3例胎儿因“胎儿超声心动图提示肺动脉瓣闭锁”就诊于青岛大学附属妇女儿童医院，生后出现不同程度血氧饱和度下降，复查超声心动图确诊为肺动脉闭锁合并右心发育不全综合征。采用顺行打孔新策略实施经皮球囊肺动脉瓣成形术，利用动脉导管构建稳定的体循环轨道并省去圈套器的使用。术后3个月超声心动图显示患儿右心功能均得到积极恢复与重塑，动脉导管闭合，成功实现双心室循环。.

Objective: To investigate the feasibility of ductus arteriosus (DA) stent placement through femoral artery approach without guiding catheter. Methods: In this retrospective case study, the birth weight, intervention age, preoperative echocardiography, intraoperative angiography and other clinical data, as well as the follow-up status (Nakata index and final circulatory status, etc.) of 15 patients with congenital heart disease who are dependent on the DA for pulmonary circulation at Women and Children's Hospital, Qingdao University between January 2018 to June 2023 were collected. All pediatric patients underwent placement of a DA stent without a guiding catheter, the efficacy and safety of this approach were analyzed. Results: Among all 15 cases, there were 9 males and 6 females, with the birth weight of (3.3±0.3) kg and the intervention age of (15.0±0.1) d. Of which, there were 9 cases of pulmonary atresia with intact ventricular septum (PA-IVS), and 6 cases of critical pulmonary stenosis (CPS) with intact ventricular septum. The tricuspid annulus diameter before intervention was (8.8±0.8) mm, and the tricuspid annulus Z-score was -0.7±0.9. There were 3 cases of mild right ventricular dysplasia, 9 cases of moderate dysplasia, and 3 cases of severe dysplasia. All patients successfully completed the intervention without major complications. The narrowest diameter of the DA was (2.2±0.1) mm, the diameter of the DA stent was (3.9±0.3) mm, the preoperative percutaneous oxygen saturation (SpO2) was 0.83±0.03, and the postoperative SpO2 was 0.96±0.02. The follow-up time was (7.0±0.5) months. The Nakata index was (163±30) mm2/m2 before intervention, and was (173±34) mm2/m2 at the last follow-up. Biventricular circulation was successfully achieved in 14 patients, and the other one patient currently under close observation. Conclusion: The placement of a ductus arteriosus stent through femoral artery retrogradely without guiding catheter could serve as an effective and safe plan for neonates with PA-IVS or CPS accompanied by hypoplastic right heart syndrome. 目的： 探讨经股动脉入路无导引导管行动脉导管支架置入术的可行性。 方法： 回顾性总结青岛大学附属妇女儿童医院2018年1月至2023年6月接受动脉导管支架置入术的15例肺循环依赖动脉导管的先天性心脏病患儿的出生体重、手术时年龄、术前超声心动图及术中血管造影等临床资料及随访情况（Nakata指数和最终循环状态等）。所有患儿选择在无导引导管的情况下置入动脉导管支架，分析该方案实施手术的有效性及安全性。 结果： 15例患儿中男9例、女6例，出生体重（3.3±0.3）kg，手术时年龄（15.0±0.1）日龄。9例为室间隔完整型肺动脉闭锁，6例为室间隔完整的危重肺动脉瓣狭窄。术前15例患儿三尖瓣瓣环直径为（8.8±0.8）mm，三尖瓣瓣环Z值为-0.7±0.9。右心室轻度发育不良3例、中度发育不良9例、重度发育不良3例。15例患儿均成功完成手术，无重大并发症。动脉导管最窄处直径（2.2±0.1）mm，动脉导管支架直径（3.9±0.3）mm，术前血氧饱和度0.83±0.03，术后血氧饱和度0.96±0.02。15例患儿随访时间为（7.0±0.5）个月，术前Nakata指数为（163±30）mm2/m2，末次随访为（173±34）mm2/m2。14例患儿动脉导管支架均闭合，实现双心室循环；1例仍密切随访。 结论： 经股动脉逆行无导引导管置入动脉导管支架可以作为室间隔完整型肺动脉闭锁或危重肺动脉瓣狭窄伴右心发育不良综合征新生儿安全有效的实施方法。.

Outcomes of single ventricle heart defects may be influenced by the morphological type of the hypoplastic ventricle. Recent multi-centre studies have targeted on outcomes of hypoplastic left heart syndrome with limited focus on outcomes of hypoplastic right heart syndrome. We aimed at studying the clinical outcomes of hypoplastic right heart syndrome in the recent era. We performed a retrospective analysis of all hypoplastic right heart syndrome patients (n = 153) born between January 2010 and January 2023. Five-year transplant-free survival was compared with hypoplastic left heart syndrome patients without heterotaxy (n = 144) born during the same time. Double-inlet left ventricle was the most common anatomic hypoplastic right heart syndrome subtype (n = 39, 25%). Twenty-six (17%) patients with hypoplastic right heart had associated heterotaxy. Five-year transplant-free survival was high for most groups (double inlet left ventricle: 100%, pulmonary atresia: 94%, Ebstein's anomaly: 92%, tricuspid atresia: 90% respectively). The heterotaxy group had worse early outcomes with 3 deaths and 3 heart transplants, before Fontan completion. Heterotaxy was a significant risk factor for death/transplant prior to Fontan completion in hypoplastic right heart syndrome patients (p = 0.03). Patients with hypoplastic right heart syndrome with heterotaxy had significantly worse 5-year transplant-free survival (71%) when compared to hypoplastic right heart syndrome without heterotaxy (95%) and hypoplastic left heart syndrome without heterotaxy (75%), p < 0.001. Hypoplastic right heart syndrome patients have excellent clinical outcomes and better early childhood survival than hypoplastic left heart syndrome in the current era, except when associated with heterotaxy. The survival advantage conferred by a single left ventricle appears to be negated by heterotaxy syndrome and should be strongly considered during caregiver counselling and medical decision-making.

• CTD may be associated with ASD or hypoplastic right heart syndrome. • CTD may be associated with Ebstein anomaly, TV dysplasia, or PV stenosis. • Clinical presentation varies by the degree of CTD obstruction to blood flow. • TTE is the diagnostic method of choice for CTD. [Figure: see text]