Clinical and hemodynamic characteristics of the pediatric failing Fontan,The Journal of Heart and Lung Transplantation

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Clinical and hemodynamic characteristics of the pediatric failing Fontan
The Journal of Heart and Lung Transplantation ( IF 8.9 ) Pub Date : 2021-07-29 , DOI: 10.1016/j.healun.2021.07.017
John C Dykes ₁ , David N Rosenthal ₁ , Daniel Bernstein ₁ , Doff B McElhinney ₂ , Maryanne R K Chrisant ₃ , Kevin P Daly ₄ , Rebecca K Ameduri ₅ , Kenneth Knecht ₆ , Marc E Richmond ₇ , Kimberly Y Lin ₈ , Simon Urschel ₉ , Jacob Simmonds ₁₀ , Kathleen E Simpson ₁₁ , Erin L Albers ₁₂ , Asma Khan ₁₃ , Kurt Schumacher ₁₄ , Christopher S Almond ₁ , Sharon Chen ₁ ,

Affiliation

Department of Pediatrics, Lucile Packard Children's Hospital, Stanford University.
Department of Pediatrics, Lucile Packard Children's Hospital, Stanford University; Department of Cardiovascular Surgery, Stanford University.
Joe DiMaggio Children's Hospital.
Boston Children's Hospital, Harvard Medical School.
University of Minnesota Masonic Children's Hospital.
Arkansas Children's Hospital, University of Arkansas for Medical Sciences.
Morgan Stanley Children's Hospital, Columbia University College of Physicians & Surgeons.
Children's Hospital of Philadelphia, University of Pennsylvania.
Stollery Children's Hospital University of Alberta.
Great Ormond Street Hospital for Children.
St. Louis Children's Hospital, Washington University in St. Louis.
Seattle Children's Hospital, University of Washington.
Ann and Robert H Lurie Children's Hospital, Northwestern University Feinberg School of Medicine.
C.S. Mott Children's Hospital, University of Michigan.

Aim

To describe the clinical and hemodynamic characteristics of Fontan failure in children listed for heart transplant.

Methods

In a nested study of the Pediatric Heart Transplant Society, 16 centers contributed information on Fontan patients listed for heart transplant between 2005and 2013. Patients were classified into four mutually exclusive phenotypes: Fontan with abnormal lymphatics (FAL), Fontan with reduced systolic function (FRF), Fontan with preserved systolic function (FPF), and Fontan with “normal” hearts (FNH). Primary outcome was waitlist and post-transplant mortality.

Results

177 children listed for transplant were followed over a median 13 (IQR 4-31) months, 84 (47%) were FAL, 57 (32%) FRF, 22 (12%) FNH, and 14 (8%) FPF. Hemodynamic characteristics differed between the 4 groups: Fontan pressure (FP) was most elevated with FPF (median 22, IQR 18-23, mmHg) and lowest with FAL (16, 14-20, mmHg); cardiac index (CI) was lowest with FRF (2.8, 2.3-3.4, L/min/m²). In the entire cohort, 66% had FP >15 mmHg, 21% had FP >20 mmHg, and 10% had CI <2.2 L/min/m². FRF had the highest risk of waitlist mortality (21%) and FNH had the highest risk of post-transplant mortality (36%).

Conclusions

Elevated Fontan pressure is more common than low cardiac output in pediatric failing Fontan patients listed for transplant. Subtle hemodynamic differences exist between the various phenotypes of pediatric Fontan failure. Waitlist and post-transplant mortality risks differ by phenotype.

中文翻译：

小儿失败 Fontan 的临床和血流动力学特征

目的

描述被列入心脏移植名单的儿童 Fontan 衰竭的临床和血流动力学特征。

方法

在儿科心脏移植协会的一项嵌套研究中，16 个中心提供了有关 2005 年至 2013 年间被列入心脏移植名单的 Fontan 患者的信息。患者被分为四种相互排斥的表型：Fontan 淋巴管异常 (FAL)、Fontan 收缩功能降低 (FRF) )、保留收缩功能 (FPF) 的 Fontan 和具有“正常”心脏 (FNH) 的 Fontan。主要结果是候补名单和移植后死亡率。

结果

177 名接受移植的儿童在中位 13 (IQR 4-31) 个月内进行了随访，其中 84 名 (47%) 为 FAL，57 名 (32%) 为 FRF，22 名 (12%) 为 FNH，14 名 (8%) 为 FPF。4 组之间的血流动力学特征不同： Fontan 压力 (FP) 以 FPF 最高（中位数 22，IQR 18-23，mmHg）而 FAL 最低（16，14-20，mmHg）；^{FRF (2.8, 2.3-3.4, L/min/m 2} )时心脏指数 (CI) 最低。在整个队列中，66% 的 FP >15 mmHg，21% 的 FP >20 mmHg，10% 的 CI <2.2 L/min/m ²。FRF 的候补死亡率风险最高（21%），而 FNH 的移植后死亡率风险最高（36%）。