{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T14:27:50Z","timestamp":1787495270684,"version":"build-2736575974"},"reference-count":30,"publisher":"BMJ","issue":"6","funder":[{"DOI":"10.13039\/100000097","name":"National Center for Research Resources","doi-asserted-by":"publisher","award":["UL1 TR001102"],"award-info":[{"award-number":["UL1 TR001102"]}],"id":[{"id":"10.13039\/100000097","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["Heart"],"accepted":{"date-parts":[[2016,8,25]]},"published-print":{"date-parts":[[2017,3]]},"abstract":"<jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To define whether adults with a Fontan circulation, who have lifelong venous congestion and limited cardiac output, have impaired glomerular filtration rate (GFR) or elevated urinary biomarkers of kidney injury.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We measured circulating cystatin C and creatinine (n=70) and urinary creatinine, albumin, kidney injury molecule-1 (KIM-1), neutrophil gelatinase-associated lipocalin (NGAL) and N-acetyl glucosaminidase (NAG) (n=59) in ambulatory adult Fontan patients and 20 age-matched and sex-matched controls. Urinary biomarkers were normalised to urine creatinine concentration. Survival free from non-elective cardiovascular hospitalisation was compared by estimated GFR and urinary biomarker levels using survival analysis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Cystatin C GFR was lower in the Fontan group compared with controls (114.2\u00b122.8 vs 136.3\u00b112.8\u2005mL\/min\/1.73\u2005m\n                      <jats:sup>2<\/jats:sup>\n                      , p&lt;0.0001); GFR&lt;90\u2005mL\/min\/1.73\u2005m\n                      <jats:sup>2<\/jats:sup>\n                      in 14.3% vs 0% of controls. Albumin-to-creatinine ratio (ACR), KIM-1 and NAG were elevated compared with controls; ACR=23.2 (7.6\u201338.3) vs 3.6 (2.5\u20135.7) mg\/g, p&lt;0.0001; NAG=1.8 (1.1\u20132.6) vs 1.1 (0.9\u20131.6) U\/g, p=0.02; KIM-1=0.91 (0.52\u20131.45) vs 0.33 (0.24\u20130.74) ng\/mg, p=0.001. Microalbuminuria, ACR&gt;30\u2005mg\/g, was present in 33.9% of the Fontan patients but in none of the controls. Over median 707 (IQR 371\u2013942)-day follow-up, 31.4% of patients had a clinical event. Higher KIM-1 and NAG were associated with higher risk of non-elective hospitalisation or death (HR\/+1 SD=2.1, 95% CI 1.3 to 3.3, p=0.002; HR\/+1 SD=1.6, 95% CI 1.05 to 2.4, p=0.03, respectively); cystatin C GFR was associated with risk of the outcome (HR\/+1 SD=0.66, 95% CI 0.48 to 0.90, p=0.009) but creatinine-based GFR was not (HR\/+1 SD=0.91, 95% CI 0.61 to 1.38, p=0.66). Neither ACR nor NGAL was associated with events.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The Fontan circulation is commonly associated with reduced estimated GFR and evidence for glomerular and tubular injury. Those with lower cystatin C GFR and tubular injury are at increased risk of adverse outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1136\/heartjnl-2016-309729","type":"journal-article","created":{"date-parts":[[2016,9,26]],"date-time":"2016-09-26T23:38:41Z","timestamp":1474933121000},"page":"434-442","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":67,"title":["Estimated glomerular filtration rate and urine biomarkers in patients with single-ventricle Fontan circulation"],"prefix":"10.1136","volume":"103","author":[{"given":"Alexander R","family":"Opotowsky","sequence":"first","affiliation":[{"name":"Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts, USA"},{"name":"Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fernando R","family":"Baraona","sequence":"additional","affiliation":[{"name":"Divisi\u00f3n de Enfermedades Cardiovasculares, Facultad de Medicina, Pontificia Universidad Cat\u00f3lica de Chile, Santiago, Chile"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Finnian R","family":"Mc Causland","sequence":"additional","affiliation":[{"name":"Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brittani","family":"Loukas","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth","family":"Landzberg","sequence":"additional","affiliation":[{"name":"Columbia University Medical Center, New York, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael J","family":"Landzberg","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts, USA"},{"name":"Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Venkata","family":"Sabbisetti","sequence":"additional","affiliation":[{"name":"Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sushrut S","family":"Waikar","sequence":"additional","affiliation":[{"name":"Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2016,9,26]]},"reference":[{"key":"2025092005183984000_103.6.434.1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2008.07.051"},{"key":"2025092005183984000_103.6.434.2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.0000116764.53225.A9"},{"key":"2025092005183984000_103.6.434.3","doi-asserted-by":"publisher","DOI":"10.1093\/eurjhf\/hfr123"},{"key":"2025092005183984000_103.6.434.4","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.109.933275"},{"key":"2025092005183984000_103.6.434.5","doi-asserted-by":"publisher","DOI":"10.1053\/ajkd.2002.34487"},{"key":"2025092005183984000_103.6.434.6","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1114248"},{"key":"2025092005183984000_103.6.434.7","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2007.11.079"},{"key":"2025092005183984000_103.6.434.8","doi-asserted-by":"publisher","DOI":"10.1093\/ndt\/gft510"},{"key":"2025092005183984000_103.6.434.9","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2013070758"},{"key":"2025092005183984000_103.6.434.10","doi-asserted-by":"crossref","first-page":"481520","DOI":"10.1155\/2012\/481520","article-title":"Mechanisms 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S , Ruebner RL , Furth SL , et al . Assessment of kidney function in survivors following Fontan palliation. Congenit Heart Dis Published Online First: 22 Apr 2016. doi:10.1111\/chd.12358 doi:10.1111\/chd.12358","DOI":"10.1111\/chd.12358"},{"key":"2025092005183984000_103.6.434.22","doi-asserted-by":"publisher","DOI":"10.1136\/heartjnl-2013-304249"},{"key":"2025092005183984000_103.6.434.23","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.107.734921"},{"key":"2025092005183984000_103.6.434.24","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1214234"},{"key":"2025092005183984000_103.6.434.25","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2016.02.042"},{"key":"2025092005183984000_103.6.434.26","doi-asserted-by":"publisher","DOI":"10.1053\/j.ajkd.2014.01.432"},{"key":"2025092005183984000_103.6.434.27","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2013010019"},{"key":"2025092005183984000_103.6.434.28","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2006080882"},{"key":"2025092005183984000_103.6.434.29","doi-asserted-by":"publisher","DOI":"10.1258\/acb.2011.011105"},{"key":"2025092005183984000_103.6.434.30","doi-asserted-by":"publisher","DOI":"10.1053\/j.ajkd.2009.01.009"}],"container-title":["Heart"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/heartjnl-2016-309729","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,20]],"date-time":"2025-09-20T12:18:47Z","timestamp":1758370727000},"score":1,"resource":{"primary":{"URL":"https:\/\/heart.bmj.com\/lookup\/doi\/10.1136\/heartjnl-2016-309729"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,9,26]]},"references-count":30,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2017,2,27]]},"published-print":{"date-parts":[[2017,3]]}},"alternative-id":["10.1136\/heartjnl-2016-309729"],"URL":"https:\/\/doi.org\/10.1136\/heartjnl-2016-309729","relation":{"has-review":[{"id-type":"doi","id":"10.3410\/f.726781429.793547133","asserted-by":"object"}]},"ISSN":["1355-6037","1468-201X"],"issn-type":[{"value":"1355-6037","type":"print"},{"value":"1468-201X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,9,26]]}}}