Clinical and hemodynamic characteristics of the pediatric failing Fontan

Pediatric Heart Transplant Society; Rebecca K. Ameduri

doi:10.1016/j.healun.2021.07.017

Clinical and hemodynamic characteristics of the pediatric failing Fontan

Pediatric Heart Transplant Society, Rebecca K. Ameduri

Pediatric Cardiology

Research output: Contribution to journal › Article › peer-review

13 Scopus citations

Abstract

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/m 2). In the entire cohort, 66% had FP >15 mmHg, 21% had FP >20 mmHg, and 10% had CI <2.2 L/min/m 2. 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.

Original language	English (US)
Pages (from-to)	1529-1539
Number of pages	11
Journal	Journal of Heart and Lung Transplantation
Volume	40
Issue number	12
Early online date	Jul 29 2021
DOIs	https://doi.org/10.1016/j.healun.2021.07.017
State	Published - Dec 2021

Bibliographical note

Funding Information:
The authors would like to thank the data coordinating center at the Pediatric Heart Transplant Society, University of Alabama, and Yulin Zhang at the Clinical and Translational Research Program of the Betty Irene Moore Children's Heart Center, Stanford University, for their assistance in performing this study

Publisher Copyright:
© 2021 International Society for Heart and Lung Transplantation

Keywords

Fontan
heart failure
heart transplant
hemodynamics
single ventricle

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10.1016/j.healun.2021.07.017

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title = "Clinical and hemodynamic characteristics of the pediatric failing Fontan",

abstract = "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/m 2). In the entire cohort, 66% had FP >15 mmHg, 21% had FP >20 mmHg, and 10% had CI <2.2 L/min/m 2. 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.",

keywords = "Fontan, heart failure, heart transplant, hemodynamics, single ventricle",

author = "{Pediatric Heart Transplant Society} and Dykes, {John C.} and Rosenthal, {David N.} and Daniel Bernstein and McElhinney, {Doff B.} and Chrisant, {Maryanne R.K.} and Daly, {Kevin P.} and Ameduri, {Rebecca K.} and Kenneth Knecht and Richmond, {Marc E.} and Lin, {Kimberly Y.} and Simon Urschel and Jacob Simmonds and Simpson, {Kathleen E.} and Albers, {Erin L.} and Asma Khan and Kurt Schumacher and Almond, {Christopher S.} and Sharon Chen",

note = "Funding Information: The authors would like to thank the data coordinating center at the Pediatric Heart Transplant Society, University of Alabama, and Yulin Zhang at the Clinical and Translational Research Program of the Betty Irene Moore Children's Heart Center, Stanford University, for their assistance in performing this study Publisher Copyright: {\textcopyright} 2021 International Society for Heart and Lung Transplantation",

year = "2021",

month = dec,

doi = "10.1016/j.healun.2021.07.017",

language = "English (US)",

volume = "40",

pages = "1529--1539",

journal = "Journal of Heart and Lung Transplantation",

issn = "1053-2498",

publisher = "Elsevier Inc.",

number = "12",

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TY - JOUR

T1 - Clinical and hemodynamic characteristics of the pediatric failing Fontan

AU - Pediatric Heart Transplant Society

AU - Dykes, John C.

AU - Rosenthal, David N.

AU - Bernstein, Daniel

AU - McElhinney, Doff B.

AU - Chrisant, Maryanne R.K.

AU - Daly, Kevin P.

AU - Ameduri, Rebecca K.

AU - Knecht, Kenneth

AU - Richmond, Marc E.

AU - Lin, Kimberly Y.

AU - Urschel, Simon

AU - Simmonds, Jacob

AU - Simpson, Kathleen E.

AU - Albers, Erin L.

AU - Khan, Asma

AU - Schumacher, Kurt

AU - Almond, Christopher S.

AU - Chen, Sharon

N1 - Funding Information: The authors would like to thank the data coordinating center at the Pediatric Heart Transplant Society, University of Alabama, and Yulin Zhang at the Clinical and Translational Research Program of the Betty Irene Moore Children's Heart Center, Stanford University, for their assistance in performing this study Publisher Copyright: © 2021 International Society for Heart and Lung Transplantation

PY - 2021/12

Y1 - 2021/12

N2 - 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/m 2). In the entire cohort, 66% had FP >15 mmHg, 21% had FP >20 mmHg, and 10% had CI <2.2 L/min/m 2. 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.

AB - 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/m 2). In the entire cohort, 66% had FP >15 mmHg, 21% had FP >20 mmHg, and 10% had CI <2.2 L/min/m 2. 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.

KW - Fontan

KW - heart failure

KW - heart transplant

KW - hemodynamics

KW - single ventricle

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U2 - 10.1016/j.healun.2021.07.017

DO - 10.1016/j.healun.2021.07.017

M3 - Article

C2 - 34412962

AN - SCOPUS:85112798592

SN - 1053-2498

VL - 40

SP - 1529

EP - 1539

JO - Journal of Heart and Lung Transplantation

JF - Journal of Heart and Lung Transplantation

IS - 12

ER -

Clinical and hemodynamic characteristics of the pediatric failing Fontan

Abstract

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