{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,4]],"date-time":"2026-09-04T17:19:49Z","timestamp":1788542389180,"version":"build-2803163510"},"reference-count":55,"publisher":"MDPI AG","issue":"14","license":[{"start":{"date-parts":[[2023,7,10]],"date-time":"2023-07-10T00:00:00Z","timestamp":1688947200000},"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>At rest, a good Fontan circulation can provide a normal cardiac output (CO). However, as a consequence of its unique hemodynamic nature, the limitations of the Fontan circuit are exposed during exercise. We aimed to provide a comprehensive assessment of the pathophysiology of exercise in adult Fontan patients (FPs) and identify factors limiting their functional capacity (FC). In a single-center study conducted in 37 FPs aged \u226516 years and 19 healthy-controls (HCs) who underwent CPET on a cycle ergometer in February and March 2022, the mean peakVO2 was 21 \u00b1 5.4 mL\/kg\/min, which was 55% of the predicted value. Morphologically, the left single ventricle showed a higher peakVO2% predicted value (57.4 \u00b1 14.4% vs. 43.4 \u00b1 8.1%, p = 0.045). The factors associated with low peakVO2 values were an early flattened or descending O2 pulse at maximal exertion (52 \u00b1 14% vs. 62 \u00b1 12.5, p = 0.04 and 47.6 \u00b1 9% vs. 60 \u00b1 14, p = 0.018, respectively) and chronotropic insufficiency (53 \u00b1 12% vs. 69.8 \u00b1 20%, p = 0.008). The OUES was found to be a useful parameter to assess the FC in FPs in maximal and submaximal exercise testing. A strong positive correlation was observed between the %OUES and peakVO2%predicted (r = 0.726, p &gt; 0.001). The lung function was impaired in the FPs, mostly with a mild restrictive pattern (56.8%). The FPs showed lower inspiratory muscle strength compared to the HCs but it was not statistically associated with either the peakVO2 or VE\/VCO2 slope. Regular intense physical activity improves one\u2019s FC. Although FPs have inspiratory muscle weakness, its impact on their FC is unclear. The peakVO2% predicted grew progressively higher as the level of physical activity increased (low level 49.5 \u00b1 14%, moderate level 55 \u00b1 12%, intense level 69 \u00b1 20%).<\/jats:p>","DOI":"10.3390\/jcm12144593","type":"journal-article","created":{"date-parts":[[2023,7,11]],"date-time":"2023-07-11T01:58:14Z","timestamp":1689040694000},"page":"4593","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Respiratory Muscle Function and Exercise Performance in Adult Patients with Fontan Circulation"],"prefix":"10.3390","volume":"12","author":[{"given":"Raquel","family":"Ladr\u00f3n-Abia","sequence":"first","affiliation":[{"name":"Adult Congenital Heart Disease Unit, Hospital Universitario Virgen del Roc\u00edo, Instituto de BioMedicina de Sevilla (IBIS), Avenida Manuel Siurot s\/n, 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pilar","family":"Cejudo Ramos","sequence":"additional","affiliation":[{"name":"Medical-Surgical Service of Respiratory Diseases, Hospital Universitario Virgen del Roc\u00edo, Instituto de BioMedicina de Sevilla (IBIS), 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5661-9900","authenticated-orcid":false,"given":"Israel","family":"Valverde P\u00e9rez","sequence":"additional","affiliation":[{"name":"Paediatric Cardiology Unit, Hospital Infantil Virgen del Roc\u00edo, Instituto de BioMedicina de Sevilla (IBIS), 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0701-5048","authenticated-orcid":false,"given":"Roc\u00edo","family":"Camacho Fern\u00e1ndez De Liger","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Disease Unit, Hospital Universitario Virgen del Roc\u00edo, Instituto de BioMedicina de Sevilla (IBIS), Avenida Manuel Siurot s\/n, 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amadeo-Jos\u00e9","family":"Wals-Rodr\u00edguez","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Disease Unit, Hospital Universitario Virgen del Roc\u00edo, Instituto de BioMedicina de Sevilla (IBIS), Avenida Manuel Siurot s\/n, 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mar\u00eda Jos\u00e9","family":"Rodr\u00edguez Puras","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Disease Unit, Hospital Universitario Virgen del Roc\u00edo, Instituto de BioMedicina de Sevilla (IBIS), Avenida Manuel Siurot s\/n, 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bego\u00f1a","family":"Manso Garc\u00eda","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Disease Unit, Hospital Universitario Virgen del Roc\u00edo, Instituto de BioMedicina de Sevilla (IBIS), Avenida Manuel Siurot s\/n, 41013 Sevilla, Spain"},{"name":"Paediatric Cardiology Unit, Hospital Infantil Virgen del Roc\u00edo, Instituto de BioMedicina de Sevilla (IBIS), 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pastora","family":"Gallego","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Disease Unit, Hospital Universitario Virgen del Roc\u00edo, Instituto de BioMedicina de Sevilla (IBIS), Avenida Manuel Siurot s\/n, 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,7,10]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1700","DOI":"10.1016\/j.jacc.2015.07.065","article-title":"40-year follow-up after the Fontan operation: Long-term outcomes of 1052 patients","volume":"66","author":"Pundi","year":"2015","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"2755","DOI":"10.1016\/j.jacc.2020.10.003","article-title":"Hemodynamic and Clinical Implications of Impaired Pulmonary Vascular Reserve in the Fontan Circulation","volume":"76","author":"Egbe","year":"2020","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1186\/s12968-018-0505-4","article-title":"Maldistribution of pulmonary blood flow in patients after the Fontan operation is associated with worse exercise capacity","volume":"20","author":"Alsaied","year":"2018","journal-title":"J. Cardiovasc. Magn. Reson."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1081","DOI":"10.1136\/heartjnl-2015-307467","article-title":"The Fontan circulation after 45\u2005years: Update in physiology","volume":"102","author":"Gewilling","year":"2016","journal-title":"Heart"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1017\/S1047951106000345","article-title":"Reduced pulmonary function in children with the Fontan circulation affects their exercise capacity","volume":"16","author":"Matthews","year":"2006","journal-title":"Cardiol. Young"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"981","DOI":"10.1007\/s00246-017-1606-9","article-title":"Impaired Pulmonary Function is an Additional Potential Mechanism for the Reduction of Functional Capacity in Clinically Stable Fontan Patients","volume":"38","author":"Turquetto","year":"2017","journal-title":"Pediatr. Cardiol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"H110","DOI":"10.1152\/ajpheart.00184.2014","article-title":"Abnormal spirometry after the Fontan procedure is common and associated with impaired aerobic capacity","volume":"307","author":"Opotowsky","year":"2014","journal-title":"Am. J. Physiol. Circ. Physiol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1111\/chd.12694","article-title":"A restrictive ventilatory pattern is common in patients with univentricular heart after Fontan palliation and associated with a reduced exercise capacity and quality of life","volume":"14","author":"Callegari","year":"2019","journal-title":"Congenit. Heart Dis."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"882","DOI":"10.1161\/CIRCULATIONAHA.112.126755","article-title":"Abnormal Lung Function in Adults With Congenital Heart Disease: Prevalence, Relation to Cardiac Anatomy, and Association With Survival","volume":"127","author":"Borgia","year":"2013","journal-title":"Circulation"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1530","DOI":"10.1136\/heartjnl-2013-304249","article-title":"Skeletal muscle abnormalities and exercise capacity in adults with a Fontan circulation","volume":"99","author":"Cordina","year":"2013","journal-title":"Heart"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1702","DOI":"10.1136\/heartjnl-2014-305723","article-title":"Lean mass deficits, vitamin D status and exercise capacity in children and young adults after Fontan palliation","volume":"100","author":"Avitabile","year":"2014","journal-title":"Heart"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"683849","DOI":"10.3389\/fcvm.2021.683849","article-title":"The Influence of Respiration on Blood Flow in the Fontan Circulation: Insights for Imaging-Based Clinical Evaluation of the Total Cavopulmonary Connection","volume":"8","author":"Rijnberg","year":"2021","journal-title":"Front. Cardiovasc. Med."},{"key":"ref_13","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":"Ali","year":"2018","journal-title":"Pediatr. Cardiol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"462","DOI":"10.1053\/j.semtcvs.2018.07.014","article-title":"A Pilot Study of Inspiratory Muscle Training to Improve Exercise Capacity in Patients with Fontan Physiology","volume":"30","author":"Wu","year":"2018","journal-title":"Semin. Thorac. Cardiovasc. Surg."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1016\/j.ijcard.2020.01.015","article-title":"Inspiratory muscle training did not improve exercise capacity and lung function in adult patients with Fontan circulation: A randomized controlled trial","volume":"305","author":"Fritz","year":"2020","journal-title":"Int. J. Cardiol."},{"key":"ref_16","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_17","doi-asserted-by":"crossref","first-page":"29","DOI":"10.20517\/2574-1209.2021.98","article-title":"Cardiopulmonary exercise test as a tool in surveillance after Fontan operation","volume":"6","author":"Doshi","year":"2022","journal-title":"Vessel. Plus"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"6","DOI":"10.1016\/j.ijcard.2017.02.140","article-title":"Cardiopulmonary exercise test in adults with prior Fontan operation: The prognostic value of serial testing","volume":"235","author":"Egbe","year":"2017","journal-title":"Int. J. Cardiol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"3073","DOI":"10.1093\/eurheartj\/ehq356","article-title":"Predictors of morbidity and mortality in contemporary Fontan patients: Results from a multicenter study including cardiopulmonary exercise testing in 321 patients","volume":"31","author":"Diller","year":"2010","journal-title":"Eur. Heart J."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"294","DOI":"10.1111\/j.1747-0803.2011.00500.x","article-title":"Exercise Testing Identifies Patients at Increased Risk for Morbidity and Mortality Following Fontan Surgery","volume":"6","author":"Fernandes","year":"2011","journal-title":"Congenit. Heart Dis."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1249\/MSS.0b013e31829f8326","article-title":"Prognostic Value of Submaximal Exercise Data for Cardiac Morbidity in Fontan Patients","volume":"46","author":"Chen","year":"2014","journal-title":"Med. Sci. Sports Exerc."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1016\/j.amjcard.2021.05.049","article-title":"Prognostic Value of Maximal and Submaximal Exercise Performance in Fontan Patients < 15 Years of Age","volume":"154","author":"Monteros","year":"2021","journal-title":"Am. J. Cardiol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1079\/PHN2005898","article-title":"The International Physical Activity Questionnaire (IPAQ): A study of concurrent and construct validity","volume":"9","author":"Oja","year":"2006","journal-title":"Public Health Nutr."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"e70","DOI":"10.1164\/rccm.201908-1590ST","article-title":"Standardization of Spirometry 2019 Update. An Official American Thoracic Society and European Respiratory Society Technical Statement","volume":"200","author":"Graham","year":"2019","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1324","DOI":"10.1183\/09031936.00080312","article-title":"Multi-ethnic reference values for spirometry for the 3\u201395-yr age range: The global lung function 2012 equations","volume":"40","author":"Quanjer","year":"2012","journal-title":"Eur. Respir. J."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"1801214","DOI":"10.1183\/13993003.01214-2018","article-title":"ERS statement on respiratory muscle testing at rest and during exercise","volume":"53","author":"Laveneziana","year":"2019","journal-title":"Eur. Respir. J."},{"key":"ref_27","first-page":"1348","article-title":"The assessment of maximal respiratory mouth pressures in adults","volume":"54","author":"Evans","year":"2009","journal-title":"Respir. Care"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1136\/thx.50.4.371","article-title":"Maximal values of sniff nasal inspiratory pressure in healthy subjects","volume":"50","author":"Uldry","year":"1995","journal-title":"Thorax"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1164\/ajrccm.159.1.9804052","article-title":"Sniff Nasal Inspiratory Pressure","volume":"159","author":"Ti","year":"1999","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_30","unstructured":"Wasserman, K., James, E.H., Darryl, Y.S., William, W.S., Kathy, E.S., Sun, X.-G., and Brian, J.W. (2012). Principles of Exercise Testing and Interpretation. Including Pathophysiology and Clinical Applications, Lippincott Williams & Wilkins. [5th ed.]."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1177\/2047487314547658","article-title":"The oxygen uptake efficiency slope in 1411 Caucasian healthy men and women aged 20\u201360 years: Reference values","volume":"22","author":"Buys","year":"2015","journal-title":"Eur. J. Prev. Cardiol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1123\/pes.22.3.431","article-title":"Oxygen uptake efficiency slope in healthy children","volume":"22","author":"Akkerman","year":"2010","journal-title":"Pediatr. Exerc. Sci."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"1424","DOI":"10.1016\/S0735-1097(10)80034-8","article-title":"Exercise responses in patients with congenital heart disease after fontan repair: Patterns and determinants of performance","volume":"15","author":"Gewillig","year":"1990","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1016\/j.hfc.2013.09.010","article-title":"Failure of the Fontan Circulation","volume":"10","author":"Gewillig","year":"2014","journal-title":"Heart Fail. Clin."},{"key":"ref_35","doi-asserted-by":"crossref","unstructured":"Ladr\u00f3n-Abia, R., Manso Garc\u00eda, B., Cejudo Ramos, P., Gaboli, M., Rodriguez Puras, M.J., and Gallego, P. (2023). Differences between cycle ergometer and treadmill in cardiopulmonary exercise testing in patients with Fontan circulation. Rev. Espa\u00f1ola Cardiol., in press.","DOI":"10.1016\/j.rec.2023.03.024"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1386","DOI":"10.1093\/eurheartj\/ehr461","article-title":"Reference values for exercise limitations among adults with congenital heart disease. Relation to activities of daily life--single centre experience and review of published data","volume":"33","author":"Kempny","year":"2012","journal-title":"Eur. Heart J."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"1967","DOI":"10.1016\/S0735-1097(01)01266-9","article-title":"Influence of ventricular morphology on aerobic exercise capacity in patients after the Fontan operation","volume":"37","author":"Ohuchi","year":"2001","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1177\/21501351221087695","article-title":"Cardiopulmonary Exercise Test Outcomes in Fontan Patients with Right versus Left Single Ventricle Morphology","volume":"13","author":"Dhauna","year":"2022","journal-title":"World J. Pediatr. Congenit. Heart Surg."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1016\/j.ijcard.2007.11.092","article-title":"Accuracy of oxygen uptake efficiency slope in adults with congenital heart disease","volume":"133","author":"Giardini","year":"2009","journal-title":"Int. J. Cardiol."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1097\/HCR.0b013e3181ebf316","article-title":"The Oxygen Uptake Efficiency Slope","volume":"30","author":"Akkerman","year":"2010","journal-title":"J. Cardiopulm. Rehabil. Prev."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"1301","DOI":"10.1007\/s00246-012-0308-6","article-title":"Oxygen Pulse Kinetics in Fontan Patients During Treadmill Ramp Protocol Cardiopulmonary Exercise Testing","volume":"33","author":"Bansal","year":"2012","journal-title":"Pediatr. Cardiol."},{"key":"ref_42","first-page":"252","article-title":"Dobutamine stress echocardiography for the evaluation of cardiac reserve late after Fontan operation","volume":"48","author":"Brili","year":"2007","journal-title":"Hell. J. Cardiol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1016\/j.ijcard.2016.12.087","article-title":"Pressure\u2013volume loop-derived cardiac indices during dobutamine stress: A step towards understanding limitations in cardiac output in children with hypoplastic left heart syndrome","volume":"230","author":"Wong","year":"2017","journal-title":"Int. J. Cardiol."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1657","DOI":"10.1016\/j.amjcard.2008.01.050","article-title":"Usefulness of Cardiac Magnetic Resonance Imaging Combined with Low-Dose Dobutamine Stress to Detect an Abnormal Ventricular Stress Response in Children and Young Adults After Fontan Operation at Young Age","volume":"101","author":"Harkel","year":"2008","journal-title":"Am. J. Cardiol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1080\/4017430310001221","article-title":"Haemodynamic Adaptation during Exercise in Fontan Patients at a Long-Term Follow-Up","volume":"37","author":"Larsson","year":"2003","journal-title":"Scand. Cardiovasc. J."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1017\/S1047951105001629","article-title":"Cardiopulmonary response to exercise in patients with the Fontan circulation","volume":"15","author":"Ohuchi","year":"2005","journal-title":"Cardiol. Young"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/j.ijcard.2008.01.032","article-title":"Impaired heart rate response to exercise in adult patients with a systemic right ventricle or univentricular circulation: Prevalence, relation to exercise, and potential therapeutic implications","volume":"134","author":"Diller","year":"2009","journal-title":"Int. J. Cardiol."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"e012008","DOI":"10.1161\/JAHA.119.012008","article-title":"Heart Rate Reserve in Fontan Patients: Chronotropic Incompetence or Hemodynamic Limitation?","volume":"8","author":"Claessen","year":"2019","journal-title":"J. Am. Heart Assoc."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1016\/j.ijcha.2018.11.005","article-title":"Adults with congenital heart disease overestimate their physical activity level","volume":"22","author":"Larsson","year":"2018","journal-title":"IJC Heart Vasc."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"764273","DOI":"10.3389\/fcvm.2021.764273","article-title":"The \u201cSuper-Fontan\u201d Phenotype: Characterizing Factors Associated with High Physical Performance","volume":"8","author":"Tran","year":"2021","journal-title":"Front. Cardiovasc. Med."},{"key":"ref_51","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_52","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1016\/j.hlc.2019.09.010","article-title":"Management of People with a Fontan Circulation: A Cardiac Society of Australia and New Zealand Position statement","volume":"29","author":"Zentner","year":"2020","journal-title":"Heart Lung Circ."},{"key":"ref_53","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":"2020","journal-title":"Eur. J. Prev. Cardiol."},{"key":"ref_54","doi-asserted-by":"crossref","unstructured":"Perrone, M.A., Pomiato, E., Palmieri, R., Di Gi\u00e0, G., Piemonte, F., Porzio, O., and Gagliardi, M.G. (2022). The Effects of Exercise Training on Cardiopulmonary Exercise Testing and Cardiac Biomarkers in Adult Patients with Hypoplastic Left Heart Syndrome and Fontan Circulation. J. Cardiovasc. Dev. Dis., 9.","DOI":"10.3390\/jcdd9060171"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"1164","DOI":"10.1136\/hrt.2010.213579","article-title":"Generalised muscle weakness in young adults with congenital heart disease","volume":"97","author":"Greutmann","year":"2011","journal-title":"Heart"}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/12\/14\/4593\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T20:10:05Z","timestamp":1760127005000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/12\/14\/4593"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,7,10]]},"references-count":55,"journal-issue":{"issue":"14","published-online":{"date-parts":[[2023,7]]}},"alternative-id":["jcm12144593"],"URL":"https:\/\/doi.org\/10.3390\/jcm12144593","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,7,10]]}}}