Pediheart: Pediatric Cardiology Today
Jul 10, 2026 · 38 min · 8 segments
This week we delve again into the world of adult congenital heart disease and specifically the realm of biomarkers when we review a recent report of the proteomics of the adult Fontan patient. What is…
Robert PassHostSasha ApatowskyGuest
Ismail AsiGuest
This week's work starts with what we are all very aware of, namely the myriad negative effects of not having a subpulmonary pumping chamber, as is the case in the Fontan patient.
and how having elevated systemic venous pressure with lack of a pulsatile pulmonary blood flow can impact many organ systems, which results in things like heart failure, arrhythmia, plastic bronchitis, PLE, hepatocellular carcinoma, or even chronic kidney disease.
The authors make the observation that these complications are commonly seen in this patient group but we're fairly limited in our ability to predict when these adverse events might occur.
And they suggest that this fact therefore underlines the importance of better understanding the underlying adaptive or maladaptive molecular mechanisms of Fontan-associated disease pathology.
The authors then discuss how the field of proteomics has been rarely applied to this patient population.
For those less familiar, proteomics involves large-scale dynamic analyses of protein expressions to understand physiologic changes, distinct from the static genetic information that can be provided by genomics.
Study of these may help better understand changes in inflammation, fibrosis, and angiogenic pathways in general, and the authors make the argument that we don't really understand what they refer to as the proteomic signature of Fontan physiology, which might potentially help better understand the pathophysiology, and maybe even help us predict problems, such as the ones I mentioned a moment before, before they become clinically apparent.
The authors state, and I quote, "...to address this, we conducted a large single-center study of the application of two different targeted plasma proteomics platforms in individuals with Fontan circulation, with the goal of understanding how the Fontan circulation impacts the body at the molecular level and to elucidate potential long-term targets for preventing Fontan-associated complications." The authors used two different independent proteomic platforms to study this, and it was their hope that by better understanding the proteomics of the adult Fontan patient, connections between molecular alterations and organ-specific complications might be more obvious and might even result, potentially, in biomarker development.
The authors review how this study was performed, and basically the group used the Boston Adult Congenital Heart Disease Biobank with specimens from 2012 to 2017, which we have reviewed a few years ago now on the podcast.
And they explain how they used two different proteomic platforms called O-Link and SomaScan.
Figure 1 of this paper shows that among the 162 Fontan patients in this work, 161, or the vast majority, had the O-Link platform used, with only 71 of these 161 O-Link tested patients also getting tested via the SomaScan platform.
And they review how the O-Link testing assesses 975 proteins and the SomaScan, 1265 proteins.
The figure shows how there were 36 control participants, with 31 of 36 having O-Link testing and 8 of those also getting SomaScan testing, with 5 controls only being tested with the SomaScan test.
For those of you wondering, these two tests use different ways of identifying and quantifying these proteins.
And without being an expert on this, my reading about this suggests that O-Link testing, the one most commonly used in this work, has a very high degree of specificity, whereas SomaScan testing has less specific testing qualities but is much more sensitive.
The authors review how they used the tests and how they were able to look for toxicity-related mechanism and injury patterns, and how they did comparisons of protein findings.
for these complex granular details i would once again as usual recommend you read the paper particularly pages three to five and on to the results table one shows that there were again 192 total patients with 161 being the fontan patients and 31 controls with the o-link fontan group average age of 30.6 which was similar to the control group at 29.1 years There were no significant differences in regards to weight or BMI or sex distribution, other than that the controls were slightly taller, 171.8 cm versus 167.1 cm.
This week's work starts with what we are all very aware of, namely the myriad negative effects of not having a subpulmonary pumping chamber, as is the case in the Fontan patient.
and how having elevated systemic venous pressure with lack of a pulsatile pulmonary blood flow can impact many organ systems, which results in things like heart failure, arrhythmia, plastic bronchitis, PLE, hepatocellular carcinoma, or even chronic kidney disease.
The authors make the observation that these complications are commonly seen in this patient group but we're fairly limited in our ability to predict when these adverse events might occur.
And they suggest that this fact therefore underlines the importance of better understanding the underlying adaptive or maladaptive molecular mechanisms of Fontan-associated disease pathology.
The authors then discuss how the field of proteomics has been rarely applied to this patient population.
For those less familiar, proteomics involves large-scale dynamic analyses of protein expressions to understand physiologic changes, distinct from the static genetic information that can be provided by genomics.
Study of these may help better understand changes in inflammation, fibrosis, and angiogenic pathways in general, and the authors make the argument that we don't really understand what they refer to as the proteomic signature of Fontan physiology, which might potentially help better understand the pathophysiology, and maybe even help us predict problems, such as the ones I mentioned a moment before, before they become clinically apparent.
The authors state, and I quote, "...to address this, we conducted a large single-center study of the application of two different targeted plasma proteomics platforms in individuals with Fontan circulation, with the goal of understanding how the Fontan circulation impacts the body at the molecular level and to elucidate potential long-term targets for preventing Fontan-associated complications." The authors used two different independent proteomic platforms to study this, and it was their hope that by better understanding the proteomics of the adult Fontan patient, connections between molecular alterations and organ-specific complications might be more obvious and might even result, potentially, in biomarker development.
The authors review how this study was performed, and basically the group used the Boston Adult Congenital Heart Disease Biobank with specimens from 2012 to 2017, which we have reviewed a few years ago now on the podcast.
And they explain how they used two different proteomic platforms called O-Link and SomaScan.
Figure 1 of this paper shows that among the 162 Fontan patients in this work, 161, or the vast majority, had the O-Link platform used, with only 71 of these 161 O-Link tested patients also getting tested via the SomaScan platform.
And they review how the O-Link testing assesses 975 proteins and the SomaScan, 1265 proteins.
The figure shows how there were 36 control participants, with 31 of 36 having O-Link testing and 8 of those also getting SomaScan testing, with 5 controls only being tested with the SomaScan test.
For those of you wondering, these two tests use different ways of identifying and quantifying these proteins.
And without being an expert on this, my reading about this suggests that O-Link testing, the one most commonly used in this work, has a very high degree of specificity, whereas SomaScan testing has less specific testing qualities but is much more sensitive.
The authors review how they used the tests and how they were able to look for toxicity-related mechanism and injury patterns, and how they did comparisons of protein findings.
for these complex granular details i would once again as usual recommend you read the paper particularly pages three to five and on to the results table one shows that there were again 192 total patients with 161 being the fontan patients and 31 controls with the o-link fontan group average age of 30.6 which was similar to the control group at 29.1 years There were no significant differences in regards to weight or BMI or sex distribution, other than that the controls were slightly taller, 171.8 cm versus 167.1 cm.
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