Chapter XVII

Congenital malformations, deformations and chromosomal abnormalities

Congenital malformations of the circulatory system

Q20 Congenital malformations of cardiac chambers and connections
Excludes: dextrocardia with situs inversus ( Q89.3 )
mirror-image atrial arrangement with situs inversus ( Q89.3 )
Q20.0 Common arterial trunk
Persistent truncus arteriosus
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
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
Q20.9 Congenital malformation of cardiac chambers and connections, unspecified

Q21 Congenital malformations of cardiac septa
Excludes: acquired cardiac septal defect ( I51.0 )
Q21.0 Ventricular septal defect
Q21.1 Atrial septal defect
Coronary sinus defect
Patent or persistent:
· foramen ovale
· ostium secundum defect (type II)
Sinus venosus defect
Q21.2 Atrioventricular septal defect
Common atrioventricular canal
Endocardial cushion defect
Ostium primum atrial septal defect (type I)
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
Excludes: Eisenmenger's
· complex ( I27.8 )
· syndrome ( I27.8 )
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
Q22.4 Congenital tricuspid stenosis
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
· stenosis
Excludes: congenital subaortic stenosis ( Q24.4 )
that in hypoplastic left heart syndrome ( Q23.4 )
Q23.1 Congenital insufficiency of aortic valve
Bicuspid aortic valve
Congenital aortic insufficiency
Q23.2 Congenital mitral stenosis
Congenital mitral atresia
Q23.3 Congenital mitral insufficiency
Q23.4 Hypoplastic left heart syndrome
Atresia, or marked hypoplasia of aortic orifice or valve, with hypoplasia of ascending aorta and defective develop-ment of left ventricle (with mitral valve stenosis or atresia).
Q23.8 Other congenital malformations of aortic and mitral valves
Q23.9 Congenital malformation of aortic and mitral valves, unspecified

Q24 Other congenital malformations of heart
Excludes: endocardial fibroelastosis ( I42.4 )
Q24.0 Dextrocardia
Excludes: 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 Laevocardia
Location of heart in left hemithorax with apex pointing to the left, but with situs inversus of other viscera and defects of the heart, or corrected transposition of great vessels.
Q24.2 Cor triatriatum
Q24.3 Pulmonary infundibular 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
· diverticulum of left ventricle
· malformation of:
  · myocardium
  · pericardium
Malposition of heart
Uhl's disease
Q24.9 Congenital malformation of heart, unspecified
· anomaly
· disease
NOS 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)
Q25.2 Atresia of aorta
Q25.3 Stenosis of aorta
Supravalvular aortic stenosis
Excludes: congenital aortic stenosis ( Q23.0 )
Q25.4 Other congenital malformations of aorta
· aneurysm
· dilatation
of aorta
Aneurysm of sinus of Valsalva (ruptured)
Double aortic arch [vascular ring of aorta]
Hypoplasia of aorta
· convolutions of aortic arch
· right aortic arch
Excludes: hypoplasia of aorta in hypoplastic left heart syndrome ( Q23.4 )
Q25.5 Atresia of pulmonary artery
Q25.6 Stenosis of pulmonary artery
Q25.7 Other congenital malformations of pulmonary artery
Aberrant pulmonary artery
Aneurysm, congenital
of pulmonary artery
Pulmonary arteriovenous aneurysm
Q25.8 Other congenital malformations of 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
Q26.3 Partial anomalous pulmonary venous connection
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
Anomaly of vena cava (inferior)(superior) NOS

Q27 Other congenital malformations of peripheral vascular system
Excludes: anomalies of:
· cerebral and precerebral vessels ( Q28.0-Q28.3 )
· coronary vessels ( Q24.5 )
· pulmonary artery ( Q25.5-Q25.7 )
congenital retinal aneurysm ( Q14.1 )
haemangioma 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 Peripheral arteriovenous malformation
Arteriovenous aneurysm
Excludes: acquired arteriovenous aneurysm ( I77.0 )
Q27.4 Congenital phlebectasia
Q27.8 Other specified congenital malformations of peripheral vascular system
Aberrant subclavian artery
of artery or vein NEC
· aneurysm (peripheral)
· stricture, artery
· varix
Q27.9 Congenital malformation of peripheral vascular system, unspecified
Anomaly of artery or vein NOS

Q28 Other congenital malformations of circulatory system
Excludes: congenital aneurysm:
· NOS ( Q27.8 )
· coronary ( Q24.5 )
· peripheral ( Q27.8 )
· pulmonary ( Q25.7 )
· retinal ( Q14.1 )
· cerebral arteriovenous malformation ( I60.8 )
· malformation of precerebral vessels ( I72.- )
Q28.0 Arteriovenous malformation of precerebral vessels
Congenital arteriovenous precerebral aneurysm (nonruptured)
Q28.1 Other malformations of precerebral vessels
· malformation of precerebral vessels NOS
· 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
· cerebral aneurysm (nonruptured)
· malformation of cerebral vessels NOS
Q28.8 Other specified congenital malformations of circulatory system
Congenital aneurysm, specified site NEC
Q28.9 Congenital malformation of circulatory system, unspecified