Functional state following the Fontan procedure

Ismee A. Williams, Lynn A. Sleeper, Steven D. Colan, Minmin Lu, Elizabeth A. Stephenson, Jane W. Newburger, Welton M. Gersony, Meryl S. Cohen, James F. Cnota, Andrew M. Atz, Richard V. Williams, Renee Margossian, Andrew J. Powell, Mario P. Stylianou, Daphne T. Hsu, Gail Pearson, Judith Massicot-Fisher, Marsha Mathis, Victoria Pemberton, Paul Mitchell & 57 others Dianne Gallagher, Patti Nash, Gloria Klein, Minmin Lu, Lynn Mahony, Stephen Roth, Roger Breitbart, Jonathan Rhodes, Jodi Elder, Ellen McGrath, Seema Mital, Beth Printz, Ashwin Prakash, Darlene Servedio, Victoria Vetter, Bernard J. Clark, Mark Fogel, Steven Paridon, Jack Rychik, Margaret Harkins, Jamie Koh, Page A W Anderson, Rene Herlong, Lynne Hurwitz, Jennifer S. Li, Ann Marie Nawrocki, J. Philip Saul, Andrew D. Blaufox, Girish Shirali, Jon Lucas, Amy Blevins, LuAnn A. Minich, Linda Lambert, Michael Puchalski, Brian McCrindle, Timothy Bradley, Kevin Roman, Jennifer Russell, Shi Joon Yoo, Elizabeth Radojewski, Nancy Slater, Tal Geva, Marcy Schwartz, Michael Artman, Dana Connolly, Timothy Feltes, Julie Johnson, Jeffrey Krischer, G. Paul Matherne, John Kugler, Kathryn Davis, David J. Driscoll, Mark Galantowicz, Sally A. Hunsberger, Thomas J. Knight, Catherine L. Webb, Lawrence S Wissow

Research output: Contribution to journalArticle

Abstract

Background: Despite improvements in outcomes after completion of the Fontan circulation, long-term functional state varies. We sought to identify pre- and postoperative characteristics associated with overall function. Methods and Results: We analyzed data from 476 survivors withthe Fontan circulation enrolled in the Pediatric Heart Network Fontan Cross-sectional Study. Mean age at creation of the Fontan circulation was 3.4 plus or minus 2.1 years, with a range from 0.7 to 17.5 years, and time since completion was 8.7 plus or minus 3.4 years, the range being from 1.1 to 17.3 years. We calculated a functional score for the survivors by averaging the percentile ranks of ventricular ejection fraction, maximal consumption of oxygen, the physical summary score for the Child Health Questionnaire, and a function of brain natriuretic peptide. The mean calculated score was 49.5 plus or minus 17.3, with a range from 3 to 87. After adjustment for time since completion of the circulation, we found that a lower score, and hence worse functional state, was associated with: right ventricular morphology (p less than 0.001), higher ventricular end-diastolic pressure (p equals 0.003) and lower saturations of oxygen (p equals 0.047) prior to completion of the Fontan circulation, lower income for the caregiver (p equals 0.003), and, in subjects without a prior superior cavopulmonary anastomosis, arrhythmias after completion of the circulation (p equals 0.003). The model explained almost one-fifth (18%) of the variation in the calculated scores. The score was not associated with surgical centre, sex, age, weight, fenestration, or the period of stay in hospital after completion of the Fontan circuit. A validation model, using 71 subjects randomly excluded from initial analysis, weakly correlated (R equals 0.17, p equals 0.16) with the score calculated from the dataset. Conclusions: Right ventricular morphology, higher ventricular end-diastolic pressure and lower saturations of oxygen prior to completion of the Fontan circuit, lower income for the provider of care, and arrhythmias after creation of the circuit, are all associated with a worse functional state. Unmeasured factors also influence outcomes. © 2009

Original languageEnglish (US)
Pages (from-to)320-330
Number of pages11
JournalCardiology in the Young
Volume19
Issue number4
DOIs
StatePublished - 2009
Externally publishedYes

Fingerprint

Fontan Procedure
Survivors
Cardiac Arrhythmias
Right Heart Bypass
Oxygen
Blood Pressure
Brain Natriuretic Peptide
Oxygen Consumption
Stroke Volume
Caregivers
Length of Stay
Cross-Sectional Studies
Pediatrics
Weights and Measures
Datasets
Surveys and Questionnaires
Child Health

Keywords

  • Clinical outcomes
  • Single ventricle
  • Univentricular heart

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Pediatrics, Perinatology, and Child Health

Cite this

Williams, I. A., Sleeper, L. A., Colan, S. D., Lu, M., Stephenson, E. A., Newburger, J. W., ... Wissow, L. S. (2009). Functional state following the Fontan procedure. Cardiology in the Young, 19(4), 320-330. https://doi.org/10.1017/S1047951109990382

Functional state following the Fontan procedure. / Williams, Ismee A.; Sleeper, Lynn A.; Colan, Steven D.; Lu, Minmin; Stephenson, Elizabeth A.; Newburger, Jane W.; Gersony, Welton M.; Cohen, Meryl S.; Cnota, James F.; Atz, Andrew M.; Williams, Richard V.; Margossian, Renee; Powell, Andrew J.; Stylianou, Mario P.; Hsu, Daphne T.; Pearson, Gail; Massicot-Fisher, Judith; Mathis, Marsha; Pemberton, Victoria; Mitchell, Paul; Gallagher, Dianne; Nash, Patti; Klein, Gloria; Lu, Minmin; Mahony, Lynn; Roth, Stephen; Breitbart, Roger; Rhodes, Jonathan; Elder, Jodi; McGrath, Ellen; Mital, Seema; Printz, Beth; Prakash, Ashwin; Servedio, Darlene; Vetter, Victoria; Clark, Bernard J.; Fogel, Mark; Paridon, Steven; Rychik, Jack; Harkins, Margaret; Koh, Jamie; Anderson, Page A W; Herlong, Rene; Hurwitz, Lynne; Li, Jennifer S.; Nawrocki, Ann Marie; Saul, J. Philip; Blaufox, Andrew D.; Shirali, Girish; Lucas, Jon; Blevins, Amy; Minich, LuAnn A.; Lambert, Linda; Puchalski, Michael; McCrindle, Brian; Bradley, Timothy; Roman, Kevin; Russell, Jennifer; Yoo, Shi Joon; Radojewski, Elizabeth; Slater, Nancy; Geva, Tal; Schwartz, Marcy; Artman, Michael; Connolly, Dana; Feltes, Timothy; Johnson, Julie; Krischer, Jeffrey; Matherne, G. Paul; Kugler, John; Davis, Kathryn; Driscoll, David J.; Galantowicz, Mark; Hunsberger, Sally A.; Knight, Thomas J.; Webb, Catherine L.; Wissow, Lawrence S.

In: Cardiology in the Young, Vol. 19, No. 4, 2009, p. 320-330.

Research output: Contribution to journalArticle

Williams, IA, Sleeper, LA, Colan, SD, Lu, M, Stephenson, EA, Newburger, JW, Gersony, WM, Cohen, MS, Cnota, JF, Atz, AM, Williams, RV, Margossian, R, Powell, AJ, Stylianou, MP, Hsu, DT, Pearson, G, Massicot-Fisher, J, Mathis, M, Pemberton, V, Mitchell, P, Gallagher, D, Nash, P, Klein, G, Lu, M, Mahony, L, Roth, S, Breitbart, R, Rhodes, J, Elder, J, McGrath, E, Mital, S, Printz, B, Prakash, A, Servedio, D, Vetter, V, Clark, BJ, Fogel, M, Paridon, S, Rychik, J, Harkins, M, Koh, J, Anderson, PAW, Herlong, R, Hurwitz, L, Li, JS, Nawrocki, AM, Saul, JP, Blaufox, AD, Shirali, G, Lucas, J, Blevins, A, Minich, LA, Lambert, L, Puchalski, M, McCrindle, B, Bradley, T, Roman, K, Russell, J, Yoo, SJ, Radojewski, E, Slater, N, Geva, T, Schwartz, M, Artman, M, Connolly, D, Feltes, T, Johnson, J, Krischer, J, Matherne, GP, Kugler, J, Davis, K, Driscoll, DJ, Galantowicz, M, Hunsberger, SA, Knight, TJ, Webb, CL & Wissow, LS 2009, 'Functional state following the Fontan procedure', Cardiology in the Young, vol. 19, no. 4, pp. 320-330. https://doi.org/10.1017/S1047951109990382
Williams IA, Sleeper LA, Colan SD, Lu M, Stephenson EA, Newburger JW et al. Functional state following the Fontan procedure. Cardiology in the Young. 2009;19(4):320-330. https://doi.org/10.1017/S1047951109990382
Williams, Ismee A. ; Sleeper, Lynn A. ; Colan, Steven D. ; Lu, Minmin ; Stephenson, Elizabeth A. ; Newburger, Jane W. ; Gersony, Welton M. ; Cohen, Meryl S. ; Cnota, James F. ; Atz, Andrew M. ; Williams, Richard V. ; Margossian, Renee ; Powell, Andrew J. ; Stylianou, Mario P. ; Hsu, Daphne T. ; Pearson, Gail ; Massicot-Fisher, Judith ; Mathis, Marsha ; Pemberton, Victoria ; Mitchell, Paul ; Gallagher, Dianne ; Nash, Patti ; Klein, Gloria ; Lu, Minmin ; Mahony, Lynn ; Roth, Stephen ; Breitbart, Roger ; Rhodes, Jonathan ; Elder, Jodi ; McGrath, Ellen ; Mital, Seema ; Printz, Beth ; Prakash, Ashwin ; Servedio, Darlene ; Vetter, Victoria ; Clark, Bernard J. ; Fogel, Mark ; Paridon, Steven ; Rychik, Jack ; Harkins, Margaret ; Koh, Jamie ; Anderson, Page A W ; Herlong, Rene ; Hurwitz, Lynne ; Li, Jennifer S. ; Nawrocki, Ann Marie ; Saul, J. Philip ; Blaufox, Andrew D. ; Shirali, Girish ; Lucas, Jon ; Blevins, Amy ; Minich, LuAnn A. ; Lambert, Linda ; Puchalski, Michael ; McCrindle, Brian ; Bradley, Timothy ; Roman, Kevin ; Russell, Jennifer ; Yoo, Shi Joon ; Radojewski, Elizabeth ; Slater, Nancy ; Geva, Tal ; Schwartz, Marcy ; Artman, Michael ; Connolly, Dana ; Feltes, Timothy ; Johnson, Julie ; Krischer, Jeffrey ; Matherne, G. Paul ; Kugler, John ; Davis, Kathryn ; Driscoll, David J. ; Galantowicz, Mark ; Hunsberger, Sally A. ; Knight, Thomas J. ; Webb, Catherine L. ; Wissow, Lawrence S. / Functional state following the Fontan procedure. In: Cardiology in the Young. 2009 ; Vol. 19, No. 4. pp. 320-330.
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abstract = "Background: Despite improvements in outcomes after completion of the Fontan circulation, long-term functional state varies. We sought to identify pre- and postoperative characteristics associated with overall function. Methods and Results: We analyzed data from 476 survivors withthe Fontan circulation enrolled in the Pediatric Heart Network Fontan Cross-sectional Study. Mean age at creation of the Fontan circulation was 3.4 plus or minus 2.1 years, with a range from 0.7 to 17.5 years, and time since completion was 8.7 plus or minus 3.4 years, the range being from 1.1 to 17.3 years. We calculated a functional score for the survivors by averaging the percentile ranks of ventricular ejection fraction, maximal consumption of oxygen, the physical summary score for the Child Health Questionnaire, and a function of brain natriuretic peptide. The mean calculated score was 49.5 plus or minus 17.3, with a range from 3 to 87. After adjustment for time since completion of the circulation, we found that a lower score, and hence worse functional state, was associated with: right ventricular morphology (p less than 0.001), higher ventricular end-diastolic pressure (p equals 0.003) and lower saturations of oxygen (p equals 0.047) prior to completion of the Fontan circulation, lower income for the caregiver (p equals 0.003), and, in subjects without a prior superior cavopulmonary anastomosis, arrhythmias after completion of the circulation (p equals 0.003). The model explained almost one-fifth (18{\%}) of the variation in the calculated scores. The score was not associated with surgical centre, sex, age, weight, fenestration, or the period of stay in hospital after completion of the Fontan circuit. A validation model, using 71 subjects randomly excluded from initial analysis, weakly correlated (R equals 0.17, p equals 0.16) with the score calculated from the dataset. Conclusions: Right ventricular morphology, higher ventricular end-diastolic pressure and lower saturations of oxygen prior to completion of the Fontan circuit, lower income for the provider of care, and arrhythmias after creation of the circuit, are all associated with a worse functional state. Unmeasured factors also influence outcomes. {\circledC} 2009",
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author = "Williams, {Ismee A.} and Sleeper, {Lynn A.} and Colan, {Steven D.} and Minmin Lu and Stephenson, {Elizabeth A.} and Newburger, {Jane W.} and Gersony, {Welton M.} and Cohen, {Meryl S.} and Cnota, {James F.} and Atz, {Andrew M.} and Williams, {Richard V.} and Renee Margossian and Powell, {Andrew J.} and Stylianou, {Mario P.} and Hsu, {Daphne T.} and Gail Pearson and Judith Massicot-Fisher and Marsha Mathis and Victoria Pemberton and Paul Mitchell and Dianne Gallagher and Patti Nash and Gloria Klein and Minmin Lu and Lynn Mahony and Stephen Roth and Roger Breitbart and Jonathan Rhodes and Jodi Elder and Ellen McGrath and Seema Mital and Beth Printz and Ashwin Prakash and Darlene Servedio and Victoria Vetter and Clark, {Bernard J.} and Mark Fogel and Steven Paridon and Jack Rychik and Margaret Harkins and Jamie Koh and Anderson, {Page A W} and Rene Herlong and Lynne Hurwitz and Li, {Jennifer S.} and Nawrocki, {Ann Marie} and Saul, {J. Philip} and Blaufox, {Andrew D.} and Girish Shirali and Jon Lucas and Amy Blevins and Minich, {LuAnn A.} and Linda Lambert and Michael Puchalski and Brian McCrindle and Timothy Bradley and Kevin Roman and Jennifer Russell and Yoo, {Shi Joon} and Elizabeth Radojewski and Nancy Slater and Tal Geva and Marcy Schwartz and Michael Artman and Dana Connolly and Timothy Feltes and Julie Johnson and Jeffrey Krischer and Matherne, {G. Paul} and John Kugler and Kathryn Davis and Driscoll, {David J.} and Mark Galantowicz and Hunsberger, {Sally A.} and Knight, {Thomas J.} and Webb, {Catherine L.} and Wissow, {Lawrence S}",
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doi = "10.1017/S1047951109990382",
language = "English (US)",
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TY - JOUR

T1 - Functional state following the Fontan procedure

AU - Williams, Ismee A.

AU - Sleeper, Lynn A.

AU - Colan, Steven D.

AU - Lu, Minmin

AU - Stephenson, Elizabeth A.

AU - Newburger, Jane W.

AU - Gersony, Welton M.

AU - Cohen, Meryl S.

AU - Cnota, James F.

AU - Atz, Andrew M.

AU - Williams, Richard V.

AU - Margossian, Renee

AU - Powell, Andrew J.

AU - Stylianou, Mario P.

AU - Hsu, Daphne T.

AU - Pearson, Gail

AU - Massicot-Fisher, Judith

AU - Mathis, Marsha

AU - Pemberton, Victoria

AU - Mitchell, Paul

AU - Gallagher, Dianne

AU - Nash, Patti

AU - Klein, Gloria

AU - Lu, Minmin

AU - Mahony, Lynn

AU - Roth, Stephen

AU - Breitbart, Roger

AU - Rhodes, Jonathan

AU - Elder, Jodi

AU - McGrath, Ellen

AU - Mital, Seema

AU - Printz, Beth

AU - Prakash, Ashwin

AU - Servedio, Darlene

AU - Vetter, Victoria

AU - Clark, Bernard J.

AU - Fogel, Mark

AU - Paridon, Steven

AU - Rychik, Jack

AU - Harkins, Margaret

AU - Koh, Jamie

AU - Anderson, Page A W

AU - Herlong, Rene

AU - Hurwitz, Lynne

AU - Li, Jennifer S.

AU - Nawrocki, Ann Marie

AU - Saul, J. Philip

AU - Blaufox, Andrew D.

AU - Shirali, Girish

AU - Lucas, Jon

AU - Blevins, Amy

AU - Minich, LuAnn A.

AU - Lambert, Linda

AU - Puchalski, Michael

AU - McCrindle, Brian

AU - Bradley, Timothy

AU - Roman, Kevin

AU - Russell, Jennifer

AU - Yoo, Shi Joon

AU - Radojewski, Elizabeth

AU - Slater, Nancy

AU - Geva, Tal

AU - Schwartz, Marcy

AU - Artman, Michael

AU - Connolly, Dana

AU - Feltes, Timothy

AU - Johnson, Julie

AU - Krischer, Jeffrey

AU - Matherne, G. Paul

AU - Kugler, John

AU - Davis, Kathryn

AU - Driscoll, David J.

AU - Galantowicz, Mark

AU - Hunsberger, Sally A.

AU - Knight, Thomas J.

AU - Webb, Catherine L.

AU - Wissow, Lawrence S

PY - 2009

Y1 - 2009

N2 - Background: Despite improvements in outcomes after completion of the Fontan circulation, long-term functional state varies. We sought to identify pre- and postoperative characteristics associated with overall function. Methods and Results: We analyzed data from 476 survivors withthe Fontan circulation enrolled in the Pediatric Heart Network Fontan Cross-sectional Study. Mean age at creation of the Fontan circulation was 3.4 plus or minus 2.1 years, with a range from 0.7 to 17.5 years, and time since completion was 8.7 plus or minus 3.4 years, the range being from 1.1 to 17.3 years. We calculated a functional score for the survivors by averaging the percentile ranks of ventricular ejection fraction, maximal consumption of oxygen, the physical summary score for the Child Health Questionnaire, and a function of brain natriuretic peptide. The mean calculated score was 49.5 plus or minus 17.3, with a range from 3 to 87. After adjustment for time since completion of the circulation, we found that a lower score, and hence worse functional state, was associated with: right ventricular morphology (p less than 0.001), higher ventricular end-diastolic pressure (p equals 0.003) and lower saturations of oxygen (p equals 0.047) prior to completion of the Fontan circulation, lower income for the caregiver (p equals 0.003), and, in subjects without a prior superior cavopulmonary anastomosis, arrhythmias after completion of the circulation (p equals 0.003). The model explained almost one-fifth (18%) of the variation in the calculated scores. The score was not associated with surgical centre, sex, age, weight, fenestration, or the period of stay in hospital after completion of the Fontan circuit. A validation model, using 71 subjects randomly excluded from initial analysis, weakly correlated (R equals 0.17, p equals 0.16) with the score calculated from the dataset. Conclusions: Right ventricular morphology, higher ventricular end-diastolic pressure and lower saturations of oxygen prior to completion of the Fontan circuit, lower income for the provider of care, and arrhythmias after creation of the circuit, are all associated with a worse functional state. Unmeasured factors also influence outcomes. © 2009

AB - Background: Despite improvements in outcomes after completion of the Fontan circulation, long-term functional state varies. We sought to identify pre- and postoperative characteristics associated with overall function. Methods and Results: We analyzed data from 476 survivors withthe Fontan circulation enrolled in the Pediatric Heart Network Fontan Cross-sectional Study. Mean age at creation of the Fontan circulation was 3.4 plus or minus 2.1 years, with a range from 0.7 to 17.5 years, and time since completion was 8.7 plus or minus 3.4 years, the range being from 1.1 to 17.3 years. We calculated a functional score for the survivors by averaging the percentile ranks of ventricular ejection fraction, maximal consumption of oxygen, the physical summary score for the Child Health Questionnaire, and a function of brain natriuretic peptide. The mean calculated score was 49.5 plus or minus 17.3, with a range from 3 to 87. After adjustment for time since completion of the circulation, we found that a lower score, and hence worse functional state, was associated with: right ventricular morphology (p less than 0.001), higher ventricular end-diastolic pressure (p equals 0.003) and lower saturations of oxygen (p equals 0.047) prior to completion of the Fontan circulation, lower income for the caregiver (p equals 0.003), and, in subjects without a prior superior cavopulmonary anastomosis, arrhythmias after completion of the circulation (p equals 0.003). The model explained almost one-fifth (18%) of the variation in the calculated scores. The score was not associated with surgical centre, sex, age, weight, fenestration, or the period of stay in hospital after completion of the Fontan circuit. A validation model, using 71 subjects randomly excluded from initial analysis, weakly correlated (R equals 0.17, p equals 0.16) with the score calculated from the dataset. Conclusions: Right ventricular morphology, higher ventricular end-diastolic pressure and lower saturations of oxygen prior to completion of the Fontan circuit, lower income for the provider of care, and arrhythmias after creation of the circuit, are all associated with a worse functional state. Unmeasured factors also influence outcomes. © 2009

KW - Clinical outcomes

KW - Single ventricle

KW - Univentricular heart

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JO - Cardiology in the Young

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