Understanding Fontan Biology
Understanding the complex biological processes through advanced phenotyping and molecular analysis to transform patient care in congenital heart disease.
The Fontan Challenge
A Unique Circulation
The Fontan operation represents one of the most remarkable achievements in congenital heart surgery, allowing children born with single ventricle heart defects to survive into adulthood. However, this unique circulation creates a fundamentally different physiological state that comes with long-term challenges.
In Fontan physiology, systemic venous blood flows passively into the pulmonary arteries without a pumping ventricle, creating a low-pressure, high-resistance system that profoundly affects multiple organ systems.
Unlike normal circulation, the Fontan operates without a subpulmonary ventricle, fundamentally altering hemodynamics, metabolism, and cellular function throughout the body.
Living with Half a Heart
Despite surgical success, Fontan patients face long-term complications including protein-losing enteropathy, arrhythmias, thromboembolism, and progressive ventricular dysfunction. Patients with Fontan circulation face unique metabolic and physiological challenges that require specialized care and ongoing research to improve long-term outcomes and quality of life.
Systemic Congestion
Chronically elevated central venous pressure affects liver, kidney, and intestinal function, leading to progressive organ dysfunction over time.
Cardiac Output Limitation
Reduced cardiac output and impaired exercise capacity limit physical performance and significantly impact daily activities and quality of life.
Metabolic Disruption
Chronic low cardiac output and altered perfusion patterns lead to fundamental changes in cellular metabolism and energy production.
Inflammatory Response
Persistent inflammation and immune system dysregulation contribute to progressive organ dysfunction and reduced quality of life.
Inflammatory Response
Persistent inflammation and immune system dysregulation contribute to progressive organ dysfunction and reduced quality of life.
Inflammatory Response
Persistent inflammation and immune system dysregulation contribute to progressive organ dysfunction and reduced quality of life.
Our Phenotyping Approach
Multi-Omics Strategy
We employ cutting-edge omics technologies to create comprehensive molecular phenotypes of Fontan patients. Our integrated approach combines metabolomics, proteomics, and advanced bioinformatics to reveal hidden biological patterns.
By analyzing thousands of molecular markers simultaneously, we can identify subtle but critical changes that traditional clinical markers might miss.
Precision Medicine
Our goal is to move beyond one-size-fits-all approaches to develop personalized therapeutic strategies based on individual biological signatures and risk profiles.
Research Workflow
Key Biological Discoveries
Metabolic Reprogramming
Our research has revealed significant metabolic alterations on various molecular levels. These changes suggest adaptive mechanisms to cope with chronic hypoperfusion.
We've identified specific metabolic signatures that correlate with clinical outcomes, opening new avenues for risk stratification and potentially targeted intervention.
Future Directions
Therapeutic Development
Building on our biological insights, we are establishing the basis for the developement of potential targeted therapeutic strategies including metabolic interventions, anti-inflammatory approaches, and personalized nutrition protocols.
Our ultimate goal is to translate molecular discoveries into clinical applications that improve long-term outcomes for Fontan patients.
Vision 2030
We envision a future where every Fontan patient receives personalized care based on his/her unique biological profile, significantly contributing to improving quality of life and long-term survival.
Metabolic Therapies
Developing targeted interventions to optimize energy metabolism and reduce oxidative stress in Fontan circulation.
Predictive Biomarkers
Creating prognostic tools to identify patients at risk for complications.
Global Collaboration
Expanding international partnerships to validate findings across diverse populations and accelerate discovery.