{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T03:32:56Z","timestamp":1792812776263,"version":"4.5.0"},"reference-count":35,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"24","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2020,12,15]]},"abstract":"<jats:sec xml:lang=\"en\">\n            <jats:title>Background<\/jats:title>\n            <jats:p xml:lang=\"en\">The physiologic hallmarks of the Fontan circulation\u2014chronically elevated central venous pressures and low cardiac output\u2014have significant effects not only on cardiovascular status but also impact other organ systems. Exercise capacity is limited in many and declines with age, accelerating in adolescence, but with wide variability. We explore the relationship between exercise performance and end\u2010organ function in outpatient subjects with a Fontan circulation.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Methods and Results<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              This is a cross\u2010sectional analysis of subject end\u2010organ characterization from our outpatient Fontan circulation clinic with peak oxygen consumption (peak V\n              <jats:sc>o<\/jats:sc>\n              <jats:sub>2<\/jats:sub>\n              ) at cardiopulmonary exercise testing as the primary outcome. We perform linear regression to assess associations between clinical characteristics and peak V\n              <jats:sc>o<\/jats:sc>\n              <jats:sub>2<\/jats:sub>\n              as well as the magnitude of the association of clinical characteristics with peak V\n              <jats:sc>o<\/jats:sc>\n              <jats:sub>2<\/jats:sub>\n              . Of 265 subjects age 12.8 (9.5\u201316.4) years, there is a negative correlation between age and peak V\n              <jats:sc>o<\/jats:sc>\n              <jats:sub>2<\/jats:sub>\n              (\u22120.49,\n              <jats:italic>P<\/jats:italic>\n              &lt;0.001). Of those undergoing ramp cycle exercise testing, 34% perform above 80% predicted peak V\n              <jats:sc>o<\/jats:sc>\n              <jats:sub>2<\/jats:sub>\n              . Variables positively associated with peak V\n              <jats:sc>o<\/jats:sc>\n              <jats:sub>2<\/jats:sub>\n              and their effect size include vitamin D sufficiency (+3.00,\n              <jats:italic>P<\/jats:italic>\n              =0.020) and absolute lymphocyte count (+0.23,\n              <jats:italic>P<\/jats:italic>\n              =0.005). Status as overweight\/obese (\u22123.91,\n              <jats:italic>P<\/jats:italic>\n              =0.003) and hemoglobin (\u22120.77,\n              <jats:italic>P<\/jats:italic>\n              =0.003) are negatively associated. Neither ventricular morphology, timing of Fontan palliation, nor Fontan circulation type affect peak V\n              <jats:sc>o<\/jats:sc>\n              <jats:sub>2<\/jats:sub>\n              .\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              Higher peak V\n              <jats:sc>o<\/jats:sc>\n              <jats:sub>2<\/jats:sub>\n              in those with a Fontan circulation is associated with younger age, vitamin D sufficiency, absence of overweight\/obese, lower hemoglobin, and a healthier hepatic profile. Whether exercise training or other initiatives can modify organ characteristics in those with a Fontan circulation is worthy of exploration.\n            <\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/jaha.120.016850","type":"journal-article","created":{"date-parts":[[2020,12,2]],"date-time":"2020-12-02T13:27:12Z","timestamp":1606915632000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":45,"title":["End\u2010Organ Function and Exercise Performance in Patients With Fontan Circulation: What Characterizes the High Performers?"],"prefix":"10.1161","volume":"9","author":[{"given":"Scott J.","family":"Weinreb","sequence":"first","affiliation":[{"name":"Division of Cardiology Department of Pediatrics Children\u2019s Hospital of Philadelphia PA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathryn M.","family":"Dodds","sequence":"additional","affiliation":[{"name":"Division of Cardiology Department of Pediatrics Children\u2019s Hospital of Philadelphia PA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9870-7607","authenticated-orcid":false,"given":"Danielle S.","family":"Burstein","sequence":"additional","affiliation":[{"name":"Division of Cardiology Department of Pediatrics Children\u2019s Hospital of Philadelphia PA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jing","family":"Huang","sequence":"additional","affiliation":[{"name":"Department of Biostatistics, Epidemiology and Informatics University of Pennsylvania Perelman School of Medicine Philadelphia PA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth B.","family":"Rand","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology, Hepatology and Nutrition Department of Pediatrics Children\u2019s Hospital of Philadelphia PA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edna","family":"Mancilla","sequence":"additional","affiliation":[{"name":"Division of Endocrinology Department of Pediatrics Children\u2019s Hospital of Philadelphia PA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer R.","family":"Heimall","sequence":"additional","affiliation":[{"name":"Division of Allergy and Immunology Department of Pediatrics Children\u2019s Hospital of Philadelphia PA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael G.","family":"McBride","sequence":"additional","affiliation":[{"name":"Division of Cardiology Department of Pediatrics Children\u2019s Hospital of Philadelphia PA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen M.","family":"Paridon","sequence":"additional","affiliation":[{"name":"Division of Cardiology Department of Pediatrics Children\u2019s Hospital of Philadelphia 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