ICD-10-CM Book 2026 Edition

Chapters  /  Chapter 17  /  Q20-Q28

Congenital malformations of the circulatory system (Q20-Q28)

Q20 Congenital malformations of cardiac chambers and connections
Excludes1 dextrocardia with situs inversus (Q89.3)
mirror-image atrial arrangement with situs inversus (Q89.3)
Q20.0 Common arterial trunk
Persistent truncus arteriosus
Excludes1 aortic septal defect (Q21.4)
Q20.1 Double outlet right ventricle
Taussig-Bing syndrome
Q20.2 Double outlet left ventricle
Q20.3 Discordant ventriculoarterial connection
Dextrotransposition of aorta
Transposition of great vessels (complete)
Q20.4 Double inlet ventricle
Common ventricle
Cor triloculare biatriatum
Single ventricle
Q20.5 Discordant atrioventricular connection
Corrected transposition
Levotransposition
Ventricular inversion
Q20.6 Isomerism of atrial appendages
Isomerism of atrial appendages with asplenia or polysplenia
Q20.8 Other congenital malformations of cardiac chambers and connections
Cor binoculare
Q20.9 Congenital malformation of cardiac chambers and connections, unspecified
Q21 Congenital malformations of cardiac septa
Excludes1 acquired cardiac septal defect (I51.0)
Q21.0 Ventricular septal defect
Roger's disease
Q21.1 Atrial septal defect
Excludes2 ostium primum atrial septal defect (type I) (Q21.20)
Q21.10 Atrial septal defect, unspecified
Q21.11 Secundum atrial septal defect
Fenestrated atrial septum
Patent or persistent ostium secundum defect (type II)
Q21.12 Patent foramen ovale
Persistent foramen ovale
Q21.13 Coronary sinus atrial septal defect
Coronary sinus defect
Unroofed coronary sinus
Q21.14 Superior sinus venosus atrial septal defect
Superior vena cava type atrial septal defect
Q21.15 Inferior sinus venosus atrial septal defect
Inferior vena cava type atrial septal defect
Q21.16 Sinus venosus atrial septal defect, unspecified
Sinus venosus defect, NOS
Q21.19 Other specified atrial septal defect
Common atrium
Other specified atrial septal abnormality
Q21.2 Atrioventricular septal defect
Atrioventricular canal defect
Endocardial cushion defect
Ostium primum atrial septal defect (type I)
Q21.20 Atrioventricular septal defect, unspecified as to partial or complete
Atrioventricular canal, NOS
Endocardial cushion defect NOS
Ostium primum atrial septal defect (type I) NOS
Q21.21 Partial atrioventricular septal defect
Incomplete atrioventricular canal
Incomplete atrioventricular septal defect
Incomplete endocardial cushion defect
Ostium primum atrial septal defect (type I) with separate atrioventricular valves
Partial atrioventricular canal
Partial endocardial cushion defect
Q21.22 Transitional atrioventricular septal defect
Intermediate atrioventricular canal
Intermediate atrioventricular septal defect
Intermediate endocardial cushion defect
Ostium primum atrial septal defect (type I) with separate atrioventricular valves and a small or restrictive inlet VSD
Transitional atrioventricular canal
Transitional endocardial cushion defect
Q21.23 Complete atrioventricular septal defect
Common atrioventricular canal
Common atrioventricular septal defect
Common endocardial cushion defect
Ostium primum atrial septal defect (type I) with common atrioventricular valve and a moderate or larger inlet VSD
Q21.3 Tetralogy of Fallot
Ventricular septal defect with pulmonary stenosis or atresia, dextroposition of aorta and hypertrophy of right ventricle.
Q21.4 Aortopulmonary septal defect
Aortic septal defect
Aortopulmonary window
Q21.8 Other congenital malformations of cardiac septa
Eisenmenger's defect
Pentalogy of Fallot
CodeAlso , if applicable:
Eisenmenger's complex (I27.83)
Eisenmenger's syndrome (I27.83)
Q21.9 Congenital malformation of cardiac septum, unspecified
Septal (heart) defect NOS
Q22 Congenital malformations of pulmonary and tricuspid valves
Q22.0 Pulmonary valve atresia
Q22.1 Congenital pulmonary valve stenosis
Q22.2 Congenital pulmonary valve insufficiency
Congenital pulmonary valve regurgitation
Q22.3 Other congenital malformations of pulmonary valve
Congenital malformation of pulmonary valve NOS
Supernumerary cusps of pulmonary valve
Q22.4 Congenital tricuspid stenosis
Congenital tricuspid atresia
Q22.5 Ebstein's anomaly
Q22.6 Hypoplastic right heart syndrome
Q22.8 Other congenital malformations of tricuspid valve
Q22.9 Congenital malformation of tricuspid valve, unspecified
Q23 Congenital malformations of aortic and mitral valves
Q23.0 Congenital stenosis of aortic valve
Congenital aortic atresia
Congenital aortic stenosis NOS
Excludes1 congenital stenosis of aortic valve in hypoplastic left heart syndrome (Q23.4)
congenital subaortic stenosis (Q24.4)
supravalvular aortic stenosis (congenital) (Q25.3)
Q23.1 Congenital insufficiency of aortic valve
Congenital aortic insufficiency
Q23.2 Congenital mitral stenosis
Congenital mitral atresia
Q23.3 Congenital mitral insufficiency
Q23.4 Hypoplastic left heart syndrome
Q23.8 Other congenital malformations of aortic and mitral valves
Q23.81 Bicuspid aortic valve
Congenital bicuspid aortic valve
Unicuspid (congenital) aortic valve (at birth)
CodeAlso , if applicable, acquired aortic valve disorders, such as:
aortic (valve) insufficiency (nonrheumatic) (I35.1)
aortic (valve) stenosis (nonrheumatic) (I35.0)
aortic (valve) stenosis with insufficiency (nonrheumatic) (I35.2)
Q23.82 Congenital mitral valve cleft leaflet
Cleft mitral valve leaflet at birth
Q23.88 Other congenital malformations of aortic and mitral valves
Q23.9 Congenital malformation of aortic and mitral valves, unspecified
Q24 Other congenital malformations of heart
Excludes1 endocardial fibroelastosis (I42.4)
Q24.0 Dextrocardia
Excludes1 dextrocardia with situs inversus (Q89.3)
isomerism of atrial appendages (with asplenia or polysplenia) (Q20.6)
mirror-image atrial arrangement with situs inversus (Q89.3)
Q24.1 Levocardia
Q24.2 Cor triatriatum
Q24.3 Pulmonary infundibular stenosis
Subvalvular pulmonic stenosis
Q24.4 Congenital subaortic stenosis
Q24.5 Malformation of coronary vessels
Congenital coronary (artery) aneurysm
Q24.6 Congenital heart block
Q24.8 Other specified congenital malformations of heart
Congenital diverticulum of left ventricle
Congenital malformation of myocardium
Congenital malformation of pericardium
Malposition of heart
Uhl's disease
Q24.9 Congenital malformation of heart, unspecified
Congenital anomaly of heart
Congenital disease of heart
Q25 Congenital malformations of great arteries
Q25.0 Patent ductus arteriosus
Patent ductus Botallo
Persistent ductus arteriosus
Q25.1 Coarctation of aorta
Coarctation of aorta (preductal) (postductal)
Stenosis of aorta
Q25.2 Atresia of aorta
Q25.21 Interruption of aortic arch
Atresia of aortic arch
Q25.29 Other atresia of aorta
Atresia of aorta
Q25.3 Supravalvular aortic stenosis
Excludes1 congenital aortic stenosis NOS (Q23.0)
congenital stenosis of aortic valve (Q23.0)
Q25.4 Other congenital malformations of aorta
Excludes1 hypoplasia of aorta in hypoplastic left heart syndrome (Q23.4)
Q25.40 Congenital malformation of aorta unspecified
Q25.41 Absence and aplasia of aorta
Q25.42 Hypoplasia of aorta
Q25.43 Congenital aneurysm of aorta
Congenital aneurysm of aortic root
Congenital aneurysm of aortic sinus
Q25.44 Congenital dilation of aorta
Q25.45 Double aortic arch
Vascular ring of aorta
Q25.46 Tortuous aortic arch
Persistent convolutions of aortic arch
Q25.47 Right aortic arch
Persistent right aortic arch
Q25.48 Anomalous origin of subclavian artery
Q25.49 Other congenital malformations of aorta
Aortic arch
Bovine arch
Q25.5 Atresia of pulmonary artery
Q25.6 Stenosis of pulmonary artery
Supravalvular pulmonary stenosis
Q25.7 Other congenital malformations of pulmonary artery
Q25.71 Coarctation of pulmonary artery
Q25.72 Congenital pulmonary arteriovenous malformation
Congenital pulmonary arteriovenous aneurysm
Q25.79 Other congenital malformations of pulmonary artery
Aberrant pulmonary artery
Agenesis of pulmonary artery
Congenital aneurysm of pulmonary artery
Congenital anomaly of pulmonary artery
Hypoplasia of pulmonary artery
Q25.8 Other congenital malformations of other great arteries
Q25.9 Congenital malformation of great arteries, unspecified
Q26 Congenital malformations of great veins
Q26.0 Congenital stenosis of vena cava
Congenital stenosis of vena cava (inferior)(superior)
Q26.1 Persistent left superior vena cava
Q26.2 Total anomalous pulmonary venous connection
Total anomalous pulmonary venous return [TAPVR], subdiaphragmatic
Total anomalous pulmonary venous return [TAPVR], supradiaphragmatic
Q26.3 Partial anomalous pulmonary venous connection
Partial anomalous pulmonary venous return
Q26.4 Anomalous pulmonary venous connection, unspecified
Q26.5 Anomalous portal venous connection
Q26.6 Portal vein-hepatic artery fistula
Q26.8 Other congenital malformations of great veins
Absence of vena cava (inferior) (superior)
Azygos continuation of inferior vena cava
Persistent left posterior cardinal vein
Scimitar syndrome
Q26.9 Congenital malformation of great vein, unspecified
Congenital anomaly of vena cava (inferior) (superior) NOS
Q27 Other congenital malformations of peripheral vascular system
Excludes2 anomalies of cerebral and precerebral vessels (Q28.0-Q28.3)
anomalies of coronary vessels (Q24.5)
anomalies of pulmonary artery (Q25.5-Q25.7)
congenital retinal aneurysm (Q14.1)
hemangioma and lymphangioma (D18.-)
Q27.0 Congenital absence and hypoplasia of umbilical artery
Single umbilical artery
Q27.1 Congenital renal artery stenosis
Q27.2 Other congenital malformations of renal artery
Congenital malformation of renal artery NOS
Multiple renal arteries
Q27.3 Arteriovenous malformation (peripheral)
Arteriovenous aneurysm
Excludes1 acquired arteriovenous aneurysm (I77.0)
Excludes2 arteriovenous malformation of cerebral vessels (Q28.2)
arteriovenous malformation of precerebral vessels (Q28.0)
Q27.30 Arteriovenous malformation, site unspecified
Q27.31 Arteriovenous malformation of vessel of upper limb
Q27.32 Arteriovenous malformation of vessel of lower limb
Q27.33 Arteriovenous malformation of digestive system vessel
Q27.34 Arteriovenous malformation of renal vessel
Q27.39 Arteriovenous malformation, other site
Q27.4 Congenital phlebectasia
Q27.8 Other specified congenital malformations of peripheral vascular system
Absence of peripheral vascular system
Atresia of peripheral vascular system
Congenital aneurysm (peripheral)
Congenital stricture, artery
Congenital varix
Excludes1 arteriovenous malformation (Q27.3-)
Q27.9 Congenital malformation of peripheral vascular system, unspecified
Anomaly of artery or vein NOS
Q28 Other congenital malformations of circulatory system
Excludes1 congenital aneurysm NOS (Q27.8)
congenital coronary aneurysm (Q24.5)
ruptured cerebral arteriovenous malformation (I60.8)
ruptured malformation of precerebral vessels (I72.0)
Excludes2 congenital peripheral aneurysm (Q27.8)
congenital pulmonary aneurysm (Q25.79)
congenital retinal aneurysm (Q14.1)
Q28.0 Arteriovenous malformation of precerebral vessels
Congenital arteriovenous precerebral aneurysm (nonruptured)
Q28.1 Other malformations of precerebral vessels
Congenital malformation of precerebral vessels NOS
Congenital precerebral aneurysm (nonruptured)
Q28.2 Arteriovenous malformation of cerebral vessels
Arteriovenous malformation of brain NOS
Congenital arteriovenous cerebral aneurysm (nonruptured)
Q28.3 Other malformations of cerebral vessels
Congenital cerebral aneurysm (nonruptured)
Congenital malformation of cerebral vessels NOS
Developmental venous anomaly
Q28.8 Other specified congenital malformations of circulatory system
Congenital aneurysm, specified site NEC
Spinal vessel anomaly
Excludes2 disorders of pyrophosphate metabolism (E83.82-)
Q28.9 Congenital malformation of circulatory system, unspecified