摘要本文报道1例诊断为危重肺动脉瓣狭窄、右心室发育差、重度三尖瓣反流等合并重度肾积水胎儿的诊治情况。经心脏超声检查及多学科会诊评估后，孕30周 +3行胎儿肺动脉瓣球囊成形术，促进右心室及三尖瓣的发育，为生后的双心室循环创造条件。孕31周行经皮胎儿肾穿刺术，经生化检查证实肾积水的性质为尿液。孕37周 +3孕妇阴道分娩后，使用前列环素维持患儿动脉导管开放，保持血氧饱和度80%以上。患儿于生后6 d行左肾造瘘术改善肾积水及肾功能，生后16 d成功行双心室矫治手术（经皮肺动脉瓣球囊扩张术），生后37 d行左侧离断式肾盂输尿管成形术。术后复查超声心脏及肾脏结构及功能均逐渐恢复。本例总结胎儿危重肺动脉瓣狭窄合并重度肾积水病例的特点和治疗方案，期待能为将来类似患儿的成功救治提供经验。

abstractsWe report the management of a fetus diagnosed with critical pulmonary stenosis, right ventricular hypoplasia, severe tricuspid regurgitation and severe hydronephrosis. After echocardiography and multidisciplinary team consultation, fetal pulmonary valvuloplasty was performed at 30 +3 weeks of gestation, to facilitate the development of the right ventricle and tricuspid valve. Fetal transdermal renal puncture performed at 31 weeks of gestation showed that the hydronephrotic fluid was urine. The mother gave birth vaginally at 37 +3 weeks. Prostacyclin was given to the baby to keep the ductus arteriosus open and maintain oxygen saturation above 80%. Nephrostomy was performed on the 6th day after birth for improvement of the hydronephrosis and renal function. Biventricular correction (percutaneous balloon pulmonary valvuloplasty) and left dismembered pyeloureteroplasty were successfully performed on 16 and 37 d after birth, respectively. Ultrasound follow-ups showed the structure and function of both heart and kidney recovered. We summarized the characteristics and management of fetal critical pulmonary stenosis complicated by severe hydronephrosis in this case, aiming to enhance experiences for appropriate treatment of similar cases in the future.