Oka Norihiko, Tomoyasu Takahiro, Kaneko Masahiro, Matsui Kenta
Pediatric and Congenital Cardiovascular Surgery, Jichi Children's Medical Center Tochigi, Shimotsuke, Japan.
World J Pediatr Congenit Heart Surg. 2025 Nov;16(6):855-858. doi: 10.1177/21501351251345787. Epub 2025 Jun 26.
We report the case of a four-month-old child with complex congenital heart disease, including tricuspid atresia, absent pulmonary valve, hypoplastic right ventricle, right ventricular coronary artery fistula, and Uhl anomaly, and to describe the challenges and management decisions involved. A Blalock-Taussig-Thomas (BTT) shunt was initially planned. However, severe ST-segment depression occurred upon oral feeding, indicating coronary insufficiency. Cardiac catheterization revealed a single coronary artery with a right ventricular coronary artery fistula, which increased the risk of myocardial ischemia with the BTT shunt. Consequently, a bidirectional Glenn (BDG) procedure with left pulmonary artery reconstruction was performed after maintaining ductal patency with prostaglandin for four months. The patient had a favorable postoperative course without ischemic events. In patients with complex congenital heart defects and coronary anomalies, the presence of a right ventricular coronary artery fistula may increase the risk of ischemia with a BTT shunt. A BDG procedure can offer a safer alternative, leading to a positive clinical outcome and avoiding ischemic complications.
我们报告了一例4个月大患有复杂先天性心脏病的患儿，其病情包括三尖瓣闭锁、肺动脉瓣缺如、右心室发育不全、右心室冠状动脉瘘和乌尔异常，并描述了其中涉及的挑战和治疗决策。最初计划进行布莱洛克-陶西格-托马斯（BTT）分流术。然而，经口喂养时出现严重的ST段压低，提示冠状动脉供血不足。心导管检查发现单支冠状动脉伴右心室冠状动脉瘘，这增加了BTT分流术导致心肌缺血的风险。因此，在使用前列腺素维持动脉导管通畅4个月后，进行了带左肺动脉重建的双向格林（BDG）手术。患者术后病程顺利，未发生缺血事件。对于患有复杂先天性心脏缺陷和冠状动脉异常的患者，右心室冠状动脉瘘的存在可能会增加BTT分流术导致缺血的风险。BDG手术可提供更安全的选择，带来良好的临床结果并避免缺血性并发症。
