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Tricuspid atresia
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Tricuspid atresia

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Contributors: Ryan Hoefen MD, PhD
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Synopsis

Congenital lack of tricuspid valve formation resulting in cyanosis and a murmur. An atrial septal defect, hypoplastic right ventricle, and ventricular septal defect are generally present. Other congenital lesions such as transposition of the great arteries may also be present. About half of cases present with cyanosis and a murmur on the day of birth. An additional 30% are diagnosed in the first month. With advances in fetal ultrasound, tricuspid atresia can be diagnosed on antenatal screening at 18-22 weeks gestation. If untreated, mortality is very high with 1-year survival estimated at 10%.

For more information, see OMIM.

Codes

ICD10CM:
Q22.4 – Congenital tricuspid stenosis

SNOMEDCT:
63042009 – Congenital atresia of tricuspid valve

Differential Diagnosis & Pitfalls

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Last Updated: 03/29/2017
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Tricuspid atresia
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Tricuspid atresia : Cyanosis, Heart murmur, Hypoxemia
Imaging Studies image of Tricuspid atresia
Axial CT image of the chest demonstrates a hypoplastic right ventricle consistent with patient's history of congenital tricuspid atresia.
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