Division of Cardiology, Department of Medicine, Columbia University Medical Center, New York, New York, USA.
Department of Medicine, Division of Cardiology, Ahmason/UCLA Adult Congenital Heart Disease Center, University of California Los Angeles, Los Angeles, California, USA.
J Am Coll Cardiol. 2023 Jun 6;81(22):2149-2160. doi: 10.1016/j.jacc.2023.03.421.
The impact of Fontan-associated liver disease (FALD) on post-transplant mortality and indications for combined heart-liver transplant (CHLT) in adult Fontan patients remains unknown.
The purpose of this study was to assess the impact of FALD on post-transplant outcomes and compare HT vs CHLT in adult Fontan patients.
We performed a retrospective-cohort study of adult Fontan patients who underwent HT or CHLT across 15 centers. Inclusion criteria were as follows: 1) Fontan; 2) HT/CHLT referral; and 3) age ≥16 years at referral. Pretransplant FALD score was calculated using the following: 1) cirrhosis; 2) varices; 3) splenomegaly; or 4) ≥2 paracenteses.
A total of 131 patients (91 HT and 40 CHLT) were included. CHLT recipients were more likely to be older (P = 0.016), have a lower hemoglobin (P = 0.025), require ≥2 diuretic agents pretransplant (P = 0.051), or be transplanted in more recent decades (P = 0.001). Postmatching, CHLT demonstrated a trend toward improved survival at 1 year (93% vs 74%; P = 0.097) and improved survival at 5 years (86% vs 52%; P = 0.041) compared with HT alone. In patients with a FALD score ≥2, CHLT was associated with improved survival (1 year: 85% vs 62%; P = 0.044; 5 years: 77% vs 42%; P = 0.019). In a model with transplant decade and FALD score, CHLT was associated with improved survival (HR: 0.33; P = 0.044) and increasing FALD score was associated with worse survival (FALD score: 2 [HR: 14.6; P = 0.015], 3 [HR: 22.2; P = 0.007], and 4 [HR: 27.8; P = 0.011]).
Higher FALD scores were associated with post-transplant mortality. Although prospective confirmation of our findings is necessary, compared with HT alone, CHLT recipients were older with higher FALD scores, but had similar survival overall and superior survival in patients with a FALD score ≥2.
Fontan 相关肝疾病（FALD）对成年 Fontan 患者移植后死亡率和联合心肝移植（CHLT）适应证的影响尚不清楚。
本研究旨在评估 FALD 对移植后结局的影响，并比较成年 Fontan 患者行 HT 与 CHLT 的效果。
我们对在 15 个中心行 HT 或 CHLT 的成年 Fontan 患者进行了回顾性队列研究。纳入标准如下：1）Fontan；2）HT/CHLT 转诊；3）转诊时年龄≥16 岁。采用以下方法计算移植前 FALD 评分：1）肝硬化；2）静脉曲张；3）脾肿大；或 4）≥2 次腹腔穿刺术。
共纳入 131 例患者（91 例 HT 和 40 例 CHLT）。CHLT 受者更可能年龄较大（P = 0.016），血红蛋白较低（P = 0.025），移植前需要≥2 种利尿剂（P = 0.051）或在更近的几十年接受移植（P = 0.001）。匹配后，CHLT 在 1 年（93%比 74%；P = 0.097）和 5 年（86%比 52%；P = 0.041）时的生存率均有改善的趋势，与单独 HT 相比。在 FALD 评分≥2 的患者中，CHLT 与生存率的改善相关（1 年：85%比 62%；P = 0.044；5 年：77%比 42%；P = 0.019）。在包含移植年代和 FALD 评分的模型中，CHLT 与生存率的改善相关（HR：0.33；P = 0.044），而 FALD 评分的增加与生存率的下降相关（FALD 评分：2 [HR：14.6；P = 0.015]，3 [HR：22.2；P = 0.007]，4 [HR：27.8；P = 0.011]）。
较高的 FALD 评分与移植后死亡率相关。尽管需要前瞻性证实我们的发现，但与单独 HT 相比，CHLT 受者年龄较大且 FALD 评分较高，但总体生存率相似，FALD 评分≥2 的患者生存率更高。
