scholarly journals Mitochondrial Respiration Defects in Single-Ventricle Congenital Heart Disease

2021 ◽  
Vol 8 ◽  
Author(s):  
Xinxiu Xu ◽  
Jiuann-Huey Ivy Lin ◽  
Abha S. Bais ◽  
Michael John Reynolds ◽  
Tuantuan Tan ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Mitochondrial Respiration ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Mononuclear Cells ◽  
Heart Tissue ◽  
Mouse Heart ◽  
Peripheral Blood Mononuclear ◽  
Metabolic Defects

Background: Congenital heart disease (CHD) with single-ventricle (SV) physiology is now survivable with a three-stage surgical course ending with Fontan palliation. However, 10-year transplant-free survival remains at 39–50%, with ventricular dysfunction progressing to heart failure (HF) being a common sequela. For SV-CHD patients who develop HF, undergoing the surgical course would not be helpful and could even be detrimental. As HF risk cannot be predicted and metabolic defects have been observed in Ohia SV-CHD mice, we hypothesized that respiratory defects in peripheral blood mononuclear cells (PBMCs) may allow HF risk stratification in SV-CHD.Methods: SV-CHD (n = 20), biventricular CHD (BV-CHD; n = 16), or healthy control subjects (n = 22) were recruited, and PBMC oxygen consumption rate (OCR) was measured using the Seahorse Analyzer. Respiration was similarly measured in Ohia mouse heart tissue.Results: Post-Fontan SV-CHD patients with HF showed higher maximal respiratory capacity (p = 0.004) and respiratory reserve (p < 0.0001), parameters important for cell stress adaptation, while the opposite was found for those without HF (reserve p = 0.037; maximal p = 0.05). This was observed in comparison to BV-CHD or healthy controls. However, respiration did not differ between SV patients pre- and post-Fontan or between pre- or post-Fontan SV-CHD patients and BV-CHD. Reminiscent of these findings, heart tissue from Ohia mice with SV-CHD also showed higher OCR, while those without CHD showed lower OCR.Conclusion: Elevated mitochondrial respiration in PBMCs is correlated with HF in post-Fontan SV-CHD, suggesting that PBMC respiration may have utility for prognosticating HF risk in SV-CHD. Whether elevated respiration may reflect maladaptation to altered hemodynamics in SV-CHD warrants further investigation.

scholarly journals Effect of Cardiopulmonary Bypass on Annexin A1 Expression in Peripheral Blood Mononuclear Cells of Children with Congenital Heart Disease

2012 ◽  
Vol 31 (3) ◽  
pp. 193-198
Author(s):  
Gengxu Zhou ◽  
Xiaoyang Hong ◽  
Yuhang Liu ◽  
Zhichun Feng
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Peripheral Blood Mononuclear Cells ◽  
Peripheral Blood ◽  
Congenital Heart ◽  
Mononuclear Cells ◽  
Annexin A1 ◽  
Differentially Expressed Proteins ◽  
Peripheral Blood Mononuclear ◽  
Blood Mononuclear Cells

Effect of Cardiopulmonary Bypass on Annexin A1 Expression in Peripheral Blood Mononuclear Cells of Children with Congenital Heart DiseaseThis study aimed to investigate the effect of cardiopulmonary bypass (CPB) on Annexin A1 expression in the peripheral blood mononuclear cells (PBMCs) of children with congenital heart disease (CHD). A total of 30 children receiving CPB for interventricular septal defect were included. Peripheral blood was collected before and after CPB. PBMCs were collected by density gradient centrifugation. Protein extraction was performed by lysis and subjected to 2D-QUANT for protein quantitation. Isoelectric focusing electrophoresis (IEF) was carried out followed by gel image analysis. Protein spots with a difference in expression of >1.5 fold were collected as candidate proteins which were subjected to mass spectrometry for the identification of differentially expressed proteins. Western blot assay was employed to confirm the expressions of target proteins. Peripheral blood collected at two time points was subjected to two-dimensional electrophoresis, and a total of 12 differentially expressed proteins were identified. Of them, 5 proteins had decreased expression before CPB (T0) but their expressions increased after CPB (T1); the remaining 7 proteins had increased expressions before CPB but their expressions reduced after CPB. One of these differentially expressed proteins was Annexin A1. Western blot assay confirmed that Annexin A1 expression began to increase at 0.5 h after CPB, and the increase of Annexin A1 was more obvious after CPB. Our findings primarily indicate the potential mechanism underlying the role of PBMC in inflammatory response following CPB, and provide a target for the prevention and control of post-CPB systemic inflammatory response syndrome (SIRS).

scholarly journals Down syndrome and congenital heart disease: perioperative planning and management

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Dennis R. Delany ◽  
Stephanie S. Gaydos ◽  
Deborah A. Romeo ◽  
Heather T. Henderson ◽  
Kristi L. Fogg ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Down Syndrome ◽  
Cardiac Surgery ◽  
Heart Disease ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Heart Defects ◽  
Careful Consideration ◽  
Children With Down Syndrome ◽  
Perioperative Planning

AbstractApproximately 50% of newborns with Down syndrome have congenital heart disease. Non-cardiac comorbidities may also be present. Many of the principles and strategies of perioperative evaluation and management for patients with congenital heart disease apply to those with Down syndrome. Nevertheless, careful planning for cardiac surgery is required, evaluating for both cardiac and noncardiac disease, with careful consideration of the risk for pulmonary hypertension. In this manuscript, for children with Down syndrome and hemodynamically significant congenital heart disease, we will summarize the epidemiology of heart defects that warrant intervention. We will review perioperative planning for this unique population, including anesthetic considerations, common postoperative issues, nutritional strategies, and discharge planning. Special considerations for single ventricle palliation and heart transplantation evaluation will also be discussed. Overall, the risk of mortality with cardiac surgery in pediatric patients with Down syndrome is no more than the general population, except for those with functional single ventricle heart defects. Underlying comorbidities may contribute to postoperative complications and increased length of stay. A strong understanding of cardiac and non-cardiac considerations in children with Down syndrome will help clinicians optimize perioperative care and long-term outcomes.

scholarly journals The association of fetal cerebrovascular resistance with early neurodevelopment in single ventricle congenital heart disease

2013 ◽  
Vol 165 (4) ◽  
pp. 544-550.e1 ◽  
Author(s):  
Ismee A. Williams ◽  
Carlen Fifer ◽  
Edgar Jaeggi ◽  
Jami C. Levine ◽  
Erik C. Michelfelder ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Cerebrovascular Resistance

Angiographic classification of a single ventricle of the heart

1982 ◽  
Vol 63 (6) ◽  
pp. 44-46
Author(s):  
B. E. Shakhov
Keyword(s):  
Congenital Heart Disease ◽  
Cardiac Surgery ◽  
Heart Disease ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Complex Congenital Heart Disease ◽  
Heart Ventricle ◽  
New Classification ◽  
Angiographic Examination

Based on the data of angiographic examination of 25 patients with a single heart ventricle, a new classification of complex congenital heart disease is proposed. It indicates the type of defect without taking into account the morphology of the single ventricular chamber, which is angiographically difficult or impossible to determine in some cases. This classification simplifies the interpretation of angiographic images, reflects the anatomical variants of the defect and meets the modern requirements of cardiac surgery.

scholarly journals MELD-XI SCORE AND FONTAN FAILURE IN PATIENTS WITH SINGLE VENTRICLE PALLIATION FOR COMPLEX CONGENITAL HEART DISEASE

2011 ◽  
Vol 57 (14) ◽  
pp. E409
Author(s):  
Gabriele Egidy Assenza ◽  
Kimberlee Gauvreau ◽  
Dionne Graham ◽  
Michael Job Landzberg ◽  
Koenraad J. Mortele ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Complex Congenital Heart Disease ◽  
Fontan Failure ◽  
Single Ventricle Palliation

Abstract 11041: Use of Digoxin is Associated With Reduced Interstage Mortality: Results From the Pediatric Heart Network Single Ventricle Reconstruction Trial

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Matthew Oster ◽  
Michael Kelleman ◽  
Courtney McCracken ◽  
Richard P Ohye ◽  
William T Mahle
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Right Ventricle ◽  
Congenital Heart ◽  
Single Ventricle ◽  
Norwood Procedure ◽  
Survival Models ◽  
Number Needed To Treat ◽  
Interstage Mortality ◽  
Parametric Survival

Introduction: Despite medical and surgical advances over the past few decades, mortality for infants with single ventricle congenital heart disease remains as high as 8-12% during the interstage period, the time between discharge after the Norwood procedure and before the stage II palliation. The objective of our study was to determine the effect of digoxin use on interstage mortality in infants with single ventricle congenital heart disease. Hypothesis: We hypothesized that digoxin would be associated with lower interstage mortality. Methods: We conducted a retrospective cohort study using the Pediatric Heart Network Single Ventricle Reconstruction Trial public use dataset, which includes data on infants with single right ventricle congenital heart disease randomized to receive either a Blalock-Taussig shunt or right ventricle-to-pulmonary artery shunt during the Norwood procedure at 15 institutions in North America from 2005-2008. Parametric survival models were used to compare the risk of interstage mortality between those discharged to home on digoxin vs. those discharged to home not on digoxin, adjusting for center volume, ascending aorta diameter, shunt type, and socioeconomic status. Further comparisons were made to compare the number of other adverse events in the two groups. Results: Of the 330 infants eligible for this study, 102 (31%) were discharged home on digoxin. Interstage mortality for those not on digoxin was 12.3%, compared to 2.9% among those on digoxin (Figure), with a number needed to treat of 11 patients to prevent one death. The adjusted hazard ratio was 3.5 (95%CI 1.1-11.7, p=0.04). There were no differences in complications between the two groups during the interstage period. Conclusions: Digoxin use in infants with single ventricle congenital heart disease is associated with significantly reduced interstage mortality and should be considered for all such infants unless otherwise contraindicated.

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