Tran Dai Dac, Do Tien Anh, Bui Huy Quang, Le Thanh Ngoc, Vo Long Hoang
Cardiovascular Center, E Hospital, Hanoi, Vietnam.
Department of Science, Technology, Communication & International Cooperation, E Hospital, Hanoi, Vietnam.
Cardiol Young. 2025 May;35(5):1057-1061. doi: 10.1017/S1047951125001581. Epub 2025 Apr 14.
Protein-losing enteropathy remains a formidable and underrecognised complication following Fontan surgery, contributing significantly to morbidity and mortality among affected patients. Despite substantial progress in managing single-ventricle CHDs, protein-losing enteropathy persists as a major clinical challenge, particularly in resource-constrained environments where specialised diagnostic modalities are scarce. This manuscript discusses the prevalence, clinical presentation, diagnostic hurdles, and management of protein-losing enteropathy in post-Fontan patients, drawing from clinical experiences in Vietnam. Among 95 post-Fontan patients reviewed, the prevalence of protein-losing enteropathy was 4.2%, with considerable morbidity necessitating invasive interventions and, in some cases, resulting in mortality. Diagnostic limitations, including restricted access to alpha-1-antitrypsin clearance testing, highlight the urgent need for heightened clinical suspicion and pragmatic diagnostic approaches. We advocate for integrating low-cost, widely accessible screening measures, such as routine serum protein monitoring, into standard post-Fontan care protocols to enhance early detection and management of protein-losing enteropathy. Furthermore, we underscore the critical importance of global collaboration and multicentre research initiatives to address the worldwide burden of protein-losing enteropathy, facilitate resource-sharing, and develop evidence-based interventions. Coordinated international efforts are essential to closing diagnostic and treatment gaps, ultimately improving survival and quality of life for patients living with the Fontan circulation.
蛋白质丢失性肠病仍然是Fontan手术后一个严峻且未得到充分认识的并发症，对受影响患者的发病率和死亡率有重大影响。尽管在单心室先天性心脏病的管理方面取得了重大进展，但蛋白质丢失性肠病仍然是一个主要的临床挑战，特别是在资源有限的环境中，那里缺乏专门的诊断手段。本文从越南的临床经验出发，讨论了Fontan手术后患者蛋白质丢失性肠病的患病率、临床表现、诊断障碍和管理。在95例接受回顾的Fontan手术后患者中，蛋白质丢失性肠病的患病率为4.2%，有相当高的发病率需要进行侵入性干预，在某些情况下还导致死亡。诊断限制，包括难以进行α-1抗胰蛋白酶清除试验，凸显了迫切需要提高临床怀疑度和采用务实的诊断方法。我们主张将低成本、易于获得的筛查措施，如常规血清蛋白监测，纳入Fontan手术后的标准护理方案，以加强对蛋白质丢失性肠病的早期检测和管理。此外，我们强调全球合作和多中心研究倡议对于应对全球蛋白质丢失性肠病负担、促进资源共享以及制定循证干预措施的至关重要性。协调一致的国际努力对于弥合诊断和治疗差距至关重要，最终可改善接受Fontan循环治疗患者的生存率和生活质量。
