应用超声心动图对室间隔完整型肺动脉闭锁（PA/IVS）胎儿进行评估和预后分析。

回顾性选取2017年7月1日至2022年6月30日在苏州市立医院及苏州市吴江区第二人民医院产前超声诊断为PA/IVS的胎儿19例。依据PA/IVS胎儿超声心动图测量的三尖瓣瓣环内径Z值（TV-Z）、左右心室长径比值（RV/LV）、三尖瓣反流及有无右心室冠状动脉交通对PA/IVS胎儿右心室发育情况进行分型，并对胎儿预后进行分析。

19例胎儿中，Ⅰ型PA/IVS胎儿9例（9/19，47%）；Ⅱ型PA/IVS胎儿4例（4/19，21%）；Ⅲ型PA/IVS胎儿3例（3/19，16%）；Ⅳ型PA/IVS胎儿2例（2/19，11%）。合并右心室依赖性冠状动脉循环（RVDCC）1例。右心室发育良好或轻度发育不良7例（7/19，37%）：TV-Z值均值为-1.28，RV/LV均值为0.84。右心室中度发育不良8例（8/19，42%）：TV-Z值均值为-3.36，RV/LV均值为0.53。右心室重度发育不良3例（3/19，16%）：TV-Z值均值为-5.02，RV/LV均值为0.43。本组病例中8例进行了肺动脉瓣球囊扩张术或肺动脉瓣成形术及双心室矫正手术，其中6例为右心室发育良好或轻度发育不良，2例为中度发育不良，TV-Z值均>-2.3，RV/LV比值>0.7，三尖瓣中度反流1例，重度反流7例。9例胎儿引产，其中1例为轻度右心室发育不良，1例为中度发育不良，6例为重度发育不良；1例胎儿合并RVDCC。2例右心室中度发育不良胎儿进行了锁骨下动脉-肺动脉分流及单心室姑息治疗手术，TV-Z值均>-4，RV/LV比值<0.6，均为三尖瓣重度反流。

TV-Z值大小、三尖瓣反流程度、右心室发育不良的程度及有无RVDCC影响PA/IVS胎儿的预后，超声心动图可作为PA/IVS胎儿预后的有效评估手段。

To evaluate and analyze the prognosis of pulmonary atresia with intact interventricular septum (PA/IVS) in fetuses by echocardiography.

This is a retrospective study in which 19 fetuses diagnosed with PA/IVS by prenatal ultrasound at Suzhou City Hospital and Suzhou Wujiang Second People's Hospital from July 1, 2017 to June 30, 2022 were included. The development of the right ventricle of PA/IVS fetuses was classified according to the internal diameter Z value of the tricuspid value annulus (TV-Z), the ratio of left and right ventricular length to diameter (RV/LV), tricuspid regurgitation, and the presence of right ventricular dependent coronary circulation (RVDCC) detected by fetal echocardiography, and the fetal prognosis was analyzed.

According to the classification of the structure, 9 (9/19, 47%) fetuses with PA/IVS type Ⅰ were found to have right ventricular inflow tract and trabecular and infundibular regions, 4 (4/19, 21%) cases of type Ⅱ PA/IVS had the inflow tract and infundibulum, 3 (3/19, 16%) cases of type Ⅲ PA/IVS had only the inflow, and 2 (2/19, 11%) cases of type Ⅳ had the inflow tract and trabecular region. And one case with RVDCC.There were 7 cases (7/19, 37%) with a well-developed right ventricle; the TV-Z scores ranged from -1.83 to 0.90, with an average of -1.28; RV/LV ranged from 0.72 to 0.90, with an average value of 0.84. Eight cases (8/19, 42%) had moderate right ventricular dysplasia; the TV-Z scores ranged from -2.82 to -3.92, with an average value of -3.36; RV/LV ranged from 0.47 to 0.60, with an average value of 0.53. Three cases (3/19, 16%) had severe right ventricular dysplasia; TV-Z scores ranged from -4.13 to 5.86, with an average value of -5.02; RV/LV ranged from 0.36 to 0.49, with an average value of 0.43. In this series, 8 cases underwent balloon dilatation of the pulmonary valve or pulmonary valvuloplasty and biventricular correction, and 9 cases underwent induced labor. Two fetuses with moderate right ventricular dysplasia underwent Blalock-Taussig shunt and single ventricular palliative treatment.

TV-Z value, severity of tricuspid regurgitation, severity of right ventricular hypoplasia, and the presence or absence of RVDCC can affect the prognosis of PA/IVS fetuses. Echocardiography can be an effective means of evaluating the prognosis of PA/IVS fetuses.