Anterior (frontal) view of the opened heart in tricuspid atresia with ASD+VSD. White arrows indicate blood flow. (Atresic tricuspid valve labeled at bottom left.)
Specialty
Medical genetics
Tricuspid atresia is a form of congenital heart disease whereby there is a complete absence of the tricuspid valve.[2] Therefore, there is an absence of right atrioventricular connection.[2] This leads to a hypoplastic (undersized) or absent right ventricle. This defect is contracted during prenatal development, when the heart does not finish developing. It causes the systemic circulation to be filled with relatively deoxygenated blood. The causes of tricuspid atresia are unknown.[3]
In most cases of tricuspid atresia, additional defects exist to allow exchange of blood between the loops of systematic circulation and pulmonary circulation, filling in the role of the missing atrioventricular connection. An atrial septal defect (ASD) must be present to fill the left atrium and the left ventricle with blood.[4] Since there is a lack of a right ventricle, there must also be a way to pump blood into the pulmonary artery. This can be accomplished by a ventricular septal defect (VSD) connecting the left ventricle to the pulmonary artery or by a patent ductus arteriosus (PDA) connecting the aorta to the pulmonary artery. In the latter case, prostaglandin E1 is used to maintain the PDA connection until emergency corrective surgery can be completed. As oxygenated blood is mixed with deoxygenated blood in both cases, there is a reduction in the oxygen-carrying capacity.[4]
It is also possible for tricuspid atresia to appear without the life-saving defects. In this case, the systemic and pulmonary circulations would be cut off from each other and no useful breathing can occur. An experimental procedure called fetal balloon atrial septostomy can be used to artificially create the required defect in utero.[5]
^"Tricuspid atresia: MedlinePlus Medical Encyclopedia". medlineplus.gov. Retrieved 28 May 2019.
^ abMurthy, Raghav; Nigro, John; Karamlou, Tara (2019-01-01), Ungerleider, Ross M.; Meliones, Jon N.; Nelson McMillan, Kristen; Cooper, David S. (eds.), "65 - Tricuspid Atresia", Critical Heart Disease in Infants and Children (Third Edition), Philadelphia: Elsevier, pp. 765–777.e3, doi:10.1016/b978-1-4557-0760-7.00065-6, ISBN 978-1-4557-0760-7, S2CID 214741527, retrieved 2020-11-27
^ abLok, Josephine M.; Spevak, Philip J.; Nichols, David G. (2006-01-01), Nichols, David G.; Ungerleider, Ross M.; Spevak, Philip J.; Greeley, William J. (eds.), "Chapter 39 - Tricuspid Atresia", Critical Heart Disease in Infants and Children (Second Edition), Philadelphia: Mosby, pp. 799–822, ISBN 978-0-323-01281-2, retrieved 2020-12-05
^Plackett, Benjamin (25 November 2021). "The surgical solution to congenital heart defects". Nature. 599 (7886): S21. doi:10.1038/d41586-021-03517-z.
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Marked in black arrow. Tricuspidatresia Cavo-tricuspid isthmus Lancisi's sign Right atrial enlargement "Anatomy of the Tricuspid Valve". e-echocardiography...
(link) CDC (19 November 2019). "Congenital Heart Defects – Facts about TricuspidAtresia | CDC". Centers for Disease Control and Prevention. Retrieved 20 November...
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two types of pulmonary atresia, classified by whether or not the baby also has a ventricular septal defect. Pulmonary atresia with an intact ventricular...
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