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Tricuspid Atresia is a congenital heart defect characterized by the absence of the tricuspid valve, which prevents blood from flowing from the right atrium to the right ventricle. As a result, blood flow to the lungs for oxygenation is severely compromised. This condition is life-threatening if not treated, as it affects the ability of the heart to circulate oxygenated blood throughout the body.
Management Option | Description | Comments |
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Prostaglandin Infusion | PGE1 to keep the DA open, allowing some blood flow to the lungs for oxygenation. | Emergency measure after birth to ensure oxygenation until more definitive surgical interventions can be performed. |
Atrial Septostomy | Enlarge an opening between the atria, allows mixing of oxygen-poor and oxygen-rich blood. | Improves oxygenation in newborns with tricuspid atresia. |
Blalock-Taussig Shunt | A palliative surgical procedure where a shunt is created between a systemic artery and the pulmonary artery to increase blood flow to the lungs. | Temporary measure to improve oxygenation until the child is old enough for more definitive surgeries. |
Bidirectional Glenn Procedure | A surgical procedure where the superior vena cava is connected to the pulmonary arteries, allowing blood from the upper body to flow directly to the lungs. | Second stage of surgical repair, performed at 4-6 months of age, as part of the pathway towards the Fontan procedure. |
Fontan Procedure | Final stage of surgical repair where the IVC is connected to the pulmonary arteries, allowing all systemic venous blood to bypass the heart and flow directly to the lungs. | This is typically performed between 18 months and 4 years of age and aims to provide long-term palliation by routing blood to the lungs without passing through a ventricle. |
Long-term Follow-up | Regular monitoring and management of complications such as arrhythmias, heart failure, and protein-losing enteropathy. | Lifelong cardiology follow-up is essential for individuals who have undergone the Fontan procedure or other palliative surgeries for tricuspid atresia. |