IPCCC ICD-11 Congenital Heart Atlas

• Structural developmental anomaly of heart or great vessels
  • Congenital anomaly of position or spatial relationships of thoraco-abdominal organs
    • Anomalous position-orientation of heart
      • Laevocardia
        • ■ Marina TEst new term - unlikely
      • ■ Dextrocardia
      • ■ Mesocardia
      • ■ Extrathoracic heart
    • ■ Usual atrial arrangement
    • Abnormal atrial arrangement
      • ■ Atrial situs inversus
      • ■ Isomerism of right atrial appendages
      • ■ Isomerism of left atrial appendages
    • ■ Abnormal ventricular relationships
    • Abnormal relationship of great arterial roots
      • ■ Aortic root directly anterior to pulmonary root
      • ■ Aortic root anterior and rightward to pulmonary root
      • ■ Aortic root anterior and leftward to pulmonary root
      • ■ Aortic root side by side and directly rightward to pulmonary root
      • ■ Aortic root side by side and directly leftward to pulmonary root
      • ■ Aortic root directly posterior to pulmonary root
      • ■ Aortic root posterior and rightward to pulmonary root
      • ■ Aortic root posterior and leftward to pulmonary root
    • Abnormal intrapericardial course of great arteries
      • ■ Spiralling course of great arteries
      • ■ Parallel course of great arteries
    • Visceral heterotaxy
      • ■ Right hand pattern ventricular topology
      • ■ Left hand pattern ventricular topology
      • ■ Right isomerism
      • ■ Left Isomerism
    • ■ Total mirror imagery
  • Congenital anomaly of an atrioventricular or ventriculo-arterial connection
    • ■ Concordant atrioventricular connections
    • Discordant atrioventricular connections
      • ■ Congenitally corrected transposition of great arteries
    • Transposition of the great arteries
      • ■ Transposition of the great arteries with concordant atrioventricular connections and intact ventricular septum
      • ■ Transposition of the great arteries with concordant atrioventricular connections and ventricular septal defect
      • ■ Transposition of the great arteries with concordant atrioventricular connections and ventricular septal defect and left ventricular outflow tract obstruction
    • Concordant ventriculo-arterial connections
      • ■ Concordant ventriculo-arterial connections with parallel great arteries
    • Double outlet right ventricle
      • Double outlet right ventricle with subaortic or doubly committed ventricular septal defect and pulmonary stenosis, Fallot type
        • ■ Double outlet right ventricle with subaortic ventricular septal defect and pulmonary stenosis, Fallot type
        • ■ Double outlet right ventricle with doubly committed ventricular septal defect and pulmonary stenosis, Fallot type
      • ■ Double outlet right ventricle with subpulmonary ventricular septal defect, transposition type
      • ■ Double outlet right ventricle with non-committed ventricular septal defect
      • Double outlet right ventricle with subaortic or doubly committed ventricular septal defect without pulmonary stenosis, ventricular septal defect type
        • ■ Double outlet right ventricle with subaortic ventricular septal defect without pulmonary stenosis
        • ■ Double outlet right ventricle with doubly committed ventricular septal defect without pulmonary stenosis
      • ■ Double outlet right ventricle with intact ventricular septum
    • ■ Double outlet left ventricle
    • Common arterial trunk
      • Common arterial trunk with aortic dominance
        • ■ Common arterial trunk with aortic dominance and both pulmonary arteries arising from trunk
        • ■ Common arterial trunk with aortic dominance and one pulmonary artery absent from the trunk, isolated pulmonary artery
      • Common arterial trunk with pulmonary dominance and aortic arch obstruction
        • ■ Common arterial trunk with pulmonary dominance and interrupted aortic arch
        • ■ Common arterial trunk with pulmonary dominance and aortic coarctation
      • Atypical truncal valve
        • ■ Congenital truncal valvar regurgitation
        • ■ Congenital truncal valvar stenosis
        • ■ Dysplasia of truncal valve
  • Congenital anomaly of mediastinal vein
    • Congenital anomaly of mediastinal systemic vein
      • Congenital anomaly of superior caval vein
        • ■ Absent right superior caval vein
        • Left superior caval vein
          • ■ Left superior caval vein to coronary sinus
          • ■ Left superior caval vein to left-sided atrium
        • ■ Congenital stenosis of superior caval vein
      • Congenital anomaly of inferior caval vein
        • ■ Interrupted inferior caval vein with absent suprarenal segment and azygos continuation
        • ■ Congenital anomaly of the coronary sinus
      • Congenital stenosis of inferior caval vein
        • Unroofed coronary sinus
          • ■ Completely unroofed coronary sinus
          • ■ Partially unroofed coronary sinus
        • ■ Coronary sinus orifice atresia or stenosis
      • ■ Anomalous hepatic venous connection to heart
    • Congenital anomaly of pulmonary vein
      • Anomalous pulmonary venous connection
        • Total anomalous pulmonary venous connection
          • ■ Total anomalous pulmonary venous connection of the supracardiac type
          • ■ Total anomalous pulmonary venous connection of the cardiac type
          • ■ Total anomalous pulmonary venous connection of the infracardiac type
          • ■ Total anomalous pulmonary venous connection of the mixed type
        • ■ Partial anomalous pulmonary venous connection
        • Partial anomalous pulmonary venous connection of Scimitar type
          • ■ Scimitar syndrome
        • ■ Obstructed anomalous pulmonary venous pathway or connection
      • ■ Congenital pulmonary venous stenosis or hypoplasia
      • ■ Congenital atresia of pulmonary vein
  • Congenital anomaly of an atrium or atrial septum
    • Congenital anomaly of atrial septum
      • ■ Restrictive interatrial communication or intact atrial septum when an interatrial shunt is physiologically necessary
      • ■ Aneurysm of the atrial septum
      • Interatrial communication
        • ■ Patent oval foramen
        • ■ Atrial septal defect within oval fossa
        • ■ Sinus venosus defect
        • ■ Common atrium with separate atrioventricular junctions
        • ■ Interatrial communication through coronary sinus orifice
    • Congenital anomaly of right atrium
      • ■ Divided right atrium
      • ■ Chiari network
      • ■ Left-sided juxtaposition of the atrial appendages
      • ■ Congenital giant right atrium
    • Congenital anomaly of left atrium
      • ■ Divided left atrium
      • ■ Right-sided juxtaposition of the atrial appendages
  • Congenital anomaly of an atrioventricular valve or atrioventricular septum
    • Congenital anomaly of tricuspid valve
      • ■ Congenital tricuspid regurgitation
      • ■ Congenital tricuspid valvar stenosis
      • ■ Tricuspid annular hypoplasia
      • ■ Dysplasia of tricuspid valve
      • ■ Straddling tricuspid valve
      • ■ Overriding tricuspid valve
      • ■ Ebstein malformation of tricuspid valve
      • ■ Absent tricuspid valve leaflet
      • ■ True cleft of tricuspid valve leaflet
    • Congenital anomaly of mitral valve
      • ■ Congenital mitral regurgitation
      • ■ Congenital mitral valvar stenosis
      • ■ Mitral annular hypoplasia
      • ■ Straddling mitral valve
      • ■ Overriding mitral valve
      • ■ Dysplasia of mitral valve
      • Supravalvar or intravalvar mitral ring
        • ■ Congenital intravalvar mitral ring
        • ■ Congenital supravalvar mitral ring
      • ■ Congenital mitral valvar prolapse
      • ■ True cleft of anterior mitral leaflet
      • Congenital anomaly of mitral subvalvar apparatus
        • ■ Congenital mitral subvalvar stenosis
        • ■ Parachute malformation of mitral valve
      • ■ Accessory tissue on mitral valve leaflet
      • ■ Congenital unguarded mitral orifice
      • ■ Double orifice of mitral valve
    • ■ Congenital anomaly of left-sided atrioventricular valve in double inlet ventricle
    • ■ Congenital anomaly of right-sided atrioventricular valve in double inlet ventricle
    • Common atrioventricular junction
      • Common atrioventricular junction with atrioventricular septal defect
        • ■ Atrioventricular septal defect with balanced ventricles
        • Atrioventricular septal defect with ventricular imbalance
          • ■ Atrioventricular septal defect with ventricular imbalance with dominant right ventricle and hypoplastic left ventricle
          • ■ Atrioventricular septal defect with ventricular imbalance with dominant left ventricle and hypoplastic right ventricle
        • ■ Atrioventricular septal defect with communication at the atrial level only
        • ■ Atrioventricular septal defect with communication at the ventricular level only
        • ■ Atrioventricular septal defect with communication at atrial level and restrictive communication at ventricular level
        • ■ Atrioventricular septal defect with communication at atrial level and unrestrictive communication at ventricular level
        • ■ Atrioventricular septal defect and tetralogy of Fallot
        • ■ Common atrium with common atrioventricular junction
        • ■ Common atrioventricular valvar regurgitation
        • Atypical common atrioventricular valve
          • Common atrioventricular valve with unbalanced commitment of valve to ventricles
            • ■ Common atrioventricular valve with unbalanced commitment of valve to right ventricle
            • ■ Common atrioventricular valve with unbalanced commitment of valve to left ventricle
          • ■ Atypical right ventricular component of common atrioventricular valve
          • Atypical left ventricular component of common atrioventricular valve
            • ■ Double orifice of left ventricular component of common atrioventricular valve
            • ■ Deficient mural leaflet of left ventricular component of common atrioventricular valve
      • ■ Common atrioventricular junction without an atrioventricular septal defect
    • ■ Communication between left ventricle and right atrium
  • Congenital anomaly of a ventricle or the ventricular septum
    • Congenital right ventricular anomaly
      • ■ Right ventricular hypoplasia
      • ■ Congenital right ventricular outflow tract obstruction
      • ■ Double chambered right ventricle
      • ■ Right ventricular myocardial sinusoids
      • ■ Parchment right ventricle
      • Tetralogy of Fallot
        • ■ Tetralogy of Fallot with absent pulmonary valve syndrome
        • ■ Tetralogy of Fallot with pulmonary atresia
        • ■ Tetralogy of Fallot with pulmonary atresia and systemic-to-pulmonary collateral artery
    • Congenital left ventricular anomaly
      • ■ Left ventricular hypoplasia
      • Congenital left ventricular aneurysm or diverticulum
        • ■ Congenital left ventricular aneurysm
        • ■ Congenital left ventricular diverticulum
      • Congenital left ventricular outflow tract obstruction
        • ■ Congenital left ventricular outflow tract obstruction due to atrioventricular valve
      • ■ Left heart obstruction at multiple sites
      • ■ Left ventricular myocardial sinusoids
    • ■ Anomalous ventricular bands
    • Congenital anomaly of ventricular septum
      • ■ Restrictive interventricular communication when an interventricular shunt is physiologically necessary
      • Ventricular septal defect
        • ■ Perimembranous central ventricular septal defect
        • Inlet ventricular septal defect without a common atrioventricular junction
          • ■ Inlet perimembranous ventricular septal defect without atrioventricular septal malalignment without a common atrioventricular junction
          • ■ Inlet perimembranous ventricular septal defect with atrioventricular septal malalignment and without a common atrioventricular junction
          • ■ Inlet muscular ventricular septal defect
        • Trabecular muscular ventricular septal defect
          • ■ Trabecular muscular ventricular septal defect midseptal
          • ■ Trabecular muscular ventricular septal defect apical
          • ■ Trabecular muscular ventricular septal defect postero-inferior
          • ■ Trabecular muscular ventricular septal defect anterosuperior
          • ■ Multiple trabecular muscular ventricular septal defects
        • Outlet ventricular septal defect
          • Outlet ventricular septal defect without malalignment
            • ■ Outlet muscular ventricular septal defect without malalignment
            • Doubly committed juxta-arterial ventricular septal defect without malalignment
              • ■ Doubly committed juxta-arterial ventricular septal defect without malalignment and with muscular postero-inferior rim
              • ■ Doubly committed juxta-arterial ventricular septal defect without malalignment and with perimembranous extension
          • Outlet ventricular septal defect with anteriorly malaligned outlet septum
            • ■ Outlet muscular ventricular septal defect with anteriorly malaligned outlet septum
            • ■ Outlet perimembranous ventricular septal defect with anteriorly malaligned outlet septum
            • Doubly committed juxta-arterial ventricular septal defect with anteriorly malaligned fibrous outlet septum
              • ■ Doubly committed juxta-arterial ventricular septal defect with anteriorly malaligned fibrous outlet septum and muscular postero-inferior rim
              • ■ Doubly committed juxta-arterial ventricular septal defect with anteriorly malaligned fibrous outlet septum and perimembranous extension
          • Outlet ventricular septal defect with posteriorly malaligned outlet septum
            • ■ Outlet muscular ventricular septal defect with posteriorly malaligned outlet septum
            • ■ Outlet perimembranous ventricular septal defect with posteriorly malaligned outlet septum
            • Doubly committed juxta-arterial ventricular septal defect with posteriorly malaligned fibrous outlet septum
              • ■ Doubly committed juxta-arterial ventricular septal defect with posteriorly malaligned fibrous outlet septum and muscular postero-inferior rim
              • ■ Doubly committed juxta-arterial ventricular septal defect with posteriorly malaligned fibrous outlet septum and perimembranous extension
        • ■ Ventricular septal defect haemodynamically insignificant
        • ■ Multiple ventricular septal defects
  • Functionally univentricular heart
    • Double inlet atrioventricular connection
      • ■ Double inlet left ventricle
      • ■ Double inlet right ventricle
      • ■ Double inlet to solitary ventricle of indeterminate morphology
    • Tricuspid atresia
      • ■ Tricuspid atresia with absent atrioventricular connection
      • ■ Tricuspid atresia with imperforate tricuspid valve
    • Mitral atresia
      • ■ Mitral atresia with absent atrioventricular connection
      • ■ Mitral atresia with imperforate mitral valve
    • ■ Hypoplastic left heart syndrome
  • Congenital anomaly of a ventriculo-arterial valve or adjacent regions
    • Congenital anomaly of pulmonary valve
      • ■ Congenital pulmonary valvar stenosis
      • ■ Pulmonary annular hypoplasia
      • ■ Congenital pulmonary regurgitation
      • ■ Dysplasia of pulmonary valve
      • ■ Bicuspid pulmonary valve
    • ■ Congenital subpulmonary stenosis
    • ■ Congenital supravalvar pulmonary stenosis
    • Congenital pulmonary atresia
      • ■ Congenital pulmonary valvar atresia
      • ■ Pulmonary atresia with intact ventricular septum
    • Congenital anomaly of aortic valve
      • ■ Congenital aortic valvar stenosis
      • ■ Congenital aortic regurgitation
      • ■ Bicuspid aortic valve
      • ■ Unicuspid aortic valve
      • ■ Aortic valvar prolapse
      • ■ Aortic valvar atresia
      • ■ Aortic annular hypoplasia
      • ■ Dysplasia of aortic valve
    • Congenital subaortic stenosis
      • ■ Subaortic stenosis due to fibromuscular shelf
      • ■ Subaortic stenosis due to fibromuscular tunnel
    • ■ Congenital supravalvar aortic stenosis
    • ■ Aneurysm of aortic sinus of Valsalva
    • Aortoventricular tunnel
      • ■ Aorto-left ventricular tunnel
      • ■ Aorto-right ventricular tunnel
  • Congenital anomaly of great arteries including arterial duct
    • ■ Congenital aortopulmonary window
    • Congenital anomaly of pulmonary arterial tree
      • ■ Congenital dilation of pulmonary arterial tree
      • Congenital pulmonary trunk anomaly
        • ■ Congenital pulmonary trunk hypoplasia
        • ■ Absent or atretic pulmonary trunk
      • Congenital pulmonary arterial branch anomaly
        • Congenital pulmonary arterial branch stenosis
          • ■ Congenital right pulmonary arterial stenosis
          • ■ Congenital left pulmonary arterial stenosis
        • Congenital pulmonary arterial branch hypoplasia
          • ■ Congenital right pulmonary arterial hypoplasia
          • ■ Congenital left pulmonary arterial hypoplasia
        • Absent or atretic right or left pulmonary artery
          • ■ Absent or atretic right pulmonary artery
          • ■ Absent or atretic left pulmonary artery
        • ■ Congenital central pulmonary arterial stenosis or hypoplasia proximal to hilar bifurcation
        • ■ Congenital peripheral pulmonary arterial stenosis or hypoplasia at or beyond hilar bifurcation
        • ■ Congenitally discontinuous, non-confluent right and left pulmonary arteries
        • Pulmonary artery origin from ascending aorta
          • ■ Right pulmonary artery from ascending aorta
          • ■ Left pulmonary artery from ascending aorta
        • Pulmonary artery from arterial duct
          • ■ Right pulmonary artery from arterial duct
          • ■ Left pulmonary artery from arterial duct
    • Congenital anomaly of aorta or its branches
      • Congenital anomaly of ascending aorta
        • ■ Hypoplasia of ascending aortic
        • ■ Congenital ascending aortic aneurysm or dilation
      • Congenital anomaly of aortic arch
        • ■ Hypoplasia of aortic arch
        • Interrupted aortic arch
          • ■ Interrupted aortic arch distal to subclavian artery, type A
          • ■ Interrupted aortic arch between subclavian and common carotid arteries, type B
          • ■ Interrupted aortic arch between carotid arteries, type C
        • ■ Right aortic arch
        • ■ Left aortic arch
        • ■ Cervical aortic arch
        • ■ Aortic diverticulum of Kommerell
        • ■ Persistent fifth aortic arch
      • Coarctation of aorta
        • ■ Preductal coarctation of aorta
        • ■ Postductal coarctation of aorta
        • ■ Juxtaductal coarctation of aorta
      • Congenital anomaly of aortic arch branch
        • ■ Aberrant origin of right subclavian artery
        • ■ Aberrant origin of left subclavian artery
        • Isolation of an aortic arch branch
          • ■ Isolation of innominate artery
          • ■ Isolation of left subclavian artery
          • ■ Isolation of right subclavian artery
          • ■ Isolation of left common carotid artery
          • ■ Isolation of right common carotid artery
        • ■ Aberrant origin of innominate artery
        • ■ Common origin of the innominate artery and left common carotid artery
        • ■ Separate origins of internal and external carotid arteries
      • Congenital anomaly of descending thoracic or abdominal aorta
        • Descending thoracic or abdominal aortic coarctation
          • ■ Coarctation of the descending thoracic aorta
          • ■ Coarctation of the abdominal aorta
    • Tracheo-oesophageal compressive syndrome
      • Innominate artery compression syndrome
        • ■ Retro-oesophageal origin of aberrant innominate artery
    • Vascular Ring
      • ■ Double aortic arch
      • ■ Vascular ring of right aortic arch and left arterial duct or ligament
      • ■ Vascular ring of left aortic arch and right arterial duct or ligament
    • ■ Anomalous origin of left pulmonary artery from right pulmonary artery
    • Congenital arterial duct anomaly
      • ■ Patent arterial duct
      • ■ Absent arterial duct
      • ■ Congenital aneurysm of arterial duct
      • ■ Anomalous origin of arterial duct
      • ■ Anomalous origin of arterial ligament
      • ■ Fetal arterial duct narrowing-closure
    • ■ Systemic-to-pulmonary collateral arteries
  • Congenital anomaly of coronary artery
    • Anomalous origin of coronary artery from pulmonary arterial tree
      • ■ Anomalous origin of left coronary artery from pulmonary artery
    • Anomalous aortic origin or course of coronary artery
      • Anomalous aortic origin of coronary artery with ventriculo-arterial concordance
        • ■ Right coronary artery from left aortic sinus with ventriculo-arterial concordance
        • ■ Left coronary artery from right aortic sinus with ventriculo-arterial concordance
      • ■ Anterior descending from right coronary artery across right ventricular outflow tract
      • ■ Intramural proximal coronary arterial course
      • ■ Single coronary artery supplying all of heart
    • ■ Myocardial bridging of coronary artery
    • ■ Congenital coronary arterial orifice stenosis
    • ■ Congenital coronary arterial orifice atresia
    • Congenital coronary arterial fistula
      • ■ Congenital coronary arterial fistula to right ventricle
      • ■ Congenital coronary arterial fistula to left ventricle
    • ■ Congenital coronary arterial aneurysm
    • ■ Accessory coronary artery
    • ■ Congenital absence of coronary artery
    • ■ Coronary arterial hypoplasia
  • Congenital pericardial anomaly
    • ■ Complete agenesis of pericardium
    • ■ Partial agenesis of pericardium
    • ■ Pleuropericardial cyst
  • ■ Congenital cardiac tumour
  • ■ Pulmonary arteriovenous fistula
  • ■ Bifid apex of heart