一名接受Fontan循环手术的成年患者发生心外管道部分阻塞
Extracardiac Conduit Subocclusion in an Adult Patient With a Fontan Circulation.
作者信息
D'Angelo Emanuela C, Palmieri Rosalinda, Lisignoli Veronica, di Già Giulia, Della Porta Marco, Iuzzolino Rossella, Curione Davide, Albanese Sonia, Butera Gianfranco, Montanaro Claudia
机构信息
Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
出版信息
JACC Case Rep. 2025 Apr 2;30(7):103399. doi: 10.1016/j.jaccas.2025.103399.
BACKGROUND
Conduit obstruction is a complication of patients with Fontan circulation.
CASE SUMMARY
This case report describes a Fontan patient who presented with exertional dyspnea and bilateral peripheral edema. Echocardiography identified a hyperechogenic structure within the extracardiac conduit, leading to further investigations with computed tomography and cardiac magnetic resonance imaging. These studies confirmed severe conduit obstruction due to a calcific mass and demonstrated extensive collateral circulation.
MANAGEMENT
After a multidisciplinary team discussion, surgical extracardiac conduit replacement was performed. Postoperatively, the patient developed a pleural effusion, managed with surgical drainage and octreotide treatment.
DISCUSSION
Fontan failure could be due to many etiologies. Extracardiac conduit obstruction should be suspected in Fontan patients with heart failure symptoms and investigated with multimodality imaging techniques.
TAKE-HOME MESSAGE: This case highlights the necessity of a differential diagnosis in Fontan failure patients and the need of multimodal imaging to reach definitive information necessary for a correct surgical planning.
背景
管道梗阻是接受Fontan循环手术患者的一种并发症。
病例摘要
本病例报告描述了一名Fontan手术患者，出现劳力性呼吸困难和双侧外周水肿。超声心动图检查发现心外管道内有高回声结构，进而采用计算机断层扫描和心脏磁共振成像进行进一步检查。这些研究证实因钙化肿块导致严重的管道梗阻，并显示有广泛的侧支循环。
治疗
经过多学科团队讨论后，进行了心外管道置换手术。术后，患者出现胸腔积液，通过手术引流和奥曲肽治疗进行处理。
讨论
Fontan循环衰竭可能由多种病因引起。对于出现心力衰竭症状的Fontan手术患者，应怀疑心外管道梗阻，并采用多模态成像技术进行检查。
经验教训
本病例强调了对Fontan循环衰竭患者进行鉴别诊断的必要性，以及采用多模态成像获取正确手术规划所需明确信息的必要性。
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