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Glenn Procedure in a Baby with Single Pulmonary Artery and Single Lung.

ABSTRACT: Glenn followed by Fontan are still the main two procedures for the treatment of patients with single ventricle. Some patients may be challenging due to unfavorable cardiopulmonary anatomy. In this report, we present Glenn shunt procedure in a patient with single pulmonary artery and congenitally agenetic lung.

SUBMITTER: Ugurlucan M

PROVIDER: S-EPMC6448474 | biostudies-literature | 2018 Jul-Sep

REPOSITORIES: biostudies-literature

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Glenn Procedure in a Baby with Single Pulmonary Artery and Single Lung.

Ugurlucan Murat M Oztas Didem Melis DM Bahseliyev Shiraslan S Aliyev Bahruz B Yozgat Yilmaz Y Tireli Emin E Alpagut Ufuk U

Heart views : the official journal of the Gulf Heart Association 20180701 3

Glenn followed by Fontan are still the main two procedures for the treatment of patients with single ventricle. Some patients may be challenging due to unfavorable cardiopulmonary anatomy. In this report, we present Glenn shunt procedure in a patient with single pulmonary artery and congenitally agenetic lung. ...[more]

PMID: 31007860

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Project description:NFU1 is an iron-sulfur (Fe-S) scaffold protein, involved in Fe-S assembly and transfer to a range of mitochondrial metalloproteins. Patients with the NFU1G208C mutation develop pulmonary arterial hypertension (PAH) with 70% penetrance. Rats with NFU1G206C homozygous mutation demonstrated the PAH phenotype showing increased pulmonary vasculature remodeling, right ventricular (RV) hypertrophy, and RV pressure. In the present study, we discovered phenotypic metabolic changes in pulmonary arterial smooth muscle cells (PASMC) from NFU1G206C homozygous mutant rats associated with alterations in the mitochondrial proteome. Quantitative analysis of the mitochondrial proteome showed significant changes in the abundance of 208 proteins involved in various metabolic and antioxidant functions in response to the NFU1 mutation. Our data indicates that the NFU1G206C homozygous mutant rats have decreased expression of complex I and II, which are known to depend on iron-sulfur clusters, and increased expression of complexes III to V, accompanied with a significant decrease in mitochondrial function, pyruvate dehydrogenase (PDH) activity and amplified glycolysis and anabolism in PASMC. The NFU1 mutation produced a dysregulated antioxidant system in the mitochondria which leads to increased levels of reactive oxygen species. Due to alterations in apoptosis regulating proteins, the NFU1G206C cells were found to exhibit high proliferation rates and resistance to apoptosis as compared with the wild type (WT). Finally, the NFU1G206C mitochondrial proteome showed significant abundance changes in proteins that regulate fatty acid (FA) metabolism and exhibited increased FA oxidation compared to WT, suggesting increased FA oxidation compensates for the decreased PDH activity. In conclusion, our data indicates that the NFU1G206C mutation induces a metabolic shift in the PASMC, which results in a hyper-proliferative and apoptosis resistant phenotype and presents a novel cellular model to study PAH.

Dataset Information

Glenn Procedure in a Baby with Single Pulmonary Artery and Single Lung.

Publications

Glenn Procedure in a Baby with Single Pulmonary Artery and Single Lung.

Similar Datasets

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