Diastolic Dysfunction With Preserved Ejection Fraction After the Fontan Procedure
Background Heart failure phenotyping in single-ventricle Fontan patients is challenging, particularly in patients with normal ejection fraction (EF). The objective of this study was to identify Fontan patients with abnormal diastolic function, who are high risk for heart failure with preserved eject...
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| Published in: | Journal of the American Heart Association Vol. 11; no. 2; p. e024095 |
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| Main Authors: | , , , , , , , , |
| Format: | Journal Article |
| Language: | English |
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John Wiley and Sons Inc
18.01.2022
Wiley |
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| ISSN: | 2047-9980, 2047-9980 |
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| Abstract | Background Heart failure phenotyping in single-ventricle Fontan patients is challenging, particularly in patients with normal ejection fraction (EF). The objective of this study was to identify Fontan patients with abnormal diastolic function, who are high risk for heart failure with preserved ejection fraction (HFpEF), and characterize their cardiac mechanics, exercise function, and functional health status. Methods and Results Data were obtained from the Pediatric Heart Network Fontan Cross-sectional Study database. EF was considered abnormal if <50%. Diastolic function was defined as abnormal if the diastolic pressure:volume quotient (lateral E:e'/end-diastolic volume) was >90th percentile (≥0.26 mL
). Patients were divided into: controls=normal EF and diastolic function; systolic dysfunction (SD) = abnormal EF with normal diastolic function; diastolic dysfunction (DD) = normal EF with abnormal diastolic pressure:volume quotient. Exercise function was quantified as percent predicted peak VO
. Physical Functioning Summary Score (FSS) was reported from the Child Health Questionnaire. A total of 239 patients were included, 177 (74%) control, 36 (15%) SD, and 26 (11%) DD. Median age was 12.2 (5.4) years. Arterial elastance, a measure of arterial stiffness, was higher in DD (3.6±1.1 mm Hg/mL) compared with controls (2.5±0.8 mm Hg/mL),
<0.01. DD patients had lower predicted peak VO
compared with controls (52% [20] versus 67% [23],
<0.01). Physical FSS was lower in DD (45±13) and SD (44±13) compared with controls (50±7),
<0.01. Conclusions Fontan patients with abnormal diastolic function and normal EF have decreased exercise tolerance, decreased functional health status, and elevated arterial stiffness. Identification of patients at high risk for HFpEF is feasible and should be considered when evaluating Fontan patients. |
|---|---|
| AbstractList | Background Heart failure phenotyping in single-ventricle Fontan patients is challenging, particularly in patients with normal ejection fraction (EF). The objective of this study was to identify Fontan patients with abnormal diastolic function, who are high risk for heart failure with preserved ejection fraction (HFpEF), and characterize their cardiac mechanics, exercise function, and functional health status. Methods and Results Data were obtained from the Pediatric Heart Network Fontan Cross-sectional Study database. EF was considered abnormal if <50%. Diastolic function was defined as abnormal if the diastolic pressure:volume quotient (lateral E:e'/end-diastolic volume) was >90th percentile (≥0.26 mL
). Patients were divided into: controls=normal EF and diastolic function; systolic dysfunction (SD) = abnormal EF with normal diastolic function; diastolic dysfunction (DD) = normal EF with abnormal diastolic pressure:volume quotient. Exercise function was quantified as percent predicted peak VO
. Physical Functioning Summary Score (FSS) was reported from the Child Health Questionnaire. A total of 239 patients were included, 177 (74%) control, 36 (15%) SD, and 26 (11%) DD. Median age was 12.2 (5.4) years. Arterial elastance, a measure of arterial stiffness, was higher in DD (3.6±1.1 mm Hg/mL) compared with controls (2.5±0.8 mm Hg/mL),
<0.01. DD patients had lower predicted peak VO
compared with controls (52% [20] versus 67% [23],
<0.01). Physical FSS was lower in DD (45±13) and SD (44±13) compared with controls (50±7),
<0.01. Conclusions Fontan patients with abnormal diastolic function and normal EF have decreased exercise tolerance, decreased functional health status, and elevated arterial stiffness. Identification of patients at high risk for HFpEF is feasible and should be considered when evaluating Fontan patients. Background Heart failure phenotyping in single-ventricle Fontan patients is challenging, particularly in patients with normal ejection fraction (EF). The objective of this study was to identify Fontan patients with abnormal diastolic function, who are high risk for heart failure with preserved ejection fraction (HFpEF), and characterize their cardiac mechanics, exercise function, and functional health status. Methods and Results Data were obtained from the Pediatric Heart Network Fontan Cross-sectional Study database. EF was considered abnormal if <50%. Diastolic function was defined as abnormal if the diastolic pressure:volume quotient (lateral E:e'/end-diastolic volume) was >90th percentile (≥0.26 mL-1). Patients were divided into: controls=normal EF and diastolic function; systolic dysfunction (SD) = abnormal EF with normal diastolic function; diastolic dysfunction (DD) = normal EF with abnormal diastolic pressure:volume quotient. Exercise function was quantified as percent predicted peak VO2. Physical Functioning Summary Score (FSS) was reported from the Child Health Questionnaire. A total of 239 patients were included, 177 (74%) control, 36 (15%) SD, and 26 (11%) DD. Median age was 12.2 (5.4) years. Arterial elastance, a measure of arterial stiffness, was higher in DD (3.6±1.1 mm Hg/mL) compared with controls (2.5±0.8 mm Hg/mL), P<0.01. DD patients had lower predicted peak VO2 compared with controls (52% [20] versus 67% [23], P<0.01). Physical FSS was lower in DD (45±13) and SD (44±13) compared with controls (50±7), P<0.01. Conclusions Fontan patients with abnormal diastolic function and normal EF have decreased exercise tolerance, decreased functional health status, and elevated arterial stiffness. Identification of patients at high risk for HFpEF is feasible and should be considered when evaluating Fontan patients.Background Heart failure phenotyping in single-ventricle Fontan patients is challenging, particularly in patients with normal ejection fraction (EF). The objective of this study was to identify Fontan patients with abnormal diastolic function, who are high risk for heart failure with preserved ejection fraction (HFpEF), and characterize their cardiac mechanics, exercise function, and functional health status. Methods and Results Data were obtained from the Pediatric Heart Network Fontan Cross-sectional Study database. EF was considered abnormal if <50%. Diastolic function was defined as abnormal if the diastolic pressure:volume quotient (lateral E:e'/end-diastolic volume) was >90th percentile (≥0.26 mL-1). Patients were divided into: controls=normal EF and diastolic function; systolic dysfunction (SD) = abnormal EF with normal diastolic function; diastolic dysfunction (DD) = normal EF with abnormal diastolic pressure:volume quotient. Exercise function was quantified as percent predicted peak VO2. Physical Functioning Summary Score (FSS) was reported from the Child Health Questionnaire. A total of 239 patients were included, 177 (74%) control, 36 (15%) SD, and 26 (11%) DD. Median age was 12.2 (5.4) years. Arterial elastance, a measure of arterial stiffness, was higher in DD (3.6±1.1 mm Hg/mL) compared with controls (2.5±0.8 mm Hg/mL), P<0.01. DD patients had lower predicted peak VO2 compared with controls (52% [20] versus 67% [23], P<0.01). Physical FSS was lower in DD (45±13) and SD (44±13) compared with controls (50±7), P<0.01. Conclusions Fontan patients with abnormal diastolic function and normal EF have decreased exercise tolerance, decreased functional health status, and elevated arterial stiffness. Identification of patients at high risk for HFpEF is feasible and should be considered when evaluating Fontan patients. Background Heart failure phenotyping in single‐ventricle Fontan patients is challenging, particularly in patients with normal ejection fraction (EF). The objective of this study was to identify Fontan patients with abnormal diastolic function, who are high risk for heart failure with preserved ejection fraction (HFpEF), and characterize their cardiac mechanics, exercise function, and functional health status. Methods and Results Data were obtained from the Pediatric Heart Network Fontan Cross‐sectional Study database. EF was considered abnormal if <50%. Diastolic function was defined as abnormal if the diastolic pressure:volume quotient (lateral E:e’/end‐diastolic volume) was >90th percentile (≥0.26 mL‐1). Patients were divided into: controls=normal EF and diastolic function; systolic dysfunction (SD) = abnormal EF with normal diastolic function; diastolic dysfunction (DD) = normal EF with abnormal diastolic pressure:volume quotient. Exercise function was quantified as percent predicted peak VO2. Physical Functioning Summary Score (FSS) was reported from the Child Health Questionnaire. A total of 239 patients were included, 177 (74%) control, 36 (15%) SD, and 26 (11%) DD. Median age was 12.2 (5.4) years. Arterial elastance, a measure of arterial stiffness, was higher in DD (3.6±1.1 mm Hg/mL) compared with controls (2.5±0.8 mm Hg/mL), P<0.01. DD patients had lower predicted peak VO2 compared with controls (52% [20] versus 67% [23], P<0.01). Physical FSS was lower in DD (45±13) and SD (44±13) compared with controls (50±7), P<0.01. Conclusions Fontan patients with abnormal diastolic function and normal EF have decreased exercise tolerance, decreased functional health status, and elevated arterial stiffness. Identification of patients at high risk for HFpEF is feasible and should be considered when evaluating Fontan patients. |
| Author | Chowdhury, Shahryar M. Zile, Michael R. Taylor, Carolyn L. McHugh, Kimberly E. Graham, Eric M. Nutting, Arni C. Atz, Andrew M. Savage, Andrew Gaydos, Stephanie |
| AuthorAffiliation | 1 Division of Cardiology Department of Pediatrics Medical University of South Carolina Charleston SC 2 Division of Cardiology Department of Medicine Medical University of South Carolina Charleston SC |
| AuthorAffiliation_xml | – name: 2 Division of Cardiology Department of Medicine Medical University of South Carolina Charleston SC – name: 1 Division of Cardiology Department of Pediatrics Medical University of South Carolina Charleston SC |
| Author_xml | – sequence: 1 givenname: Shahryar M. orcidid: 0000-0001-7685-7877 surname: Chowdhury fullname: Chowdhury, Shahryar M. organization: Division of Cardiology Department of Pediatrics Medical University of South Carolina Charleston SC – sequence: 2 givenname: Eric M. surname: Graham fullname: Graham, Eric M. organization: Division of Cardiology Department of Pediatrics Medical University of South Carolina Charleston SC – sequence: 3 givenname: Carolyn L. surname: Taylor fullname: Taylor, Carolyn L. organization: Division of Cardiology Department of Pediatrics Medical University of South Carolina Charleston SC – sequence: 4 givenname: Andrew surname: Savage fullname: Savage, Andrew organization: Division of Cardiology Department of Pediatrics Medical University of South Carolina Charleston SC – sequence: 5 givenname: Kimberly E. surname: McHugh fullname: McHugh, Kimberly E. organization: Division of Cardiology Department of Pediatrics Medical University of South Carolina Charleston SC – sequence: 6 givenname: Stephanie surname: Gaydos fullname: Gaydos, Stephanie organization: Division of Cardiology Department of Medicine Medical University of South Carolina Charleston SC – sequence: 7 givenname: Arni C. surname: Nutting fullname: Nutting, Arni C. organization: Division of Cardiology Department of Pediatrics Medical University of South Carolina Charleston SC – sequence: 8 givenname: Michael R. orcidid: 0000-0001-7076-221X surname: Zile fullname: Zile, Michael R. organization: Division of Cardiology Department of Medicine Medical University of South Carolina Charleston SC – sequence: 9 givenname: Andrew M. surname: Atz fullname: Atz, Andrew M. organization: Division of Cardiology Department of Pediatrics Medical University of South Carolina Charleston SC |
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| Cites_doi | 10.1016/j.echo.2016.02.016 10.1016/j.ijcard.2018.04.089 10.1161/CIRCULATIONAHA.119.044352 10.1016/j.jacc.2006.05.051 10.1136/hrt.2004.051789 10.1161/CIRCULATIONAHA.113.006191 10.1152/ajpheart.00184.2014 10.1111/chd.12358 10.1161/01.CIR.0000048123.22359.A0 10.1161/JAHA.117.006000 10.1161/CIRCULATIONAHA.106.659763 10.1017/S1047951113000632 10.1007/s10554-018-1440-z 10.1161/01.CIR.0000018621.96210.72 10.1007/s00467-005-2122-3 10.1139/h94-003 10.1007/s10741-020-09932-0 10.1016/j.jacc.2008.01.074 10.1017/S1047951119001859 10.1016/j.echo.2017.11.010 10.1016/j.ahj.2006.02.009 10.3389/fphys.2019.00638 10.1111/chd.12368 10.1007/s00392-015-0863-y 10.1016/j.amjcard.2009.03.058 10.1016/j.ijcard.2017.04.073 10.1007/s00246-014-0888-4 10.1161/hc3801.096326 10.1186/s12872-017-0705-0 10.1007/s10741-019-09904-z 10.1001/jamacardio.2019.5459 10.1161/CIRCULATIONAHA.117.029058 10.1016/j.echo.2016.07.016 10.1007/s00246-018-1819-6 10.1136/heartjnl-2015-309042 10.1161/CIRCIMAGING.112.000175 10.1016/j.jacc.2017.03.582 |
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| Keywords | heart failure diastolic function single ventricle Fontan |
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| Notes | ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23 For Sources of Funding and Disclosures, see page 7. An abstract describing the results of this study was presented at the American College of Cardiology Scientific Sessions, March 28–30, 2020. Supplemental Material for this article is available at https://www.ahajournals.org/doi/suppl/10.1161/JAHA.121.024095 |
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| Snippet | Background Heart failure phenotyping in single-ventricle Fontan patients is challenging, particularly in patients with normal ejection fraction (EF). The... Background Heart failure phenotyping in single‐ventricle Fontan patients is challenging, particularly in patients with normal ejection fraction (EF). The... |
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| SubjectTerms | Child Cross-Sectional Studies Diastole diastolic function Fontan Fontan Procedure - adverse effects Heart Failure Humans Original Research single ventricle Stroke Volume Ventricular Function, Left |
| Title | Diastolic Dysfunction With Preserved Ejection Fraction After the Fontan Procedure |
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