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Establishing Heart Rate Norms in Children with Fontan Palliation Using Holter Monitors
Peter Barrale1, Alex R Sigman2, Emily Pajazetovic3
1Division of Pediatric Cardiology, University of Iowa Health Care, Stead Family Children's Hospital.
Insights
This study establishes normative Holter heart rate data for Fontan patients, finding they do not have significantly lower minimum heart rates compared to healthy children. This provides a crucial reference for clinical evaluation.
Area of Science:
- Pediatric Cardiology
- Electrophysiology
- Congenital Heart Disease
Background:
- Fontan palliation increases risks of arrhythmias and sinus node dysfunction.
- Annual Holter monitoring is recommended but lacks normative data for Fontan patients.
- Existing data is limited, with only one study on healthy children.
Purpose of the Study:
- Establish normative Holter heart rate data for pediatric Fontan patients.
- Compare Holter data between Fontan patients and healthy children.
- Create a reference dataset for ambulatory ECG screening in Fontan physiology.
Main Methods:
- Retrospective, single-center analysis of 642 Holter monitors from 133 Fontan patients (aged 5-21).
- Exclusion criteria: pacemakers, significant AV valve regurgitation, or depressed ventricular function.
- Comparison of heart rate values with a cohort of 502 healthy controls.
Main Results:
- Fontan patients showed higher minimum HRs in adolescents and lower average HRs in younger children compared to controls.
- Fontan patients universally had lower maximum heart rates.
- Males and digoxin users had lower minimum HRs; heterotaxy correlated with higher minimum/average HRs.
Conclusions:
- This study provides the first normative Holter heart rate data for pediatric Fontan patients.
- Findings indicate Fontan patients do not exhibit significantly lower minimum heart rates.
- The data serves as a valuable reference for clinical assessment, highlighting the need for multicenter studies.
Background:
Fontan palliation is associated with an increased risk for arrhythmias and sinus node dysfunction. Although annual Holter monitoring is recommended for routine surveillance, there is no normative Holter data for this population, with only one study describing such data in healthy children.
Objective:
To describe Holter data for patients with Fontan physiology, compare this data to healthy children, and establish a normative data set for those undergoing routine ambulatory ECG screening.
Methods:
This retrospective, single-center study analyzed 642 Holter monitors from 133 Fontan patients aged 5-21 years, collected between 1970 and 2023. Patients with pacemakers, significant atrioventricular valve regurgitation, or severely depressed ventricular function were excluded. Demographic and clinical data were collected, and HR values were compared to a healthy control cohort.
Results:
The Fontan cohort was predominantly male (69%), with right ventricular dominance (59%) and extracardiac Fontan palliation (80%). Compared to 502 healthy controls, Fontan patients had significantly higher minimum HRs in adolescents (14-16 years), lower average HRs in younger age groups (4-12 years), and universally lower maximum HRs. Within the Fontan group, males and those on digoxin had lower minimum HRs, while heterotaxy was associated with higher minimum and average HRs.
Conclusion:
This is the first study to establish normative Holter HR data in pediatric Fontan patients and compare it to healthy counterparts. These findings suggest Fontan patients do not have significantly lower minimum heart rates and provide a valuable reference for clinical evaluation, supporting the need for larger, multicenter studies.

