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
Main Authors: Chowdhury, Shahryar M., Graham, Eric M., Taylor, Carolyn L., Savage, Andrew, McHugh, Kimberly E., Gaydos, Stephanie, Nutting, Arni C., Zile, Michael R., Atz, Andrew M.
Format: Journal Article
Language:English
Published: England John Wiley and Sons Inc 18.01.2022
Wiley
Subjects:
Child
Cross-Sectional Studies
Diastole
diastolic function
Fontan
Fontan Procedure - adverse effects
Heart Failure
Humans
Original Research
single ventricle
Stroke Volume
Ventricular Function, Left
heart failure
diastolic function
single ventricle
Fontan
ISSN:2047-9980, 2047-9980
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Summary: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.
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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
ISSN:2047-9980
2047-9980
DOI:10.1161/JAHA.121.024095