title: Serum Bile Acids in Repaired Tetralogy of Fallot: A Marker for Liver and Heart?
abstract: OBJECTIVE
  Patients with repaired tetralogy of Fallot may develop chronic right ventricular dysfunction and hepatic congestion over time. We hypothesized that bile acid metabolism is altered in repaired tetralogy of Fallot patients and therefore sought to correlate right ventricular indices with serum bile acid levels.
  
  
  METHODS
  Indexed right ventricular end diastolic volume, as assessed by cardiac magnetic-resonance imaging, was classified as <100ml/m2 (Group 1, n = 5), 100-150ml/m2 (Group 2, n = 18), and >150ml/m2 (Group 3, n = 6) in 29 patients with repaired tetralogy of Fallot. Pulmonary regurgitation fraction and right ventricular ejection fraction were calculated. The serum bile acid profile, including 15 species, in these patients was determined by liquid chromatography coupled with mass spectrometry.
  
  
  RESULTS
  Serum bile acid levels increased from Group 1 to Group 3 (2.5 ± 0.7; 4.1 ± 2.5; 6.0 ± 2.8 μmol/l, respectively) with significantly increased bile acid values in Group 3 compared to Group 1 (p≤0.05). In Group 3, but not in Group 1 and 2, a significant increase in glycine-conjugated bile acids was observed. Pulmonary regurgitation fraction increased (12 ± 1; 28 ± 16; 43 ± 3%, Groups 1-3, respectively) and right ventricular ejection fraction decreased (48.4 ± 6.4; 48.5 ± 6.5; 42.1 ± 5.3%, Groups 1-3, respectively) with rising indexed right ventricular end diastolic volume.
  
  
  CONCLUSIONS
  These preliminary results suggest that serum bile acid levels are positively correlated with indexed right ventricular end-diastolic volume in patients with repaired tetralogy of Fallot; however, this needs to be confirmed in a larger patient cohort.
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Division of Pediatric Cardiology, Medical University Graz, Graz, Austria.
authors.forename: Gernot
authors.initials: G
authors.lastname: Grangl
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Division of Pediatric Cardiology, Medical University Graz, Graz, Austria.
authors.forename: Evelyn
authors.initials: E
authors.lastname: Zöhrer
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Division of Pediatric Cardiology, Medical University Graz, Graz, Austria.
authors.forename: Martin
authors.initials: M
authors.lastname: Köstenberger
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Division of Pediatric Cardiology, Medical University Graz, Graz, Austria.
authors.forename: Alexandra
authors.initials: A
authors.lastname: Jud
authors.affiliation: Clinical Institute of Medical and Chemical Laboratory Diagnostics, Medical University Graz, Graz, Austria.
authors.forename: Günter
authors.initials: G
authors.lastname: Fauler
authors.affiliation: Clinical Institute of Medical and Chemical Laboratory Diagnostics, Medical University Graz, Graz, Austria.
authors.forename: Hubert
authors.initials: H
authors.lastname: Scharnagl
authors.affiliation: Clinical Institute of Medical and Chemical Laboratory Diagnostics, Medical University Graz, Graz, Austria.
authors.forename: Tatjana
authors.initials: T
authors.lastname: Stojakovic
authors.affiliation: Department of Radiology, Division of Pediatric Radiology, Medical University Graz, Graz, Austria.
authors.forename: Robert
authors.initials: R
authors.lastname: Marterer
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Division of Pediatric Cardiology, Medical University Graz, Graz, Austria.
authors.forename: Andreas
authors.initials: A
authors.lastname: Gamillscheg
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Division of Pediatric Cardiology, Medical University Graz, Graz, Austria.
authors.forename: Jörg
authors.initials: J
authors.lastname: Jahnel
mesh_terms: D000293:Adolescent; D000328:Adult; D001647:Bile Acids and Salts / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D002648:Child; D002853:Chromatography, Liquid; D005260:Female; D006801:Humans; D008099:Liver / Q000378:metabolism*; D008297:Male; D013058:Mass Spectrometry; D009206:Myocardium / Q000378:metabolism*; D011183:Postoperative Complications / Q000097:blood / Q000473:pathology / Q000503:physiopathology*; D011446:Prospective Studies; D011665:Pulmonary Valve Insufficiency / Q000097:blood / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D013318:Stroke Volume; D013771:Tetralogy of Fallot / Q000097:blood* / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D016278:Ventricular Function, Right
chemical_list: D001647:Bile Acids and Salts; D015415:Biomarkers