Images & Codes



Archiving Working Group

International Society for Nomenclature of

Paediatric and Congenital Heart Disease







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Septal Defects  ASD PFO
ASD, Secundum
ASD, Sinus venosus
ASD, Coronary sinus
ASD, Common atrium (single atrium)
VSD  VSD, Type 1 (Subarterial) (Supracristal) (Conal septal defect) (Infundibular)
VSD, Type 2 (Perimembranous) (Paramembranous) (Conoventricular)
VSD, Type 3 (Inlet) (AV canal type)
VSD, Type 4 (Muscular)
VSD, Type: Gerbode type (LV-RA communication)
VSD, Multiple
AV Canal  AVC (AVSD), Complete (CAVSD)
AVC (AVSD), Intermediate (transitional)
AVC (AVSD), Partial (incomplete) (PAVSD) (ASD, primum)
AP Window  AP window (aortopulmonary window)
Pulmonary artery origin from ascending aorta (hemitruncus)
Truncus Arteriosus  Truncus arteriosus
Truncal valve insufficiency
Truncus arteriosus + Interrupted aortic arch
Pulmonary Venous Anomalies Partial Anomalous Pulmonary Venous Connection Partial anomalous pulmonary venous connection (PAPVC)
Partial anomalous pulmonary venous connection (PAPVC), scimitar
Total Anomalous Pulmonary Venous Connection Total anomalous pulmonary venous connection (TAPVC), Type 1 (supracardiac)
Total anomalous pulmonary venous connection (TAPVC), Type 2 (cardiac)
Total anomalous pulmonary venous connection (TAPVC), Type 3 (infracardiac)
Total anomalous pulmonary venous connection (TAPVC), Type 4 (mixed)
Cor Triatriatum    Cor triatriatum
Pulmonary Venous Stenosis   Pulmonary venous stenosis
Systemic Venous Anomalies  Anomalous Systemic Venous Connection Systemic venous anomaly
Systemic venous obstruction Systemic venous obstruction
Right Heart Lesions  Tetralogy of Fallot TOF
TOF, Pulmonary stenosis
TOF, Absent pulmonary valve
Pulmonary Atresia Pulmonary atresia
Pulmonary atresia, IVS
Pulmonary atresia, VSD (Including TOF, PA)
Pulmonary atresia, VSD-MAPCA (pseudotruncus)
MAPCA(s) (major aortopulmonary collateral[s]) (without PA-VSD)
Tricuspid Valve Disease and Ebstein’s Anomaly Ebstein's anomaly
Tricuspid regurgitation, non-Ebstein's related
Tricuspid stenosis
Tricuspid regurgitation and tricuspid stenosis
Tricuspid valve, Other
RVOT Obstruction and/or Pulmonary Stenosis Pulmonary stenosis, Valvar
Pulmonary artery stenosis (hypoplasia), Main (trunk)
Pulmonary artery stenosis, Branch, Central (within the hilar bifurcation)
Pulmonary artery stenosis, Branch, Peripheral (at or beyond the hilar bifurcation)
Pulmonary artery, Discontinuous
Pulmonary stenosis, Subvalvar
Double Chambered RV
Pulmonary Valve Disease Pulmonary valve, Other
Pulmonary insufficiency
Pulmonary insufficiency and pulmonary stenosis
Shunt failure  Shunt failure  Shunt failure
Conduit failure  Conduit failure  Conduit failure
Left Heart Lesions Aortic Valve Disease  Aortic stenosis, Subvalvar
Aortic stenosis, Valvar
Aortic stenosis, Supravalvar
Aortic valve atresia
Aortic insufficiency
Aortic insufficiency and aortic stenosis
Aortic valve, Other
Sinus of Valsalva Fistula/Aneurysm Sinus of Valsalva aneurysm
LV to Aorta Tunnel LV to aorta tunnel
Mitral Valve Disease Mitral stenosis, Supravalvar mitral ring
Mitral stenosis, Valvar
Mitral stenosis, Subvalvar
Mitral stenosis, Subvalvar, Parachute
Mitral stenosis
Mitral regurgitation and mitral stenosis
Mitral regurgitation
Mitral valve, Other
Hypoplastic Left Heart Syndrome Hypoplastic left heart syndrome (HLHS)
Shone’s syndrome Shone’s syndrome
Cardiomyopathy   Cardiomyopathy (including dilated, restrictive, and hypertrophic)
Cardiomyopathy, End-stage congenital heart disease
Pericardial Disease    Pericardial effusion
Pericardial disease, Other
Single Ventricle    Single ventricle, DILV
Single ventricle, DIRV
Single ventricle, Mitral atresia
Single ventricle, Tricuspid atresia
Single ventricle, Unbalanced AV canal
Single ventricle, Heterotaxia syndrome
Single ventricle, Other
Single Ventricle + Total anomalous pulmonary venous connection (TAPVC)
Transposition of the Great Arteries Congenitally Corrected TGA Congenitally corrected TGA
Congenitally corrected TGA, IVS
Congenitally corrected TGA, IVS-LVOTO
Congenitally corrected TGA, VSD
Congenitally corrected TGA, VSD-LVOTO
Transposition of the Great Arteries TGA, IVS
DORV    DORV, VSD type
DORV, TOF type
DORV, TGA type
DORV, Remote VSD (uncommitted VSD)
DORV + AVSD (AV Canal)
Thoracic Arteries and Veins  Coarctation of Aorta and Aortic arch hypoplasia Coarctation of aorta
Aortic arch hypoplasia
VSD + Aortic arch hypoplasia
VSD + Coarctation of aorta
Coronary Artery Anomalies Coronary artery anomaly, Anomalous aortic origin of coronary artery from aorta (AAOCA)
Coronary artery anomaly, Anomalous pulmonary origin (includes ALCAPA)
Coronary artery anomaly, Fistula
Coronary artery anomaly, Aneurysm
Coronary artery anomaly, Other
Interrupted Arch  Interrupted aortic arch
Interrupted aortic arch + VSD
Interrupted aortic arch + AP window (aortopulmonary window)
Patent Ductus Arteriosus Patent ductus arteriosus
Vascular rings and Slings Vascular ring
Pulmonary artery sling
Aortic Aneurysm  Aortic aneurysm (including pseudoaneurysm)
Aortic Dissection  Aortic dissection
Miscellaneous, Other    Atrial Isomerism, Left or right
Endocardial fibroelastosis
Situs inversus 
Aneurysm, Ventricular, Right or Left (including pseudoaneurysm)
Aortic Arch Atresia
Aneurysm, Pulmonary artery
Aneurysm, Other
Hypoplastic RV
Hypoplastic LV
Postoperative bleeding
Rheumatic heart disease
Prosthetic valve failure
Myocardial infarction
Cardiac tumor
Pulmonary AV fistula
Pulmonary embolism
Pulmonary vascular obstructive disease
Pulmonary vascular obstructive disease (Eisenmenger’s)
Primary pulmonary hypertension
Persistent fetal circulation
Meconium aspiration
Cardiac, Other
Thoracic and/or mediastinal, Other
Peripheral vascular, Other
Status post - Transplant, Heart
Status post - Transplant, lung(s)
Status post - Transplant, Heart and lung
Normal heart
Miscellaneous, Other


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