{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,28]],"date-time":"2025-11-28T12:01:18Z","timestamp":1764331278427,"version":"build-2065373602"},"reference-count":34,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2024,9,24]],"date-time":"2024-09-24T00:00:00Z","timestamp":1727136000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicina"],"abstract":"<jats:p>Background and Objectives: The Fontan procedure, a palliative surgery for univentricular heart physiology, often reduces exercise capacity and quality of life. This study aimed to evaluate the impact of cardiac rehabilitation (CR) on improving outcomes in Fontan patients to inform evidence-based care. Materials and Methods: Fontan patients aged 8\u201330 participated in a structured CR program for at least three months. The program included weekly aerobic and resistance training sessions with educational and nutritional guidance. Baseline and post-CR assessments included cardiac function, fitness, daily activity, and health-related quality of life (HRQOL). Results: The cohort included ten Fontan patients, of whom six had a right systemic ventricle. CR significantly improved cardiorespiratory fitness, as seen in VO2 max (from 27.92 \u00b1 5.15 to 34.69 \u00b1 1.14 mL\/kg\/min, p = 0.0089) and percent predicted VO2 (from 0.67 \u00b1 0.18 to 0.90 \u00b1 0.02, p = 0.005). VCO2 increased by +8.68 \u00b1 8.59 mL\/kg\/min but did not reach statistical significance (p = 0.05). Most haemodynamic and ventilatory parameters showed no significant improvement. All the SF-36 questionnaire domains showed significant HRQOL gains (p &lt; 0.001). High adherence (85\u201393%), no adverse events, and reduced NT-proBNP levels supported the program\u2019s safety. Conclusions: This study\u2019s findings have important implications for the care of Fontan patients. CR significantly improved exercise capacity and HRQOL in Fontan patients across various anatomies, particularly in the right systemic ventricle. Integrating physical conditioning into standard care could reduce long-term morbidity and mortality; however, further research is needed to refine the protocols and confirm sustained benefits.<\/jats:p>","DOI":"10.3390\/medicina60101566","type":"journal-article","created":{"date-parts":[[2024,9,24]],"date-time":"2024-09-24T08:54:13Z","timestamp":1727168053000},"page":"1566","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["The Effect of Cardiac Rehabilitation in Paediatric Fontan Circulation Patients: A Prospective Intervention Study"],"prefix":"10.3390","volume":"60","author":[{"given":"Luna van de","family":"Ven","sequence":"first","affiliation":[{"name":"Department of Paediatric Cardiology, Children\u2019s Hospital, University Medical Center Utrecht, P.O. Box 85090, 3508 Utrecht, The Netherlands"}]},{"given":"Ana Clara","family":"F\u00e9lix","sequence":"additional","affiliation":[{"name":"Pediatric Cardiology Department, Hospital de Santa Marta, Unidade Local de Sa\u00fade S\u00e3o Jos\u00e9 EPE, 1150-293 Lisbon, Portugal"},{"name":"Cl\u00ednica Universit\u00e1ria de Cardiologia Pedi\u00e1trica, Centro Cl\u00ednico Acad\u00e9mico de Lisboa, 1150-293 Lisbon, Portugal"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9597-4689","authenticated-orcid":false,"given":"Joana","family":"Suarez","sequence":"additional","affiliation":[{"name":"Pediatric Cardiology Department, Hospital de Santa Marta, Unidade Local de Sa\u00fade S\u00e3o Jos\u00e9 EPE, 1150-293 Lisbon, Portugal"},{"name":"Cl\u00ednica Universit\u00e1ria de Cardiologia Pedi\u00e1trica, Centro Cl\u00ednico Acad\u00e9mico de Lisboa, 1150-293 Lisbon, Portugal"}]},{"given":"Bruno","family":"Rodrigues","sequence":"additional","affiliation":[{"name":"Physical and Medical Rehabilitation Department, Hospital de Santa Marta, Unidade Local de Sa\u00fade S\u00e3o Jos\u00e9 EPE, 1150-293 Lisbon, Portugal"}]},{"given":"Jorge","family":"Dias","sequence":"additional","affiliation":[{"name":"Physical and Medical Rehabilitation Department, Hospital de Santa Marta, Unidade Local de Sa\u00fade S\u00e3o Jos\u00e9 EPE, 1150-293 Lisbon, Portugal"}]},{"given":"F\u00e1tima F.","family":"Pinto","sequence":"additional","affiliation":[{"name":"Pediatric Cardiology Department, Hospital de Santa Marta, Unidade Local de Sa\u00fade S\u00e3o Jos\u00e9 EPE, 1150-293 Lisbon, Portugal"},{"name":"Cl\u00ednica Universit\u00e1ria de Cardiologia Pedi\u00e1trica, Centro Cl\u00ednico Acad\u00e9mico de Lisboa, 1150-293 Lisbon, Portugal"},{"name":"Comprehensive Health Research Center, NOVA Medical School, Faculdade de Ci\u00eancias M\u00e9dicas, NMS, FCM, Universidade NOVA de Lisboa, 1169-056 Lisbon, Portugal"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2169-4024","authenticated-orcid":false,"given":"S\u00e9rgio","family":"Laranjo","sequence":"additional","affiliation":[{"name":"Pediatric Cardiology Department, Hospital de Santa Marta, Unidade Local de Sa\u00fade S\u00e3o Jos\u00e9 EPE, 1150-293 Lisbon, Portugal"},{"name":"Cl\u00ednica Universit\u00e1ria de Cardiologia Pedi\u00e1trica, Centro Cl\u00ednico Acad\u00e9mico de Lisboa, 1150-293 Lisbon, Portugal"},{"name":"Comprehensive Health Research Center, NOVA Medical School, Faculdade de Ci\u00eancias M\u00e9dicas, NMS, FCM, Universidade NOVA de Lisboa, 1169-056 Lisbon, Portugal"}]}],"member":"1968","published-online":{"date-parts":[[2024,9,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1111\/chd.12570","article-title":"Five decades of the Fontan operation: A systematic review of international reports on outcomes after univentricular palliation","volume":"13","author":"Kverneland","year":"2018","journal-title":"Congenit. Heart Dis."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1016\/j.ijcard.2016.05.035","article-title":"The Fontan epidemic: Population projections from the Australia and New Zealand Fontan registry","volume":"219","author":"Schilling","year":"2016","journal-title":"Int. J. Cardiol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"e234","DOI":"10.1161\/CIR.0000000000000696","article-title":"Evaluation and management of the child and adult with Fontan circulation: A scientific statement from the American Heart Association","volume":"140","author":"Rychik","year":"2019","journal-title":"Circulation"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1016\/j.jtcvs.2017.01.056","article-title":"Long-term survival after the Fontan operation: Twenty years of experience at a single center","volume":"154","author":"Downing","year":"2017","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_5","unstructured":"Mazza, G.A., Gribaudo, E., and Agnoletti, G. (2021). The pathophysiology and complications of Fontan circulation. Acta Bio-Medica Atenei Parm., 92."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"839","DOI":"10.1136\/hrt.2004.051789","article-title":"The fontan circulation","volume":"91","author":"Gewillig","year":"2005","journal-title":"Heart"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"824","DOI":"10.1017\/S1047951113001649","article-title":"Exercise capacity in the Fontan circulation","volume":"23","author":"Goldberg","year":"2013","journal-title":"Cardiol. Young"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1269","DOI":"10.1177\/2047487320942869","article-title":"Physical exercise training in patients with a Fontan circulation: A systematic review","volume":"28","author":"Scheffers","year":"2021","journal-title":"Eur. J. Prev. Cardiol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"509","DOI":"10.1136\/adc.2006.105239","article-title":"Physical activity levels in children and adolescents are reduced after the Fontan procedure, independent of exercise capacity, and are associated with lower perceived general health","volume":"92","author":"McCrindle","year":"2007","journal-title":"Arch. Dis. Child."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1394","DOI":"10.1016\/j.athoracsur.2013.10.079","article-title":"Cerebral circulation in patients with Fontan circulation: Assessment by carotid arterial wave intensity and stiffness","volume":"97","author":"Saiki","year":"2014","journal-title":"Ann. Thorac. Surg."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1053\/pcsu.2002.29716","article-title":"Thromboembolic problems after the Fontan operation","volume":"5","author":"Monagle","year":"2002","journal-title":"Semin. Thorac. Cardiovasc. Surg. Pediatr. Card. Surg. Annu."},{"key":"ref_12","first-page":"2672","article-title":"The adult with a fontan: A panacea without a cure? Review of long-term complications","volume":"77","author":"Lastinger","year":"2013","journal-title":"Circ. J. Off. J. Jpn. Circ. Soc."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"2434","DOI":"10.1016\/j.jacc.2023.04.036","article-title":"Pulmonary Complications in Patients With Fontan Circulation: JACC Review Topic of the Week","volume":"81","author":"Abdulkarim","year":"2023","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1299","DOI":"10.1017\/S1047951118001166","article-title":"Home- and hospital-based exercise training programme after Fontan surgery","volume":"28","author":"Sutherland","year":"2018","journal-title":"Cardiol. Young"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1016\/j.ijcard.2021.01.058","article-title":"Aerobic exercise and inspiratory muscle training increase functional capacity in patients with univentricular physiology after Fontan operation: A randomized controlled trial","volume":"330","author":"Turquetto","year":"2021","journal-title":"Int. J. Cardiol."},{"key":"ref_16","doi-asserted-by":"crossref","unstructured":"Dirks, S., Kramer, P., Schleiger, A., Speck, H.M., Wolfarth, B., Thouet, T., Berger, F., Sallmon, H., and Ovroutski, S. (2021). Home-Based Long-Term Physical Endurance and Inspiratory Muscle Training for Children and Adults With Fontan Circulation-Initial Results From a Prospective Study. Front. Cardiovasc. Med., 8.","DOI":"10.3389\/fcvm.2021.784648"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"e027464","DOI":"10.1161\/JAHA.122.027464","article-title":"Peak Work Rate Increases with Lower Extremity-Focused Exercise Training in Adolescents with Fontan Circulation","volume":"11","author":"Avitabile","year":"2022","journal-title":"J. Am. Heart Assoc."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"780","DOI":"10.1016\/j.ijcard.2012.10.012","article-title":"Resistance training improves cardiac output, exercise capacity and tolerance to positive airway pressure in Fontan physiology","volume":"168","author":"Cordina","year":"2013","journal-title":"Int. J. Cardiol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"732","DOI":"10.1017\/S104795111800015X","article-title":"Lower limb exercise generates pulsatile flow into the pulmonary vascular bed in the setting of the Fontan circulation","volume":"28","author":"Cordina","year":"2018","journal-title":"Cardiol. Young"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/j.cjcpc.2022.05.001","article-title":"Importance of Physical Activity and Exercise in Paediatric Fontan Patients","volume":"1","author":"Longmuir","year":"2022","journal-title":"CJC Pediatr. Congenit. Hear. Dis."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1097\/00005650-199401000-00004","article-title":"The MOS 36-item Short-Form Health Survey (SF-36): III. Tests of data quality, scaling assumptions, and reliability across diverse patient groups","volume":"32","author":"McHorney","year":"1994","journal-title":"Med. Care"},{"key":"ref_22","unstructured":"Pi\u00f1a, I.L. (2024, August 10). Exercise Capacity and VO2 in Heart Failure. UpToDate. Available online: https:\/\/www.uptodate.com\/contents\/exercise-capacity-and-vo2-in-heart-failure?topicRef=3472&source=see_link."},{"key":"ref_23","first-page":"757","article-title":"The maximum oxygen intake. An international reference standard of cardiorespiratory fitness","volume":"38","author":"Shephard","year":"1968","journal-title":"Bull. World Health Organ."},{"key":"ref_24","doi-asserted-by":"crossref","unstructured":"ATS\/ACCP (2003). Statement on cardiopulmonary exercise testing. Am. J. Respir. Crit. Care Med., 167, 211\u2013277.","DOI":"10.1164\/rccm.167.2.211"},{"key":"ref_25","first-page":"219","article-title":"Excercise physiology in health and disease","volume":"112","author":"Wasserman","year":"1975","journal-title":"Am. Rev. Respir. Dis."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"S49","DOI":"10.1055\/s-2004-830515","article-title":"Applications of cardiopulmonary exercise testing in the management of cardiovascular and pulmonary disease","volume":"26","author":"Myers","year":"2005","journal-title":"Int. J. Sports Med."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"695","DOI":"10.1007\/s00246-018-1808-9","article-title":"Respiratory Training Late After Fontan Intervention: Impact on Cardiorespiratory Performance","volume":"39","author":"Pingitore","year":"2018","journal-title":"Pediatr. Cardiol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"606","DOI":"10.1016\/j.ahj.2015.06.018","article-title":"Does exercise training improve cardiopulmonary fitness and daily physical activity in children and young adults with corrected tetralogy of Fallot or Fontan circulation? A randomized controlled trial","volume":"170","author":"Duppen","year":"2015","journal-title":"Am. Heart J."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"e000902","DOI":"10.1136\/openhrt-2018-000902","article-title":"Survival in patients with univentricular hearts: The impact of right versus left ventricular morphology","volume":"5","author":"Erikssen","year":"2018","journal-title":"Open Heart"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1016\/j.jacc.2008.01.074","article-title":"Contemporary outcomes after the Fontan procedure: A Pediatric Heart Network multicenter study","volume":"52","author":"Anderson","year":"2008","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_31","first-page":"S32","article-title":"Redefining expectations of long-term survival after the Fontan procedure: Twenty-five years of follow-up from the entire population of Australia and New Zealand","volume":"130","author":"Iyengar","year":"2014","journal-title":"Circulation"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"438","DOI":"10.1017\/S1047951117002360","article-title":"Can endurance training improve physical capacity and quality of life in young Fontan patients?","volume":"28","author":"Hedlund","year":"2018","journal-title":"Cardiol Young"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1093\/eurjpc\/zwad286","article-title":"Leg-focused high-weight resistance training improves ventricular stroke volume, exercise capacity and strength in young patients with a Fontan circulation","volume":"31","author":"Scheffers","year":"2023","journal-title":"Eur. J. Prev. Cardiol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1016\/j.jpeds.2018.03.050","article-title":"Pulmonary Screening in Subjects after the Fontan Procedure","volume":"199","author":"Liptzin","year":"2018","journal-title":"J. Pediatr."}],"container-title":["Medicina"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1648-9144\/60\/10\/1566\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T16:01:40Z","timestamp":1760112100000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1648-9144\/60\/10\/1566"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,9,24]]},"references-count":34,"journal-issue":{"issue":"10","published-online":{"date-parts":[[2024,10]]}},"alternative-id":["medicina60101566"],"URL":"https:\/\/doi.org\/10.3390\/medicina60101566","relation":{},"ISSN":["1648-9144"],"issn-type":[{"type":"electronic","value":"1648-9144"}],"subject":[],"published":{"date-parts":[[2024,9,24]]}}}