Duarte Valeria E, Urey Marcus A, Adler Eric D, Merkelz Brenda, Hobeika Mark J, Suarez Erik E, Quarti Andrea G, Yousefzai Rayan
Houston Methodist DeBakey Heart & Vascular Center, Houston Methodist, Houston, Texas, US.
University of California, San Diego, California, US.
Methodist Debakey Cardiovasc J. 2025 May 15;21(3):72-82. doi: 10.14797/mdcvj.1576. eCollection 2025.
The prevalence of adult congenital heart disease (ACHD) is increasing, with heart failure being the leading cause of death. For many ACHD patients, heart transplantation is the only treatment option for advanced heart failure, though significant extracardiac involvement may require multi-organ transplantation. Despite the rising number of ACHD transplants, multi-organ transplants in this population remain challenging, and a substantial gap remains between those in need and those who receive a transplant. While short-term outcomes may be worse for ACHD patients, long-term outcomes are comparable and even superior to other cardiomyopathies. Extracardiac organ dysfunction is common in ACHD patients, often precluding heart-alone transplantation. Fontan-associated liver disease, pulmonary vascular and restrictive lung disease, and renal dysfunction frequently necessitate multi-organ transplantation. ACHD patients have a unique immunological and sensitization profile, increasing their risk for infection, rejection, and malignancies, requiring specialized pretransplant desensitization and post-transplant immunosuppression strategies. ACHD transplantation presents unique surgical challenges, including chest reentry, vascular access issues, bleeding risks, extensive anatomical reconstruction, the need for longer vascular segments from donors, and prolonged ischemic times. Decisions regarding heart-alone versus heart-liver, heart-lung, or heart-kidney transplantation demand careful evaluation. These complex surgical plans require extensive multimodal imaging and collaboration with ACHD cardiac imaging specialists and abdominal transplant teams. Comprehensive coordination and psychosocial support are crucial for ACHD patients throughout the transplant process. A dedicated multidisciplinary team and an established and separate pathway for pre-, peri-, and postoperative care in centers with ACHD and multi-organ transplant expertise are essential. There is need for a revised organ allocation system to ensure timely access to transplantation for ACHD patients.
成人先天性心脏病（ACHD）的患病率正在上升，心力衰竭是主要死因。对于许多ACHD患者而言，心脏移植是晚期心力衰竭的唯一治疗选择，不过显著的心脏外受累可能需要多器官移植。尽管ACHD移植数量不断增加，但该人群的多器官移植仍然具有挑战性，在有需求者和接受移植者之间仍存在巨大差距。虽然ACHD患者的短期预后可能较差，但长期预后相当，甚至优于其他心肌病。心脏外器官功能障碍在ACHD患者中很常见，常常使单纯心脏移植无法进行。与Fontan相关的肝病、肺血管和限制性肺病以及肾功能不全常常需要多器官移植。ACHD患者具有独特的免疫和致敏特征，增加了他们感染、排斥和患恶性肿瘤的风险，需要专门的移植前脱敏和移植后免疫抑制策略。ACHD移植带来了独特的手术挑战，包括再次开胸、血管通路问题、出血风险、广泛的解剖重建、需要供体更长的血管段以及更长的缺血时间。关于单纯心脏移植还是心脏-肝脏、心肺或心脏-肾脏移植的决策需要仔细评估。这些复杂的手术计划需要广泛的多模态成像，并与ACHD心脏成像专家和腹部移植团队合作。在整个移植过程中，全面的协调和心理社会支持对ACHD患者至关重要。一个专门的多学科团队以及在具备ACHD和多器官移植专业知识的中心建立单独的术前、术中和术后护理途径至关重要。需要修订器官分配系统，以确保ACHD患者能够及时获得移植。
