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Quantitative plasma proteomic analysis in children after superior cavopulmonary anastomosis with pulmonary
Elijah H Bolin1,2, Peter M Mourani3,4, Stephanie D Byrum3,5
1Department of Pediatrics, Section of Cardiology, University of Arkansas for Medical Sciences, Little Rock, AR, USA. ehbolin@uams.edu.
Insights
Plasma protein differences were not found in children with single ventricle heart disease and pulmonary arteriovenous malformations (PAVMs) after superior cavopulmonary anastomosis (SCPA). This suggests other factors may cause PAVMs in these patients.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease Research
- Vascular Malformation Etiology
Background:
- Single ventricle heart disease affects ~1000 US children annually.
- Pulmonary arteriovenous malformations (PAVMs) are a significant mortality cause in this population.
- PAVMs commonly develop after superior cavopulmonary anastomosis (SCPA).
Purpose of the Study:
- To investigate if differential plasma protein concentrations in superior caval blood correlate with PAVM development post-SCPA.
- To explore the role of plasma proteins in PAVM pathogenesis in single ventricle patients.
Main Methods:
- Quantitative plasma proteomics was performed on 11 children with PAVMs and 7 without PAVMs post-SCPA.
- An additional 11 children with Fontan circulation served as a reference group.
- Superior caval plasma samples were analyzed to compare protein expression profiles.
Main Results:
- No significant differences in plasma proteomes were observed between children with and without PAVMs after SCPA.
- Eighteen proteins showed differential expression when comparing Fontan circulation patients to SCPA patients with PAVMs.
- 10 proteins were downregulated and 8 were upregulated in the Fontan vs. SCPA with PAVMs group.
Conclusions:
- Plasma protein differences do not appear to be the primary driver for PAVM development in single ventricle patients post-SCPA.
- The etiology of PAVMs in this cohort likely involves factors beyond plasma protein concentration.
- Further research is necessary to elucidate the underlying causes of PAVMs following SCPA.
Abstract:
Approximately 1000 children are born every year in the United States with one effective cardiac pumping chamber, or single ventricle heart disease. One of the early causes of mortality in this population is pulmonary arteriovenous malformations (PAVMs), which allow blood to bypass gas exchange in the lungs. PAVMs most frequently occur in children after superior cavopulmonary anastomosis (SCPA), a procedure that redirects venous blood from the upper body to the lungs. Because plasma proteins are in part responsible for directing angiogenesis, we hypothesized that differential protein concentrations would be observed in superior caval blood among children after SCPA according to PAVM status. We performed quantitative plasma proteomics from 11 children with PAVMs and in seven children without PAVMs; an additional 11 children with Fontan circulation were included as a reference. Among children with SCPA, there were no significant differences in the plasma proteomes for those with and without PAVMs. When comparing children with Fontan circulation to those with SCPA and PAVMs, 18 proteins exhibited differential expression (10 downregulated and eight upregulated) in superior caval plasma. These results suggest that factors other than, or in addition to, plasma proteins may be responsible for single ventricle patients' susceptibility to PAVMs after SCPA. IMPACT: What is the key message of your article? We did not identify significant differences in plasma proteins when comparing those children with and without pulmonary arteriovenous malformations (PAVMs) after superior cavopulmonary anastomosis (SCPA). What does it add to the existing literature? The etiology of PAVMs in this population is likely due to factors other than, or in addition to, differences in plasma proteins. What is the impact? Further studies are needed to identify causes of PAVMs among children after SCPA.
