Targeted metabolomic analysis of serum phospholipid and acylcarnitine in the adult Fontan patient with a dominant left ventricle

被引：18

作者：

Michel, Miriam ^{[1
,2
]}

Dubowy, Karl-Otto ^{[2
]}

Zlamy, Manuela ^{[3
]}

Karall, Daniela ^{[3
]}

Adam, Mark Gordian ^{[4
]}

Entenmann, Andreas ^{[5
]}

Keller, Markus Andreas ^{[6
]}

Koch, Jakob ^{[6
]}

Komazec, Irena Odri ^{[1
]}

Geiger, Ralf ^{[1
]}

Salvador, Christina ^{[3
]}

Niederwanger, Christian ^{[3
]}

Mueller, Udo ^{[4
]}

Scholl-Buergi, Sabine ^{[3
]}

Laser, Kai Thorsten ^{[2
]}

机构：

[1] Med Univ Innsbruck, Div Pediat Cardiol, Dept Pediat 3, Anichstr 35, A-6020 Innsbruck, Austria

[2] Ruhr Univ Bochum, Heart & Diabet Ctr North Rhine Westphalia, Ctr Pediat Cardiol & Congenital Heart Dis, Georgstr, Bad Oeynhausen, Germany

[3] Med Univ Innsbruck, Div Inherited Metab Disorders, Dept Pediat 1, Innsbruck, Austria

[4] Biocrates Life Sci AG, Innsbruck, Austria

[5] Med Univ Innsbruck, Div Gastroenterol & Hepatol, Dept Pediat 1, Innsbruck, Austria

[6] Med Univ Innsbruck, Inst Human Genet, Innsbruck, Austria

来源：

THERAPEUTIC ADVANCES IN CHRONIC DISEASE | 2020年 / 11卷

关键词：

acylcarnitine; angiogenesis; congenital heart disease; Fontan; heart failure; inflammation; lipid; lymphatic vessel; metabolism; metabolomics; phosphatidylcholine; phospholipid; sphingomyelin; vascular stiffening; CONGENITAL HEART-DISEASE; HIGH-DENSITY-LIPOPROTEIN; PROTEIN-LOSING ENTEROPATHY; PROGNOSTIC VALUE; NATRIURETIC PEPTIDE; CHOLESTEROL LEVELS; SPHINGOMYELIN; CHILDREN; MECHANISMS; OPERATION;

D O I：

10.1177/2040622320916031

中图分类号：

R9 [药学];

学科分类号：

1007 ;

摘要：

Background: Patients with a Fontan circulation have altered cholesterol and lipoprotein values. We analysed small organic molecules in extended phopsholipid and acylcarnitine metabolic pathways ('metabolomes') in adult Fontan patients with a dominant left ventricle, seeking differences between profiles in baseline and Fontan circulations. Methods: In an observational matched cross-sectional study, we compared phosphatidylcholine (PC), sphingomyelin (SM), and acylcarnitine metabolomes (105 analytes; AbsoluteIDQ(R) p180 kit (Biocrates Life Sciences AG, Innsbruck, Austria) in 20 adult Fontan patients having a dominant left ventricle with those in 20 age- and sex-matched healthy controls. Results: Serum levels of total PC (q-value 0.01), total SM (q-value 0.0002) were significantly lower, and total acylcarnitines (q-value 0.02) were significantly higher in patients than in controls. After normalisation of data, serum levels of 12 PC and 1 SM Fontan patients were significantly lower (q-values <0.05), and concentrations of 3 acylcarnitines were significantly higher than those in controls (q-values <0.05). Conclusion: Metabolomic profiling can use small specimens to identify biomarker patterns that track derangement in multiple metabolic pathways. The striking alterations in the phospholipid and acylcarnitine metabolome that we found in Fontan patients may reflect altered cell signalling and metabolism as found in heart failure in biventricular patients, chronic low-level inflammation, and alteration of functional or structural properties of lymphatic or blood vessels.

引用

页数：25

共 61 条

[21] Histopathological abnormalities in the central arteries and veins of Fontan subjects [J].