，对症给予托烷司琼治疗后好转；1例出现腹痛，可耐受，未予特殊处理自行好转。13例孕妇围手术期无心力衰竭、流产、死亡等严重并发症。13例HRHS胎儿中，2例FPV术后当日出现严重的持续性心动过缓，1例术后随访过程中出现依赖右心室的冠状动脉循环，均选择终止妊娠。（5）遗传学筛查：13例HRHS胎儿均行染色体核型分析及染色体微阵列分析，未见典型的染色体异常及其他遗传学异常。n 结论:FPV可作为HRHS胎儿的有效干预措施，可促进胎儿右心室的发育。多学科专业技术团队围手术期科学管理对确保手术成功及孕妇、胎儿的安全尤为重要。“,”Objective:To summarize the experience of perioperative management strategy of fetal pulmonary valvuloplasty (FPV) for hypoplastic right heart syndrome (HRHS).Methods:In the retrospective study of perioperative data, 13 fetuses of HRHS were treated with FPV in Qingdao Women and Children's Hospital from July 2018 to June 2019.Results:(1) The evaluation indexes of the right ventricle in 13 fetuses before FPV: the mean ratio of tricuspid/mitral annulus, right/left ventricular length, pulmonary/aortic annulus, and tricuspid inflow time/cardiac cycle were 0.81±0.04, 0.56±0.14, 0.69±0.06, and 0.35±0.03, respectively. (2) All pregnant mothers underwent general anesthesia. The basic fetal heart rate was (156±12) beats per minutes before FPV, and 9 fetuses showed bradycardia during the operation, and recovered to normal after drug resuscitation. On the first day after FPV, two cases had bradycardia and pregnancy was terminated. The fluctuation of systolic blood pressure of pregnant mother was less than 20%, and there was no significant difference between preoperative and intraoperative pulse pressure [(36.0±5.6) vs (35.8±6.9) mmHg (1 mmHg=0.133 kPa); n t=8.102, n P=0.951]. (3) All cases of HRHS fetus successfully underwent FPV. The average gestational age was (27.3±0.8) weeks. The average operation time was (23.2±1.0) minutes. The ratio of tricuspid to mitral annulus (n t=-2.513, n P=0.022) and the ratio of right to left ventricular length (n t=-3.373, n P=0.003) were significantly improved at 6 weeks postoperatively. Ten fetuses were delivered, and there was no death after early intervention. (4) Of 13 pregnant women, 3 cases were nausea and vomiting on the day of FPV operation, the treatment of the symptoms was improved by tropisetron; one case had tolerable abdominal pain and improved without special treatment. Pregnant women had no major complications such as cardiac failure, abortion and death. (5) Chromosome karyotype analysis and microarray analysis of amniotic fluid was retained during the operation. No typical chromosome abnormality or other abnormal genetic diagnosis was found.n Conclusions:FPV colud be used as an effective intervention measure to promote the development of right ventricle in HRHS fetuses. The scientific management of multidisciplinary professional technical team in perioperative period is particularly important to ensure the success of FPV and the safety of pregnant women and fetuses.