Tricuspid Atresia
Tricuspid atresia is a cyanotic congenital heart defect (CHD), defined as a congenital absence or agenesis of the morphologic tricuspid valve with the absence of communication between the right atrium and the right ventricle. At Protheragen, we are committed to researching and developing cures for rare cardiovascular disorders, especially tricuspid atresia. Understanding the overwhelming need for new solutions, our experienced team has dedicated itself to accelerating the drug discovery and development process to address the challenges of disease therapy.
Introduction to Tricuspid Atresia
Tricuspid atresia is a cyanotic CHD in which the tricuspid valve is completely agenesis. It represents about 1% of CHD and its prevalence, equal in the two sexes, is estimated to be 0.1 per 1000 live births. This ranks it as the fourth most common cyanotic CHD, following tetralogy of Fallot, transposition of the great arteries, and hypoplastic left heart syndrome. Risk factors for tricuspid atresia include Down's syndrome, uncontrolled diabetes, high alcohol intake during pregnancy, and a family history of CHD.
Fig.1 Hemodynamics in tricuspid atresia. (Sumal, A. S., et al., 2020)Pathogenesis of Tricuspid Atresia
The etiology of tricuspid atresia remains uncertain, but it is caused by perturbations in tricuspid valvulogenesis. Normal atrioventricular valvulogenesis is a complex and multistep event that requires input from the Wnt/β-catenin, bone morphogenetic protein/transforming growth factor-β, and Ras/extracellular-signal-regulated kinase (ERK) signaling pathways. Genetic mutations in these pathways may be the basis for some cases of tricuspid atresia, including RASA1, and NFATC1 gene mutation.
Therapeutics Development for Tricuspid Atresia
| Drug Names | Targets | Mechanism of Action | Research Phase |
|---|---|---|---|
| Bosentan | ETB | An endothelin receptor antagonist, acts by blocking endothelin-1 to promote decreased pulmonary vascular resistance and increase flow in individuals with tricuspid atresia. | Approved |
| Prostaglandin E1 | EP1 | It keeps the ductus arteriosus open, increasing systemic circulation and oxygenation. | Approved |
| Warfarin | VKOR | An anticoagulant that blocks vitamin K-dependent clotting factors reduces blood clots, which is important in reducing the chance of thromboembolic events. | Approved |
Disclaimer: Protheragen focuses on providing preclinical research services. This table is for information exchange purposes only. This table is not a treatment plan recommendation. For guidance on treatment options, please visit a regular hospital.
Our Services
Protheragen provides a comprehensive range of one-stop services throughout preclinical development. We offer advanced diagnostic and research services and targeted therapeutic development toward particular pathologic mechanisms of tricuspid atresia. Our company is an expert in constructing disease models to simulate human diseases, offering a solid foundation of delivery for testing and validation.
Therapeutic Development Services
Diverse Platforms
Animal Model Development for Tricuspid Atresia
Animal models play an important role in the investigation of tricuspid atresia, where they can help to understand the pathophysiology of the disease and test therapies in a controlled setting. We provide personalized animal model development services to meet your tricuspid atresia research needs and help you fasten drug discovery and development.
Genetically engineered models for tricuspid atresia involve target-specific genes in heart formation that are altered to mimic the condition.
- SAP130 mutation model
- Other models
Surgical manipulation is employed to generate experimental models to mimic the hemodynamic features of tricuspid atresia.
- Silicone patch closure model
- Other models
Furthermore, Protheragen's facilities support full pharmacokinetic analysis and extensive safety evaluation of drugs entering the next phase of trials so that therapies that are efficacy with safety can be advanced. By working with us, you will have access to unparalleled expertise and the most advanced practice dedicated toward tricuspid atresia research. Our integrated platform shortens the drug development pipeline, bringing drugs from the bench to the bedside. If you would like to know more about our services, please contact us.
Reference
- Sumal, Anoop S et al. "Tricuspid atresia: Where are we now?" Journal of cardiac surgery 35.7 (2020): 1609-1617.
For research use only, not for clinical use.