Congenital malformations of the circulatory system (Q20-Q28)
Q20Congenital malformations of cardiac chambers and connections
Excludes 1: dextrocardia with situs inversus (Q89.3)
› mirror-image atrial arrangement with situs inversus (Q89.3)
Q20.0Common arterial trunk
› Persistent truncus arteriosus
Excludes 1: aortic septal defect (Q21.4)
Q20.1Double outlet right ventricle
› Taussig-Bing syndrome
Q20.2Double outlet left ventricle
Q20.3Discordant ventriculoarterial connection
› Dextrotransposition of aorta
› Transposition of great vessels (complete)
Q20.4Double inlet ventricle
› Common ventricle
› Cor triloculare biatriatum
› Single ventricle
Q20.5Discordant atrioventricular connection
› Corrected transposition
› Levotransposition
› Ventricular inversion
Q20.6Isomerism of atrial appendages
› Isomerism of atrial appendages with asplenia or polysplenia
Q20.8Other congenital malformations of cardiac chambers and connections
› Cor binoculare
Q20.9Congenital malformation of cardiac chambers and connections, unspecified
Q21Congenital malformations of cardiac septa
Excludes 1: acquired cardiac septal defect (I51.0)
Q21.0Ventricular septal defect
› Roger's disease
Q21.1Atrial septal defect
Excludes 2: ostium primum atrial septal defect (type I) (Q21.20)
Q21.10Atrial septal defect, unspecified
Q21.11Secundum atrial septal defect
› Fenestrated atrial septum
› Patent or persistent ostium secundum defect (type II)
Q21.12Patent foramen ovale
› Persistent foramen ovale
Q21.13Coronary sinus atrial septal defect
› Coronary sinus defect
› Unroofed coronary sinus
Q21.14Superior sinus venosus atrial septal defect
› Superior vena cava type atrial septal defect
Q21.15Inferior sinus venosus atrial septal defect
› Inferior vena cava type atrial septal defect
Q21.16Sinus venosus atrial septal defect, unspecified
› Sinus venosus defect, NOS
Q21.19Other specified atrial septal defect
› Common atrium
› Other specified atrial septal abnormality
Q21.2Atrioventricular septal defect
› Atrioventricular canal defect
› Endocardial cushion defect
› Ostium primum atrial septal defect (type I)
Q21.20Atrioventricular septal defect, unspecified as to partial or complete
› Atrioventricular canal, NOS
› Endocardial cushion defect NOS
› Ostium primum atrial septal defect (type I) NOS
Q21.21Partial 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
Q21.22Transitional 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
Q21.23Complete 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
Q21.3Tetralogy of Fallot
› Ventricular septal defect with pulmonary stenosis or atresia, dextroposition of aorta and hypertrophy of right ventricle.
Q21.4Aortopulmonary septal defect
› Aortic septal defect
› Aortopulmonary window
Q21.8Other congenital malformations of cardiac septa
› Eisenmenger's defect
› Pentalogy of Fallot
Code Also: , if applicable:
› Eisenmenger's complex (I27.83)
› Eisenmenger's syndrome (I27.83)
Q21.9Congenital malformation of cardiac septum, unspecified
› Septal (heart) defect NOS
Q22Congenital malformations of pulmonary and tricuspid valves
Q22.0Pulmonary valve atresia
Q22.1Congenital pulmonary valve stenosis
Q22.2Congenital pulmonary valve insufficiency
› Congenital pulmonary valve regurgitation
Q22.3Other congenital malformations of pulmonary valve
› Congenital malformation of pulmonary valve NOS
› Supernumerary cusps of pulmonary valve
Q22.4Congenital tricuspid stenosis
› Congenital tricuspid atresia
Q22.5Ebstein's anomaly
Q22.6Hypoplastic right heart syndrome
Q22.8Other congenital malformations of tricuspid valve
Q22.9Congenital malformation of tricuspid valve, unspecified
Q23Congenital malformations of aortic and mitral valves
Q23.0Congenital stenosis of aortic valve
› Congenital aortic atresia
› Congenital aortic stenosis NOS
Excludes 1: congenital stenosis of aortic valve in hypoplastic left heart syndrome (Q23.4)
› congenital subaortic stenosis (Q24.4)
› supravalvular aortic stenosis (congenital) (Q25.3)
Q23.1Congenital insufficiency of aortic valve
› Bicuspid aortic valve
› Congenital aortic insufficiency
Q23.2Congenital mitral stenosis
› Congenital mitral atresia
Q23.3Congenital mitral insufficiency
Q23.4Hypoplastic left heart syndrome
Q23.8Other congenital malformations of aortic and mitral valves
Q23.9Congenital malformation of aortic and mitral valves, unspecified
Q24Other congenital malformations of heart
Excludes 1: endocardial fibroelastosis (I42.4)
Q24.0Dextrocardia
Excludes 1: 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)
Q24.1Levocardia
Q24.2Cor triatriatum
Q24.3Pulmonary infundibular stenosis
› Subvalvular pulmonic stenosis
Q24.4Congenital subaortic stenosis
Q24.5Malformation of coronary vessels
› Congenital coronary (artery) aneurysm
Q24.6Congenital heart block
Q24.8Other specified congenital malformations of heart
› Congenital diverticulum of left ventricle
› Congenital malformation of myocardium
› Congenital malformation of pericardium
› Malposition of heart
› Uhl's disease
Q24.9Congenital malformation of heart, unspecified
› Congenital anomaly of heart
› Congenital disease of heart
Q25Congenital malformations of great arteries
Q25.0Patent ductus arteriosus
› Patent ductus Botallo
› Persistent ductus arteriosus
Q25.1Coarctation of aorta
› Coarctation of aorta (preductal) (postductal)
› Stenosis of aorta
Q25.2Atresia of aorta
Q25.21Interruption of aortic arch
› Atresia of aortic arch
Q25.29Other atresia of aorta
› Atresia of aorta
Q25.3Supravalvular aortic stenosis
Excludes 1: congenital aortic stenosis NOS (Q23.0)
› congenital stenosis of aortic valve (Q23.0)
Q25.4Other congenital malformations of aorta
Excludes 1: hypoplasia of aorta in hypoplastic left heart syndrome (Q23.4)
Q25.40Congenital malformation of aorta unspecified
Q25.41Absence and aplasia of aorta
Q25.42Hypoplasia of aorta
Q25.43Congenital aneurysm of aorta
› Congenital aneurysm of aortic root
› Congenital aneurysm of aortic sinus
Q25.44Congenital dilation of aorta
Q25.45Double aortic arch
› Vascular ring of aorta
Q25.46Tortuous aortic arch
› Persistent convolutions of aortic arch
Q25.47Right aortic arch
› Persistent right aortic arch
Q25.48Anomalous origin of subclavian artery
Q25.49Other congenital malformations of aorta
› Aortic arch
› Bovine arch
Q25.5Atresia of pulmonary artery
Q25.6Stenosis of pulmonary artery
› Supravalvular pulmonary stenosis
Q25.7Other congenital malformations of pulmonary artery
Q25.71Coarctation of pulmonary artery
Q25.72Congenital pulmonary arteriovenous malformation
› Congenital pulmonary arteriovenous aneurysm
Q25.79Other 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
Q25.8Other congenital malformations of other great arteries
Q25.9Congenital malformation of great arteries, unspecified
Q26Congenital malformations of great veins
Q26.0Congenital stenosis of vena cava
› Congenital stenosis of vena cava (inferior)(superior)
Q26.1Persistent left superior vena cava
Q26.2Total anomalous pulmonary venous connection
› Total anomalous pulmonary venous return [TAPVR], subdiaphragmatic
› Total anomalous pulmonary venous return [TAPVR], supradiaphragmatic
Q26.3Partial anomalous pulmonary venous connection
› Partial anomalous pulmonary venous return
Q26.4Anomalous pulmonary venous connection, unspecified
Q26.5Anomalous portal venous connection
Q26.6Portal vein-hepatic artery fistula
Q26.8Other congenital malformations of great veins
› Absence of vena cava (inferior) (superior)
› Azygos continuation of inferior vena cava
› Persistent left posterior cardinal vein
› Scimitar syndrome
Q26.9Congenital malformation of great vein, unspecified
› Congenital anomaly of vena cava (inferior) (superior) NOS
Q27Other congenital malformations of peripheral vascular system
Excludes 2: 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.-)
Q27.0Congenital absence and hypoplasia of umbilical artery
› Single umbilical artery
Q27.1Congenital renal artery stenosis
Q27.2Other congenital malformations of renal artery
› Congenital malformation of renal artery NOS
› Multiple renal arteries
Q27.3Arteriovenous malformation (peripheral)
› Arteriovenous aneurysm
Excludes 1: acquired arteriovenous aneurysm (I77.0)
Excludes 2: arteriovenous malformation of cerebral vessels (Q28.2)
› arteriovenous malformation of precerebral vessels (Q28.0)
Q27.30Arteriovenous malformation, site unspecified
Q27.31Arteriovenous malformation of vessel of upper limb
Q27.32Arteriovenous malformation of vessel of lower limb
Q27.33Arteriovenous malformation of digestive system vessel
Q27.34Arteriovenous malformation of renal vessel
Q27.39Arteriovenous malformation, other site
Q27.4Congenital phlebectasia
Q27.8Other 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
Excludes 1: arteriovenous malformation (Q27.3-)
Q27.9Congenital malformation of peripheral vascular system, unspecified
› Anomaly of artery or vein NOS
Q28Other congenital malformations of circulatory system
Excludes 1: congenital aneurysm NOS (Q27.8)
› congenital coronary aneurysm (Q24.5)
› ruptured cerebral arteriovenous malformation (I60.8)
› ruptured malformation of precerebral vessels (I72.0)
Excludes 2: congenital peripheral aneurysm (Q27.8)
› congenital pulmonary aneurysm (Q25.79)
› congenital retinal aneurysm (Q14.1)
Q28.0Arteriovenous malformation of precerebral vessels
› Congenital arteriovenous precerebral aneurysm (nonruptured)
Q28.1Other malformations of precerebral vessels
› Congenital malformation of precerebral vessels NOS
› Congenital precerebral aneurysm (nonruptured)
Q28.2Arteriovenous malformation of cerebral vessels
› Arteriovenous malformation of brain NOS
› Congenital arteriovenous cerebral aneurysm (nonruptured)
Q28.3Other malformations of cerebral vessels
› Congenital cerebral aneurysm (nonruptured)
› Congenital malformation of cerebral vessels NOS
› Developmental venous anomaly
Q28.8Other specified congenital malformations of circulatory system
› Congenital aneurysm, specified site NEC
› Spinal vessel anomaly
Q28.9Congenital malformation of circulatory system, unspecified