Serum Bile Acids in Repaired Tetralogy of Fallot: A Marker for Liver and Heart?

• Published in: PloS one 2015-12, Vol.10 (12), p.e0144745-e0144745
• Main Authors: Grangl, Gernot, Zöhrer, Evelyn, Köstenberger, Martin, Jud, Alexandra, Fauler, Günter, Scharnagl, Hubert, Stojakovic, Tatjana, Marterer, Robert, Gamillscheg, Andreas, Jahnel, Jörg
• Format: Article
• Language: English
• Subjects: Acids; Adolescent; Adult; Bile; Bile acids; Bile Acids and Salts - blood; Biomarkers - blood; Cardiovascular disease; Cardiovascular diseases; Care and treatment; Child; Chromatography; Chromatography, Liquid; Congenital diseases; Diagnosis; Female; Glycine; Heart; Heart diseases; Humans; Liquid chromatography; Liver; Liver - metabolism; Magnetic resonance; Male; Mass Spectrometry; Mass spectroscopy; Measurement; Metabolism; Myocardium - metabolism; NMR; Nuclear magnetic resonance; Patients; Physiological aspects; Postoperative Complications - blood; Postoperative Complications - pathology; Postoperative Complications - physiopathology; Prognosis; Prospective Studies; Pulmonary Valve Insufficiency - blood; Pulmonary Valve Insufficiency - diagnosis; Pulmonary Valve Insufficiency - etiology; Pulmonary Valve Insufficiency - physiopathology; Regurgitation; Risk factors; Stroke Volume; Tetralogy of Fallot; Tetralogy of Fallot - blood; Tetralogy of Fallot - pathology; Tetralogy of Fallot - physiopathology; Tetralogy of Fallot - surgery; Ventricle; Ventricular Function, Right
• ISSN: 1932-6203; 1932-6203
• DOI: 10.1371/journal.pone.0144745

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. Indexed right ventricular end diastolic volume, as assessed by cardiac magnetic-resonance imaging, was classified as 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. 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. 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.