Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure
Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking. This study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time. E...
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| Veröffentlicht in: | Journal of the American College of Cardiology Jg. 69; H. 22; S. 2735 |
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| Hauptverfasser: | , , , , , , , , , , , , , , , |
| Format: | Journal Article |
| Sprache: | Englisch |
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06.06.2017
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| ISSN: | 1558-3597, 1558-3597 |
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| Abstract | Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking.
This study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time.
Exercise testing, echocardiography, B-type natriuretic peptide, functional health assessment, and medical history abstraction were repeated 9.4 ± 0.4 years after the Fontan Cross-Sectional Study (Fontan 1) and compared with previous values. Cox regression analysis explored risk factors for interim death or cardiac transplantation.
From the original cohort of 546 subjects, 466 were contacted again, and 373 (80%) were enrolled at 21.2 ± 3.5 years of age. Among subjects with paired testing, the percent predicted maximum oxygen uptake decreased (69 ± 14% vs. 61 ± 16%; p < 0.001; n = 95), ejection fraction decreased (58 ± 11% vs. 55 ± 10%; p < 0.001; n = 259), and B-type natriuretic peptide increased (median [interquartile range] 13 [7 to 25] pg/mol vs. 18 [9 to 36] pg/mol; p < 0.001; n = 340). At latest follow-up, a lower Pediatric Quality of Life Inventory physical summary score was associated with poorer exercise performance (R
adjusted = 0.20; p < 0.001; n = 274). Cumulative complications since the Fontan procedure included additional cardiac surgery (32%), catheter intervention (62%), arrhythmia treatment (32%), thrombosis (12%), and protein-losing enteropathy (8%). Since Fontan 1, 54 subjects (10%) have received a heart transplant (n = 23) or died without transplantation (n = 31). The interval risk of death or/cardiac transplantation was associated with poorer ventricular performance and functional health status assessed at Fontan 1, but it was not associated with ventricular morphology, the subject's age, or the type of Fontan connection.
Interim transplant-free survival over 12 years in this Fontan cohort was 90% and was independent of ventricular morphology. Exercise performance decreased and was associated with worse functional health status. Future interventions might focus on preserving exercise capacity. (Relationship Between Functional Health Status and Ventricular Performance After Fontan-Pediatric Heart Network; NCT00132782). |
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| AbstractList | Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking.
This study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time.
Exercise testing, echocardiography, B-type natriuretic peptide, functional health assessment, and medical history abstraction were repeated 9.4 ± 0.4 years after the Fontan Cross-Sectional Study (Fontan 1) and compared with previous values. Cox regression analysis explored risk factors for interim death or cardiac transplantation.
From the original cohort of 546 subjects, 466 were contacted again, and 373 (80%) were enrolled at 21.2 ± 3.5 years of age. Among subjects with paired testing, the percent predicted maximum oxygen uptake decreased (69 ± 14% vs. 61 ± 16%; p < 0.001; n = 95), ejection fraction decreased (58 ± 11% vs. 55 ± 10%; p < 0.001; n = 259), and B-type natriuretic peptide increased (median [interquartile range] 13 [7 to 25] pg/mol vs. 18 [9 to 36] pg/mol; p < 0.001; n = 340). At latest follow-up, a lower Pediatric Quality of Life Inventory physical summary score was associated with poorer exercise performance (R
adjusted = 0.20; p < 0.001; n = 274). Cumulative complications since the Fontan procedure included additional cardiac surgery (32%), catheter intervention (62%), arrhythmia treatment (32%), thrombosis (12%), and protein-losing enteropathy (8%). Since Fontan 1, 54 subjects (10%) have received a heart transplant (n = 23) or died without transplantation (n = 31). The interval risk of death or/cardiac transplantation was associated with poorer ventricular performance and functional health status assessed at Fontan 1, but it was not associated with ventricular morphology, the subject's age, or the type of Fontan connection.
Interim transplant-free survival over 12 years in this Fontan cohort was 90% and was independent of ventricular morphology. Exercise performance decreased and was associated with worse functional health status. Future interventions might focus on preserving exercise capacity. (Relationship Between Functional Health Status and Ventricular Performance After Fontan-Pediatric Heart Network; NCT00132782). Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking.BACKGROUNDMulticenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking.This study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time.OBJECTIVESThis study sought to describe transplant-free survival and explore relationships between laboratory measures of ventricular performance and functional status over time.Exercise testing, echocardiography, B-type natriuretic peptide, functional health assessment, and medical history abstraction were repeated 9.4 ± 0.4 years after the Fontan Cross-Sectional Study (Fontan 1) and compared with previous values. Cox regression analysis explored risk factors for interim death or cardiac transplantation.METHODSExercise testing, echocardiography, B-type natriuretic peptide, functional health assessment, and medical history abstraction were repeated 9.4 ± 0.4 years after the Fontan Cross-Sectional Study (Fontan 1) and compared with previous values. Cox regression analysis explored risk factors for interim death or cardiac transplantation.From the original cohort of 546 subjects, 466 were contacted again, and 373 (80%) were enrolled at 21.2 ± 3.5 years of age. Among subjects with paired testing, the percent predicted maximum oxygen uptake decreased (69 ± 14% vs. 61 ± 16%; p < 0.001; n = 95), ejection fraction decreased (58 ± 11% vs. 55 ± 10%; p < 0.001; n = 259), and B-type natriuretic peptide increased (median [interquartile range] 13 [7 to 25] pg/mol vs. 18 [9 to 36] pg/mol; p < 0.001; n = 340). At latest follow-up, a lower Pediatric Quality of Life Inventory physical summary score was associated with poorer exercise performance (R2 adjusted = 0.20; p < 0.001; n = 274). Cumulative complications since the Fontan procedure included additional cardiac surgery (32%), catheter intervention (62%), arrhythmia treatment (32%), thrombosis (12%), and protein-losing enteropathy (8%). Since Fontan 1, 54 subjects (10%) have received a heart transplant (n = 23) or died without transplantation (n = 31). The interval risk of death or/cardiac transplantation was associated with poorer ventricular performance and functional health status assessed at Fontan 1, but it was not associated with ventricular morphology, the subject's age, or the type of Fontan connection.RESULTSFrom the original cohort of 546 subjects, 466 were contacted again, and 373 (80%) were enrolled at 21.2 ± 3.5 years of age. Among subjects with paired testing, the percent predicted maximum oxygen uptake decreased (69 ± 14% vs. 61 ± 16%; p < 0.001; n = 95), ejection fraction decreased (58 ± 11% vs. 55 ± 10%; p < 0.001; n = 259), and B-type natriuretic peptide increased (median [interquartile range] 13 [7 to 25] pg/mol vs. 18 [9 to 36] pg/mol; p < 0.001; n = 340). At latest follow-up, a lower Pediatric Quality of Life Inventory physical summary score was associated with poorer exercise performance (R2 adjusted = 0.20; p < 0.001; n = 274). Cumulative complications since the Fontan procedure included additional cardiac surgery (32%), catheter intervention (62%), arrhythmia treatment (32%), thrombosis (12%), and protein-losing enteropathy (8%). Since Fontan 1, 54 subjects (10%) have received a heart transplant (n = 23) or died without transplantation (n = 31). The interval risk of death or/cardiac transplantation was associated with poorer ventricular performance and functional health status assessed at Fontan 1, but it was not associated with ventricular morphology, the subject's age, or the type of Fontan connection.Interim transplant-free survival over 12 years in this Fontan cohort was 90% and was independent of ventricular morphology. Exercise performance decreased and was associated with worse functional health status. Future interventions might focus on preserving exercise capacity. (Relationship Between Functional Health Status and Ventricular Performance After Fontan-Pediatric Heart Network; NCT00132782).CONCLUSIONSInterim transplant-free survival over 12 years in this Fontan cohort was 90% and was independent of ventricular morphology. Exercise performance decreased and was associated with worse functional health status. Future interventions might focus on preserving exercise capacity. (Relationship Between Functional Health Status and Ventricular Performance After Fontan-Pediatric Heart Network; NCT00132782). |
| Author | Margossian, Renee McCrindle, Brian W Daniels, Kaitlyn Marino, Bradley S Breitbart, Roger E Williams, Richard V Goldberg, David J Henderson, Heather T Mahony, Lynn Burns, Kristin M Korsin, Rosalind Atz, Andrew M Zak, Victor D'agincourt, Nicholas Colan, Steven D Uzark, Karen |
| Author_xml | – sequence: 1 givenname: Andrew M surname: Atz fullname: Atz, Andrew M email: atzam@musc.edu organization: Medical University of South Carolina, Charleston, South Carolina. Electronic address: atzam@musc.edu – sequence: 2 givenname: Victor surname: Zak fullname: Zak, Victor organization: New England Research Institutes, Watertown, Massachusetts – sequence: 3 givenname: Lynn surname: Mahony fullname: Mahony, Lynn organization: University of Texas Southwestern Medical Center, Dallas, Texas – sequence: 4 givenname: Karen surname: Uzark fullname: Uzark, Karen organization: University of Michigan, Ann Arbor, Michigan – sequence: 5 givenname: Nicholas surname: D'agincourt fullname: D'agincourt, Nicholas organization: New England Research Institutes, Watertown, Massachusetts – sequence: 6 givenname: David J surname: Goldberg fullname: Goldberg, David J organization: Children's Hospital of Philadelphia, Philadelphia, Pennsylvania – sequence: 7 givenname: Richard V surname: Williams fullname: Williams, Richard V organization: University of Utah, Salt Lake City, Utah – sequence: 8 givenname: Roger E surname: Breitbart fullname: Breitbart, Roger E organization: Boston Children's Hospital, Boston, Massachusetts – sequence: 9 givenname: Steven D surname: Colan fullname: Colan, Steven D organization: Boston Children's Hospital, Boston, Massachusetts – sequence: 10 givenname: Kristin M surname: Burns fullname: Burns, Kristin M organization: National Heart, Lung, and Blood Institute, Bethesda, Maryland – sequence: 11 givenname: Renee surname: Margossian fullname: Margossian, Renee organization: Boston Children's Hospital, Boston, Massachusetts – sequence: 12 givenname: Heather T surname: Henderson fullname: Henderson, Heather T organization: Duke University Medical Center, Durham, North Carolina – sequence: 13 givenname: Rosalind surname: Korsin fullname: Korsin, Rosalind organization: Columbia University Medical Center, New York, New York – sequence: 14 givenname: Bradley S surname: Marino fullname: Marino, Bradley S organization: Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio – sequence: 15 givenname: Kaitlyn surname: Daniels fullname: Daniels, Kaitlyn organization: University of Utah, Salt Lake City, Utah – sequence: 16 givenname: Brian W surname: McCrindle fullname: McCrindle, Brian W organization: University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada |
| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/28571639$$D View this record in MEDLINE/PubMed |
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| ContentType | Journal Article |
| Copyright | Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved. |
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| DOI | 10.1016/j.jacc.2017.03.582 |
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| Keywords | functional health status exercise Fontan procedure adult congenital heart disease single ventricle |
| Language | English |
| License | Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved. |
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| References | 28571640 - J Am Coll Cardiol. 2017 Jun 6;69(22):2745-2747 29395194 - J Thorac Cardiovasc Surg. 2018 Apr;155(4):1727-1731 |
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| Snippet | Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking.
This study sought to describe... Multicenter longitudinal objective data for survival into adulthood of patients who have undergone Fontan procedures are lacking.BACKGROUNDMulticenter... |
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| SubjectTerms | Adolescent Child Cross-Sectional Studies Echocardiography Female Follow-Up Studies Fontan Procedure - methods Forecasting Health Status Heart Defects, Congenital - diagnosis Heart Defects, Congenital - mortality Heart Defects, Congenital - surgery Heart Ventricles - abnormalities Heart Ventricles - diagnostic imaging Humans Male Ontario - epidemiology Quality of Life Retrospective Studies Risk Factors Survival Rate - trends Treatment Outcome United States - epidemiology Young Adult |
| Title | Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure |
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