Images & Codes



Archiving Working Group

International Society for Nomenclature of

Paediatric and Congenital Heart Disease






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Position and connection of heart
Position-orientation of heart abnormal Dextrocardia: heart predominantly in R hemithorax  
Position or morphology of thoraco-abdominal organs abnormal    
Abnormal atrial arrangement Total mirror imagery (atrial situs inversus)  
  Right isomerism ('asplenia')  
  Left isomerism ('polysplenia')  
AV and/or VA connections abnormal Double inlet ventricle Double inlet RV     
    Double inlet LV     
  Tricuspid atresia  
  Mitral atresia  
  Solitary ventricle of indeterminate morphology  
  Congenitally corrected transposition of great arteries  
  Discordant VA connections (TGA)   Complete transposition of great arteries (IVS)   
    Congenitally corrected transposition of great arteries
  Double outlet RV Double outlet RV - Fallot type
    Double outlet RV - transposition type
    Double outlet RV - with non-committed VSD
  Double outlet LV      
  Common arterial trunk  
Great veins
Superior caval vein (SVC) abnormality Left SVC persisting to coronary sinus  
Inferior caval vein (IVC) abnormality Azygous continuation of IVC  
Hepatic vein abnormality    
Systemic vein abnormality - congenital       
Pulmonary vein abnormality Pulmonary vein stenosis  
  Totally anomalous pulmonary venous connection Totally anomalous pulmonary venous connection - supracardiac  
    Totally anomalous pulmonary venous connection - intracardiac  
    Totally anomalous pulmonary venous connection - infracardiac  
    Totally anomalous pulmonary venous connection - mixed  
  Partially anomalous pulmonary venous connection(s)     Partially anomalous pulmonary venous connections: Scimitar    
Coronary sinus abnormality    
Atriums and atrial septum    
RA abnormality    
LA abnormality Cor triatriatum (divided left atrium)  
Intact atrial septum    
Atrial septum abnormality Patent foramen ovale (PFO)  
  ASD ASD within oval fossa (secundum)
    Sinus venosus ASD
    Common atrium (virtual absence of atrial septum)
    Interatrial communication (ASD) through coronary sinus orifice   
AV valves and AV septal defect    
Tricuspid valvar abnormality Tricuspid regurgitation Tricuspid regurgitation - congenital
  Tricuspid valvar dysplasia (mucoid thickening)    
  Straddling tricuspid valve  
  Ebstein's malformation of tricuspid valve     
  Tricuspid stenosis  
Mitral stenosis Mitral valve stenosis Mitral valvar stenosis - congenital
  Mitral subvalvar stenosis  
  Supravalvar mitral ring  
Mitral valvar abnormality Mitral regurgitation Mitral regurgitation - congenital
  Mitral valve stenosis Mitral valvar stenosis - congenital
  Straddling mitral valve  
  Supravalvar mitral ring  
  Mitral valvar prolapse  
  True cleft of mitral valve  
Mitral subvalvar apparatus abnormality Mitral subvalvar stenosis  
  Parachute malformation of mitral valve  
Atrioventricular septal defect AVSD: isolated atrial component (primum ASD)  
  AVSD: isolated ventricular component  
  AVSD: atrial & ventricular components (complete)   
  AVSD: atrial & ventricular components + separate AV valves ('intermediate')  
  AVSD with ventricular imbalance  
AVSD AV valvar abnormality AVSD AV valvar regurgitation  
AV septal defect and Tetralogy of Fallot    
Ventricles and ventricular septum    
Functionally univentricular heart Ventricular imbalance: dominant LV + hypoplastic RV  
  Ventricular imbalance: dominant RV + hypoplastic LV  
RV abnormality RV aneurysm  
  RV hypoplasia  
  Double chambered RV  
RV outflow tract obstruction    
LV abnormality LV hypoplasia  
  LV aneurysm  
Hypoplastic left heart syndrome    
LV outflow tract obstruction    
Intact ventricular septum        
Ventricular septal abnormality VSD Tiny VSD (Maladie de Roger)
    Single VSD
    Multiple VSDs
    Perimembranous VSD
    VSD + malaligned outlet septum
    Inlet VSD
    Muscular VSD
    Subarterial VSD
    Communication between LV-RA (Gerbode defect)   
    Spontaneous closure of VSD
  Aneurysm of membranous septum  
VA valves and great arteries    
Aortopulmonary window    
Pulmonary artery from ascending aorta (hemitruncus)    
Truncal valvar abnormality Truncal valvar regurgitation      
Pulmonary stenosis Subpulmonary stenosis  
  Pulmonary valvar stenosis    Pulmonary valvar stenosis - congenital   
  Supravalvar pulmonary trunk stenosis  
Pulmonary valvar abnormality Pulmonary valvar stenosis    Pulmonary valvar stenosis - congenital   
  Pulmonary regurgitation Pulmonary regurgitation - congenital
Pulmonary atresia Pulmonary atresia - imperforate valve      
  Pulmonary atresia + intact ventricular septum  
  Coronary fistulas from RV ('sinusoidal')     
  RV dependent coronary circulation  
Pulmonary trunk (MPA) abnormality Supravalvar pulmonary trunk stenosis  
  Pulmonary trunk hypoplasia      
Pulmonary arterial abnormality Pulmonary arterial stenosis Central pulmonary arterial stenosis - proximal to hilar bifurcation
    Peripheral pulmonary arterial stenoses - at/beyond hilar bifurcation
  Pulmonary arterial hypoplasia  
  Discontinuous (non-confluent) pulmonary arteries  
  Pulmonary arterial aneurysm  
Aortic stenosis Subaortic stenosis     Subaortic stenosis due to fibromuscular shelf    
  Aortic valvar stenosis Aortic valvar stenosis - congenital
  Supravalvar aortic stenosis  
Aortic valvar abnormality Aortic valvar stenosis Aortic valvar stenosis - congenital
  Eccentric opening of tricuspid aortic valve  
  Aortic regurgitation Aortic regurgitation - congenital
  Bicuspid aortic valve  
  Aortic valvar atresia  
  Aortic valvar prolapse  
Ascending aorta abnormality Ascending aorta hypoplasia      
  Supravalvar aortic stenosis  
  Ascending aorta dilation Ascending aorta dilation associated with Marfan syndrome  
Aortic sinus of Valsalva aneurysm    
Aorto-LV tunnel    
Aortic arch abnormality Right aortic arch  
  Aortic coarctation  
  Aortic arch hypoplasia (tubular)  
  Interrupted aortic arch  
  Aortic arch branch abnormality Aberrant origin R subclavian artery
Vascular ring Double aortic arch  
  Pulmonary arterial sling  
Descending aorta dilation    
Descending/ abdominal aorta hypoplasia    
Coronary arteries/Arterial duct
Pericardium/AV fistulae    
Coronary arterial abnormality Anomalous origin of coronary artery from pulmonary artery  
  Coronary fistula  
  Coronary artery: anomalous aortic origin / course  
  Coronary arterial aneurysm(s)     
Arterial duct (ductus arteriosus) abnormality Patent arterial duct (PDA)  
Arteriovenous fistula Pulmonary arteriovenous fistula  
Distal systemic arterial abnormality    
Pericardial abnormality    
Tetralogy of Fallot and variants    
Tetralogy of Fallot    
Pulmonary atresia + VSD (including Fallot type)    
Pulmonary atresia + VSD + 'MAPCA'(s)    
Absent pulmonary valve syndrome - Fallot-type    
Solitary arterial trunk (absent intrapericardial PAs)      
Major systemic-to-pulmonary collateral artery (MAPCA)    

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