{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T23:04:37Z","timestamp":1792191877724,"version":"4.4.0"},"reference-count":31,"publisher":"MDPI AG","issue":"7","license":[{"start":{"date-parts":[[2024,4,2]],"date-time":"2024-04-02T00:00:00Z","timestamp":1712016000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: Heart rate variability (HRV) is an established, non-invasive parameter for the assessment of cardiac autonomic nervous activity and the health status in general cardiology. However, there are few studies on HRV in adults with congenital heart defects (CHDs). The aim of the present study was to evaluate the use of long-term continuous HRV measurement for the assessment of global health status in adults with cyanotic CHD. Methods: This prospective study included 45 adults (40% female, mean age = 35.2 \u00b1 9.2 [range: 19\u201358] years) after cardiac surgical repair. HRV parameters were calculated from continuous 24 h measurements using a Bittium Faros 180 sensor (Bittium Corp., Oulu, Finland). Results: Postoperative patients with transposition of the great arteries (TGA) (n = 18) achieved significantly higher values of standard deviation of NN intervals (SDNN) (175.4 \u00b1 59.9 ms vs. 133.5 \u00b1 40.6 ms; p = 0.013) compared with patients with other conotruncal anomalies (n = 22). Comparing patients with TGA after a Senning\u2013Brom or Mustard operation (n = 13) with all other heart surgery patients (n = 32), significantly higher HRV parameters were found after atrial switch (root mean square of successive RR interval differences: 53.6 \u00b1 20.7 ms vs. 38.4 \u00b1 18.3 ms; p = 0.019; SDNN: 183.5 \u00b1 58.4 ms vs. 136.3 \u00b1 45.3 ms; p = 0.006). A higher SDNN was also measured after Senning\u2013Brom or Mustard operations than after a Rastelli operations (n = 2) (SDNN: 183.5 \u00b1 58.4 ms vs. 84.5 \u00b1 5.2 ms; p = 0.037). When comparing atrial switch operations (n = 3) with Rastelli operations, the SDNN value was significantly shorter in the Rastelli group (p = 0.004). Conclusions: Our results suggest that continuous HRV monitoring may serve as a marker of cardiac autonomic dysfunction in adults with cyanotic CHD after surgical repair. Impaired cardiac autonomic nervous activity may be associated with an increased risk of adverse reactions in patients with repaired CHD. Therefore, a longitudinal assessment of HRV patterns and trends may provide a deeper insight into dynamic changes in their autonomic regulation and disease progression, lifestyle changes, or treatments. As each person has individual variability in heart rate, HRV may be useful in assessing intra-individual disease progression and may help to improve personalized medicine. Further studies are needed to better understand the underlying mechanisms and to explore the full potential of HRV analysis to optimize medical care for ACHDs.<\/jats:p>","DOI":"10.3390\/jcm13072062","type":"journal-article","created":{"date-parts":[[2024,4,2]],"date-time":"2024-04-02T10:58:31Z","timestamp":1712055511000},"page":"2062","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Continuous Long-Term Assessment of Heart Rate Variability in Adults with Cyanotic Congenital Heart Disease after Surgical Repair"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1111-5789","authenticated-orcid":false,"given":"Felix","family":"Pieringer","sequence":"first","affiliation":[{"name":"Department of Cardiac Surgery, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, 91054 Erlangen, Germany"},{"name":"International Center for Adults with Congenital Heart Disease, Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technical University Munich, 80636 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9541-9888","authenticated-orcid":false,"given":"Mathieu N.","family":"Suleiman","sequence":"additional","affiliation":[{"name":"Department of Cardiac Surgery, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, 91054 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ann-Sophie","family":"Kaemmerer-Suleiman","sequence":"additional","affiliation":[{"name":"Department of Cardiac Surgery, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, 91054 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5216-7978","authenticated-orcid":false,"given":"Oliver","family":"Dewald","sequence":"additional","affiliation":[{"name":"Department of Cardiac Surgery, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, 91054 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annika","family":"Freiberger","sequence":"additional","affiliation":[{"name":"International Center for Adults with Congenital Heart Disease, Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technical University Munich, 80636 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Huntgeburth","sequence":"additional","affiliation":[{"name":"International Center for Adults with Congenital Heart Disease, Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technical University Munich, 80636 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"Nagdyman","sequence":"additional","affiliation":[{"name":"International Center for Adults with Congenital Heart Disease, Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technical University Munich, 80636 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rhoia","family":"Neidenbach","sequence":"additional","affiliation":[{"name":"International Center for Adults with Congenital Heart Disease, Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technical University Munich, 80636 Munich, Germany"},{"name":"Department of Sport and Health Sciences, Technical University Munich, 80992 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3431-0862","authenticated-orcid":false,"given":"Fabian","family":"von Scheidt","sequence":"additional","affiliation":[{"name":"International Center for Adults with Congenital Heart Disease, Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technical University Munich, 80636 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harald","family":"Kaemmerer","sequence":"additional","affiliation":[{"name":"International Center for Adults with Congenital Heart Disease, Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technical University Munich, 80636 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0253-1190","authenticated-orcid":false,"given":"Peter","family":"Ewert","sequence":"additional","affiliation":[{"name":"International Center for Adults with Congenital Heart Disease, Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technical University Munich, 80636 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Weyand","sequence":"additional","affiliation":[{"name":"Department of Cardiac Surgery, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, 91054 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sebastian","family":"Freilinger","sequence":"additional","affiliation":[{"name":"International Center for Adults with Congenital Heart Disease, Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technical University Munich, 80636 Munich, Germany"},{"name":"Department of Sport and Health Sciences, Technical University Munich, 80992 Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1817-4207","authenticated-orcid":false,"given":"Frank","family":"Harig","sequence":"additional","affiliation":[{"name":"Department of Cardiac Surgery, University Hospital Erlangen, Friedrich-Alexander-University Erlangen-N\u00fcrnberg, 91054 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,4,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"e017704","DOI":"10.1161\/JAHA.120.017704","article-title":"Survival in Children With Congenital Heart Disease: Have We Reached a Peak at 97%?","volume":"9","author":"Mandalenakis","year":"2020","journal-title":"J. Am. Heart Assoc."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"317","DOI":"10.2217\/fca-2019-0061","article-title":"Diagnosis and treatment of adults with congenital heart disease","volume":"16","author":"Henning","year":"2020","journal-title":"Future Cardiol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1161\/CIRCULATIONAHA.106.627224","article-title":"Congenital heart disease in the general population: Changing prevalence and age distribution","volume":"115","author":"Marelli","year":"2007","journal-title":"Circulation"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"2637","DOI":"10.1161\/01.CIR.103.21.2637","article-title":"Challenges posed by adults with repaired congenital heart disease","volume":"103","author":"Perloff","year":"2001","journal-title":"Circulation"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"423","DOI":"10.21037\/cdt.2018.03.11","article-title":"Non-cardiac comorbidities in adults with inherited and congenital heart disease: Report from a single center experience of more than 800 consecutive patients","volume":"8","author":"Neidenbach","year":"2018","journal-title":"Cardiovasc. Diagn. Ther."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"518","DOI":"10.21037\/cdt-20-359","article-title":"Provision of medical health care for adults with congenital heart disease associated with aortic involvement","volume":"11","author":"Kaemmerer","year":"2021","journal-title":"Cardiovasc. Diagn. Ther."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"799","DOI":"10.21037\/cdt.2018.12.06","article-title":"Current research status on the psychological situation of adults with congenital heart disease","volume":"8","author":"Andonian","year":"2018","journal-title":"Cardiovasc. Diagn. Ther."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"481","DOI":"10.21037\/cdt-20-825","article-title":"Systematic Assessment of Health Care Perception in Adults with Congenital Heart Disease in Germany","volume":"11","author":"Neidenbach","year":"2021","journal-title":"Cardiovasc. Diagn. Ther."},{"key":"ref_9","first-page":"E637","article-title":"2018 AHA\/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary","volume":"139","author":"Stout","year":"2019","journal-title":"Circulation"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"787","DOI":"10.21037\/cdt-22-383","article-title":"The relation of aortic dimensions and obesity in adults with Marfan or Loeys-Dietz syndrome","volume":"12","author":"Suleiman","year":"2022","journal-title":"Cardiovasc. Diagn. Ther."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"e002302","DOI":"10.1136\/openhrt-2023-002302","article-title":"Continuous long-term heart rate variability and risk assessment in pulmonary hypertension","volume":"10","author":"Andersen","year":"2023","journal-title":"Open Heart"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1093\/ejcts\/ezab269","article-title":"Heart rate variability in congenital heart disease: Looking and learning","volume":"61","author":"Boris","year":"2021","journal-title":"Eur. J. Cardiothorac. Surg."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"258","DOI":"10.3389\/fpubh.2017.00258","article-title":"An Overview of Heart Rate Variability Metrics and Norms","volume":"5","author":"Shaffer","year":"2017","journal-title":"Front. Public Health"},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"Nederend, I., Jongbloed, M.R.M., de Geus, E.J.C., Blom, N.A., and Ten Harkel, A.D.J. (2016). Postnatal Cardiac Autonomic Nervous Control in Pediatric Congenital Heart Disease. J. Cardiovasc. Dev. Dis., 3.","DOI":"10.3390\/jcdd3020016"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1043","DOI":"10.1161\/01.CIR.93.5.1043","article-title":"Heart rate variability: Standards of measurement, physiological interpretation and clinical use. Task Force of the European Society of Cardiology and the North American Society of Pacing and Electrophysiology","volume":"93","author":"Camm","year":"1996","journal-title":"Circulation"},{"key":"ref_16","doi-asserted-by":"crossref","unstructured":"Ok\u00f3lska, M., \u0141ach, J., Matusik, P.T., Paj\u0105k, J., Mroczek, T., Podolec, P., and Tomkiewicz-Paj\u0105k, L. (2021). Heart Rate Variability and Its Associations with Organ Complications in Adults after Fontan Operation. J. Clin. Med., 10.","DOI":"10.3390\/jcm10194492"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"647","DOI":"10.1016\/j.jtcvs.2006.03.057","article-title":"Impaired cardiac autonomic nervous activity predicts sudden cardiac death in patients with operated and unoperated congenital cardiac disease","volume":"132","author":"Lammers","year":"2006","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1007\/s12181-023-00617-0","article-title":"\u201cErwachsene mit angeborenen Herzfehlern\u201d: Aktuelle Herausforderung in der medizinischen Versorgung Teil II: Infekti\u00f6se Endokarditis, pulmonale Hypertonie, Aortopathien, herzchirurgische Aspekte, Psychokardiologie, ungel\u00f6ste Probleme und Zukunftsaspekte","volume":"17","author":"Kaemmerer","year":"2023","journal-title":"Die Kardiol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"967","DOI":"10.1007\/s004310050979","article-title":"Clinical and haemodynamic correlates of heart rate variability in children with congenital heart disease","volume":"157","author":"Massin","year":"1998","journal-title":"Eur. J. Pediatr."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1654","DOI":"10.1016\/S0002-9149(99)00173-3","article-title":"Heart rate variability in healthy children and in those with congenital heart disease both before and after operation","volume":"83","author":"Heragu","year":"1999","journal-title":"Am. J. Cardiol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"2732","DOI":"10.1161\/01.CIR.102.22.2732","article-title":"Abnormal cardiac autonomic nervous activity after right ventricular outflow tract reconstruction","volume":"102","author":"Ohuchi","year":"2000","journal-title":"Circulation"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1510\/icvts.2008.194555","article-title":"Impaired cardiac autonomic nervous control after cardiac bypass surgery for congenital heart disease","volume":"9","author":"McGlone","year":"2009","journal-title":"Interact. Cardiovasc. Thorac. Surg."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1111\/j.1747-0803.2012.00664.x","article-title":"Vascular and autonomic function in preschool-aged children with congenital heart disease","volume":"7","author":"Currie","year":"2012","journal-title":"Congenit. Heart Dis."},{"key":"ref_24","doi-asserted-by":"crossref","unstructured":"Aletti, F., Ferrario, M., de Jesus, T.B., Stirbulov, R., Silva, A.B., Cerutti, S., and Sampaio, L.M. (September, January 28). Heart rate variability in children with cyanotic and acyanotic congenital heart disease: Analysis by spectral and non linear indices. Proceedings of the 2012 Annual International Conference of the IEEE Engineering in Medicine and Biology Society, San Diego, CA, USA.","DOI":"10.1109\/EMBC.2012.6346890"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"102696","DOI":"10.1016\/j.autneu.2020.102696","article-title":"Association between reduced heart rate variability components and supraventricular tachyarrhythmias in patients with a systemic right ventricle","volume":"227","author":"Zandstra","year":"2020","journal-title":"Auton. Neurosci."},{"key":"ref_26","first-page":"461","article-title":"Successful Anatomic Correction of Transposition of the Great Vessels. A preliminary Report","volume":"28","author":"Jatene","year":"1975","journal-title":"Arq. Bras. Cardiol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"3918","DOI":"10.1038\/ncomms4918","article-title":"Venous endothelin guides sympathetic innervation of the developing mouse heart","volume":"5","author":"Manousiouthakis","year":"2014","journal-title":"Nat. Commun."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"2414","DOI":"10.1161\/01.CIR.97.24.2414","article-title":"Sympathetic denervation and reinnervation after arterial switch operation for complete transposition","volume":"97","author":"Kondo","year":"1998","journal-title":"Circulation"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"304","DOI":"10.1007\/s00059-004-2501-8","article-title":"Patients operated for tetralogy of fallot and with non-sustained ventricular tachycardia have reduced heart rate variability","volume":"29","author":"Butera","year":"2004","journal-title":"Herz"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"1085","DOI":"10.1016\/j.amjcard.2008.05.065","article-title":"Autonomic heart rate control at rest and during unloading of the right ventricle in repaired tetralogy of Fallot in adolescents","volume":"102","author":"Wyller","year":"2008","journal-title":"Am. J. Cardiol."},{"key":"ref_31","first-page":"98","article-title":"Heart rate variability in adult patients with congenital heart disease","volume":"1","author":"Trojnarska","year":"2005","journal-title":"Arch. Med. Sci."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/7\/2062\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T14:22:32Z","timestamp":1760106152000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/13\/7\/2062"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,4,2]]},"references-count":31,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2024,4]]}},"alternative-id":["jcm13072062"],"URL":"https:\/\/doi.org\/10.3390\/jcm13072062","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,4,2]]}}}