Haddad Raymond N, Hassan Ahmed Adel, Al Soufi Mahmoud, Kasem Mohamed
Service de Cardiologie Congénitale et Pédiatrique, Centre de Référence des Malformations Cardiaques Congénitales Complexes - M3C, Hôpital Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, 149, rue de Sèvres, Paris, 75015, France.
Department of Pediatric Cardiology, Heart Centre of Excellence, Al Jalila Children's Speciality Hospital, Dubai, United Arab Emirates.
BMC Cardiovasc Disord. 2025 Jun 4;25(1):432. doi: 10.1186/s12872-025-04876-5.
We report the case of an 11-year-old boy with situs inversus, interrupted inferior vena cava with azygos continuation, persistent left superior vena cava with absent bridging vein, congenitally corrected transposition of the great arteries, pulmonary atresia, and ventricular septal defect. He underwent a Kawashima shunt at age 1 and fenestrated extracardiac conduit Fontan completion at age 9, delayed due to loss to follow-up while living abroad. Fifteen months post-op, the patient was diagnosed with plastic bronchitis. Cardiac catheterization revealed elevated Fontan pressures and proximal left pulmonary artery stenosis, which was stented (AndraTec 38 mm Optimus-XL). Although fenestration creation was considered, the procedure was initially delayed allowing for a trial of medical management. Four months later, a 6 mm Occlutech atrial flow regulator (AFR) was placed transjugularly, but thrombosis occurred 48 h later. Transjugular catheter-based clot aspiration (Penumbra Lightning 7) and stenting (10/20 mm Cook Formula stent) followed by sequential high-pressure ballooning increased the fenestration diameter, improving symptoms and hemodynamics. He had a favorable recovery with significant improvements and remained stable at 13 weeks of follow-up.
我们报告了一名11岁男孩的病例，他患有内脏反位、下腔静脉中断伴奇静脉延续、永存左上腔静脉且桥静脉缺如、先天性矫正型大动脉转位、肺动脉闭锁和室间隔缺损。他1岁时接受了川岛分流术，9岁时接受了开窗心外管道Fontan手术，但因在国外生活失访而推迟。术后15个月，患者被诊断为塑料支气管炎。心导管检查显示Fontan压力升高和左肺动脉近端狭窄，遂进行了支架置入（AndraTec 38 mm Optimus-XL）。尽管考虑了开窗术，但该手术最初推迟以便进行药物治疗试验。四个月后，经颈静脉置入了一个6 mm的Occlutech心房血流调节器（AFR），但48小时后发生了血栓形成。随后进行经颈静脉导管血栓抽吸（Penumbra Lightning 7）和支架置入（10/20 mm Cook Formula支架），接着进行序贯高压球囊扩张，增加了开窗直径，改善了症状和血流动力学。他恢复良好，有显著改善，随访13周时保持稳定。
