Images & Codes 

 

Archiving Working Group

International Society for Nomenclature of

Paediatric and Congenital Heart Disease

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IPCCC Code: 06.01.01, 01.01.22, 07.11.01
 

 

AEPC Code:    

Tricuspid atresia,  Functionally univentricular heart, Muscular VSD  
       
 

EACTS-STS Code:    

Tricuspid valve disease, Tricuspid valve pathology, Tricuspid atresia; Single ventricle, Tricuspid atresia, VSD, Type 4 (Muscular)  
       
 

Definition:  NA

Common Synonyms: NA

 

 

 

 

 

This heart with ‘classical’ tricuspid atresia is viewed in anatomic orientation, demonstrating the anterosuperior location of the rudimentary and incomplete right ventricle (RV) along with concordant ventriculo-arterial connections. This ventricle is bipartite, having only apical and outlet portions and lacking the inlet component. The right atrium and atrial appendage (RAA) are dilated and there is a restrictive muscular ventricular septal defect (VSD). (A-aorta, PA-pulmonary artery)

 

Contributor: Diane Spicer, BS

 

 

 

A similar view of ‘classical’ tricuspid valve atresia as shown in TVatres1a, with the right atrium dissected off of the ventricular mass to demonstrate the absence of a right atrioventricular connection (yellow arrows). The right atrium and atrial appendage (RAA) are dilated. (A-aorta, POF-pulmonary outflow)

 

 

 

 

Contributor: Diane Spicer, BS

 
         
     

 

 
 

 

 

The opened right atrium in this heart with ‘classical’ tricuspid atresia (same heart as TVatres1a and 1b) shows the intact muscular floor of the right atrium. There is absence of the right atrioventricular connection with no evidence of a tricuspid valve. A dimple can sometimes be seen on the right atrial floor. There is a widely patent oval foramen (POF) consistent with a secundum type atrial septal defect. (CS-coronary sinus, SCV-superior caval vein)

 

Contributor: Diane Spicer, BS

   
       

AWG Certification: Pending       

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