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Heart rate variability and pacemaker treatment in children with Fontan circulation
Umeå University, Faculty of Medicine, Department of Clinical Sciences, Paediatrics.
2019 (English)Doctoral thesis, comprehensive summary (Other academic)
Abstract [en]

Background: Fontan surgery is performed in children with univentricular heart defects. Arrhythmias are frequent complications, occasionally requiring pacemaker treatment. Previous data regarding indications and risk factors for pacemaker treatment in Fontan patients is limited and conflicting. Heart rate variability (HRV) reflects autonomous nervous activity controlling the sinus node and has been associated with tachyarrhythmias in both adults and children, as well as in adults with sinus node dysfunction (SND).

Aim: To study HRV, arrhythmia and pacemaker treatment  in children with Fontan circulation— with the purpose of contributing to the reduction of long term complications in this patient group.

Methods: We have retrospectively reviewed pacemaker therapy in all Swedish patients who underwent Fontan surgery from 1982 to 2017 (n=599). We have also analysed HRV from 24-hour Holter ECG recordings in 112 children with Fontan circulation and in children with univentricular heart defects before bidirectional Glenn (BDG) procedure (n=47), before and on completion of Fontan surgery (n=47 and 45 respectively). Analysis was performed by power spectral analysis and Poincaré method, and results compared with healthy controls. Furthermore, HRV was analysed in Fontan patients who later required a pacemaker due to severe SND. Results were compared with Fontan patients who had SND, without indication for pacemaker treatment, with patients with Fontan circulation without SND and healthy controls. In addition we evaluated the possibility to analyse arrhythmias and HRV in 27 Fontan children using intermittent ECG recordings with a handheld devices at home during a 14-day period.

Results: After a mean follow-up of 12 years, 13% (78/599) of patients with Fontan circulation had received a pacemaker. Patients operated with the extracardiac conduit (EC) had a significantly lower prevalence of pacemaker implantation (6%) than patients with a lateral tunnel (LT) (17%). The most common pacemaker indication in patients with Fontan circulation was SND (64%). Children with Fontan circulation showed significant reductions in several HRV parameters, compared with controls. No significant differences were found between patients operated with LT versus EC (paper I). After BDG the RR interval and SD2 (representing changes in heart rate over 24-hours) significantly increased compared to pre-BDG. Compared with healthy controls, patients post-BDG, had significantly longer RR intervals and reduced overall HRV. PHF (reflecting parasympathetic control of the heart) was significantly reduced after TCPC as compared to before (paper II). Fontan patients with SND showed significantly elevated SD2 (representing changes in heart rate over 24-hours), somewhat reduced in patients that later required a pacemaker (Paper V). Handheld ECG analysis revealed frequent ventricular extra systoles in one patient and episodes of supraventricular tachycardia in another. Seven Fontan patients showed reduced HRV recorded with the handheld device over a 14-day period (paper III).

Conclusions: Overall HRV was reduced in patients with univentricular heart defects during the different surgical stages of Fontan surgery, compared to healthy controls. HRV was reduced in both patients with LT and EC with no significant difference between them. After BDG heart rate was significantly reduced as compared to before. PHF, reflecting the parasympathetic innervation of the heart was reduced after as compared to before TCPC. Pacemaker treatment is commonly needed in patients with Fontan circulation, and SND was the most prevalent indication for implantation. The prevalence of Fontan patients requiring pacemaker treatment was significantly lower in patients with EC. HRV analysis can contribute to management when following-up patients with Fontan circulation.

 

 

Place, publisher, year, edition, pages
Umeå: Umeå universitet , 2019. , p. 85
Series
Umeå University medical dissertations, ISSN 0346-6612 ; 2022
Keywords [en]
Congenital heart disease, Fontan circulation, heart rate variability, pacemaker, sinus node dysfunction
National Category
Pediatrics
Research subject
Pediatrics; Cardiology
Identifiers
URN: urn:nbn:se:umu:diva-156727ISBN: 978-91-7855-007-4 (print)OAI: oai:DiVA.org:umu-156727DiVA, id: diva2:1291657
Public defence
2019-03-22, Sal D, Unod T9, Norrlands universitetssjukhus, Umeå, 09:00 (English)
Opponent
Supervisors
Available from: 2019-03-01 Created: 2019-02-25 Last updated: 2019-02-27Bibliographically approved
List of papers
1. Heart rate variability in children with fontan circulation: lateral tunnel and extracardiac conduit
Open this publication in new window or tab >>Heart rate variability in children with fontan circulation: lateral tunnel and extracardiac conduit
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2012 (English)In: Pediatric Cardiology, ISSN 0172-0643, E-ISSN 1432-1971, Vol. 33, no 2, p. 307-315Article in journal (Refereed) Published
Abstract [en]

The technique in Fontan surgery has developed from the lateral tunnel (LT) toward the extracardiac conduit (EC) used to reduce long-term complications such as atrial arrhythmia and sinus node dysfunction. Heart rate variability (HRV) examines cardiac nervous activity controlling the sinus node. This study aimed to investigate HRV in a cohort of children with univentricular hearts, focusing on the relation between HRV and surgical procedure. For 112 children with Fontan circulation, HRV was analyzed using power spectral analysis. Spectral power was determined in three regions: very-low-frequency (VLF), low-frequency (LF), and high-frequency (HF) regions. Patients were compared with 66 healthy controls subject. Patients with LT were compared with patients who had EC. The children with Fontan circulation showed a significantly reduced HRV including total power (P < 0.0001), VLF (P < 0.0001), LF (P < 0.0001), and HF (P = 0.001) compared with the control subjects. The LT and EC patients did not differ significantly. Reduced HRV was found in both the LT and EC patients. In terms of HRV reduction, EC was not superior to LT.

Place, publisher, year, edition, pages
New York: Springer-Verlag New York, 2012
Keywords
Extracardiac conduit, Fontan circulation, Heart rate variability, Lateral tunnel
National Category
Pediatrics
Identifiers
urn:nbn:se:umu:diva-48274 (URN)10.1007/s00246-011-0126-2 (DOI)000299505500016 ()21984213 (PubMedID)
Available from: 2011-10-13 Created: 2011-10-13 Last updated: 2019-02-25Bibliographically approved
2. Changes in heart rate variability during surgical stages to completed Fontan circulation
Open this publication in new window or tab >>Changes in heart rate variability during surgical stages to completed Fontan circulation
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(English)Manuscript (preprint) (Other academic)
Abstract [en]

Objectives: In patients with Fontan circulation, arrhythmia is a serious complication contributing to morbidity and mortality. Arrhythmia is related to heart rate variability (HRV), which reflects autonomic nervous regulation of the heart. Our hypothesis was that autonomic nervous ganglia, located at the junction of the superior vena cava’s entrance to the heart, may be affected during the bidirectional Glenn procedure (BDG), resulting in reduced HRV.

Methods: 24-hour Holter ECG recordings were obtained before BDG (n=47), after BDG (n=47) and after total cavopulmonary connection (TCPC) (n=45) in patients, and in 38 healthy controls. HRV was analysed by spectral and Poincaré methods. Age-related z-scores were calculated and compared using linear mixed effects modeling.

Results: HRV-parameters (Ptot, PVLF, PLF, PHF, PLF/PHF, SD2, and SD1/SD2) were significantly lower in patients before BDG when compared to healthy controls. The RR interval and SD2 were significantly increased in patients post  BDG compared to pre BDG. Compared to healthy controls; patients operated with BDG had significantly longer RR intervals and reduced Ptot and PLF. Patients post TCPC showed longer RR intervals, Ptot, PVLF, PLF, and PHF,  SD1, and SD1/SD2 compared with healthy controls. In patients post TCPC; Ptot, PVLF, PLF, PHF, PLF/PHF, SD2, and SD1/SD2 were decreased compared to pre TCPC.

Conclusions: Heart rate was reduced after BDG procedure, and further reductions of HRV were seen post-TCPC. Our results indicate that autonomic regulation of cardiac rhythm is affected both after BDG and again after TCPC. This may be reflected as, and contribute to, postoperative arrhythmic events.

Keywords
Fontan hjärtfrekvensvariabilitet
National Category
Pediatrics
Research subject
Cardiology; Pediatrics
Identifiers
urn:nbn:se:umu:diva-156713 (URN)
Funder
Swedish Heart Lung FoundationVästerbotten County Council
Available from: 2019-02-25 Created: 2019-02-25 Last updated: 2019-03-11
3. Handheld ECG in analysis of arrhythmia and heart rate variability in children with Fontan circulation
Open this publication in new window or tab >>Handheld ECG in analysis of arrhythmia and heart rate variability in children with Fontan circulation
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2014 (English)In: Journal of Electrocardiology, ISSN 0022-0736, E-ISSN 1532-8430, Vol. 47, no 3, p. 374-382Article in journal (Refereed) Published
Abstract [en]

Background: Our aim was to evaluate the intermittent use of a handheld ECG system for detecting silent arrhythmias and cardiac autonomic dysfunction in children with univentricular hearts. Methods: Twenty-seven patients performed intermittent ECG recordings with handheld devices during a 14-day period. A manual arrhythmia analysis was performed. We analyzed heart rate variability (HRV) using scatter plots of all interbeat intervals (Poincare plots) from the total observation period. Reference values of HRV indices were determined from Holter-ECGs in 41 healthy children. Results: One asymptomatic patient had frequent ventricular extra systoles. Another patient had episodes with supraventricular tachycardia (with concomitant palpitations). Seven patients showed reduced HRV. Conclusions: Asymptomatic arrhythmia was detected in one patient. The proposed method for pooling of intermittent recordings from handheld or similar devices may be used for detection of arrhythmias as well as for cardiac autonomic dysfunction.

Keywords
Handheld ECG, Arrhythmia, Heart rate variability, Fontan circulation
National Category
Cardiac and Cardiovascular Systems
Identifiers
urn:nbn:se:umu:diva-90080 (URN)10.1016/j.jelectrocard.2014.02.006 (DOI)000335712700018 ()
Available from: 2014-06-23 Created: 2014-06-17 Last updated: 2019-02-25Bibliographically approved
4. Pacemaker treatment after Fontan surgery: a Swedish national study
Open this publication in new window or tab >>Pacemaker treatment after Fontan surgery: a Swedish national study
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2019 (English)In: Congenital Heart Disease, ISSN 1747-079X, E-ISSN 1747-0803Article in journal (Other academic) Epub ahead of print
Abstract [en]

Objective: Fontan surgery is performed in children with univentricular heart defects. Previous data regarding permanent pacemaker implantation frequency and indications in Fontan patients are limited and conflicting. We examined the prevalence of and risk factors for pacemaker treatment in a consecutive national cohort of patients after Fontan surgery in Sweden.

Methods: We retrospectively reviewed all Swedish patients who underwent Fontan surgery from 1982 to 2017 (n = 599).

Results: After a mean follow‐up of 12.2 years, 13% (78/599) of the patients with Fontan circulation had received pacemakers. Patients operated with the extracardiac conduit (EC) type of total cavopulmonary connection had a significantly lower prevalence of pacemaker implantation (6%) than patients with lateral tunnel (LT; 17%). Mortality did not differ between patients with (8%) and without pacemaker (5%). The most common pacemaker indication was sinus node dysfunction (SND) (64%). Pacemaker implantation due to SND was less common among patients with EC. Pacemaker implantation was significantly more common in patients with mitral atresia (MA; 44%), double outlet right ventricle (DORV; 24%) and double inlet left ventricle (DILV; 20%). In contrast, patients with pulmonary atresia with intact ventricular septum and hypoplastic left heart syndrome were significantly less likely to receive a pacemaker (3% and 6%, respectively).

Conclusions: Thirteen percent of Fontan patients received a permanent pacemaker, most frequently due to SND. EC was associated with a significantly lower prevalence of pacemaker than LT. Permanent pacemaker was more common in patients with MA, DORV, and DILV.

Place, publisher, year, edition, pages
Wiley-Blackwell, 2019
Keywords
Congenital heart disease, Fontan circulation, pacemaker treatment, sinus node dysfunction
National Category
Pediatrics
Research subject
Pediatrics; Cardiology
Identifiers
urn:nbn:se:umu:diva-156716 (URN)10.1111/chd.12766 (DOI)
Funder
Västerbotten County CouncilSwedish Heart Lung Foundation
Note

Originally included in thesis in manuscript form.

Available from: 2019-02-25 Created: 2019-02-25 Last updated: 2019-03-19
5. Sinus node dysfunction in patients with Fontan circulation: could heart rate variability be a predictor for pacemaker implantation?
Open this publication in new window or tab >>Sinus node dysfunction in patients with Fontan circulation: could heart rate variability be a predictor for pacemaker implantation?
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(English)Manuscript (preprint) (Other academic)
Keywords
Congenital heart disease, Fontan circulation, Pacemaker, Sinus node dysfunction, Heart rate variability, Poincaré analysis
National Category
Pediatrics
Research subject
Pediatrics; Cardiology
Identifiers
urn:nbn:se:umu:diva-156717 (URN)
Funder
Västerbotten County CouncilSwedish Heart Lung Foundation
Available from: 2019-02-25 Created: 2019-02-25 Last updated: 2019-03-19

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Alenius Dahlqvist, Jenny

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