Chapter XVII
Congenital malformations, deformations and chromosomal abnormalities
(Q00-Q99)

Rules Conventions GCS Guidance

Congenital malformations of the circulatory system
(Q20-Q28)

Q20 Congenital malformations of cardiac chambers and connections
Guidance

Excl.:
dextrocardia with situs inversus ( Q89.3)
mirror-image atrial arrangement with situs inversus ( Q89.3)
Q20.0 Common arterial trunk
Incl.:
Persistent truncus arteriosus
Q20.1 Double outlet right ventricle
Incl.:
Taussig-Bing syndrome
Q20.2 Double outlet left ventricle
Q20.3 Discordant ventriculoarterial connection
Incl.:
Dextrotransposition of aorta
Transposition of great vessels (complete)
Q20.4 Double inlet ventricle
Incl.:
Common ventricle
Cor triloculare biatriatum
Single ventricle
Q20.5 Discordant atrioventricular connection
Incl.:
Corrected transposition
Laevotransposition
Ventricular inversion
Q20.6 Isomerism of atrial appendages
Incl.:
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
Guidance

Excl.:
acquired cardiac septal defect ( I51.0)
Q21.0 Ventricular septal defect
Q21.1 Atrial septal defect
Incl.:
Coronary sinus defect
Patent or persistent:
  • foramen ovale
  • ostium secundum defect (type II)
Sinus venosus defect
Q21.2 Atrioventricular septal defect
Incl.:
Common atrioventricular canal
Endocardial cushion defect
Ostium primum atrial septal defect (type I)
Q21.3 Tetralogy of Fallot
Additional Text

Ventricular septal defect with pulmonary stenosis or atresia, dextroposition of aorta and hypertrophy of right ventricle.

Q21.4 Aortopulmonary septal defect
Incl.:
Aortic septal defect
Aortopulmonary window
Q21.8 Other congenital malformations of cardiac septa
Incl.:
Eisenmenger defect
Pentalogy of Fallot
Excl.:
Eisenmenger
Q21.9 Congenital malformation of cardiac septum, unspecified
Incl.:
Septal (heart) defect NOS

Q22 Congenital malformations of pulmonary and tricuspid valves
Guidance

Q22.0 Pulmonary valve atresia
Q22.1 Congenital pulmonary valve stenosis
Q22.2 Congenital pulmonary valve insufficiency
Incl.:
Congenital pulmonary valve regurgitation
Q22.3 Other congenital malformations of pulmonary valve
Incl.:
Congenital malformation of pulmonary valve NOS
Q22.4 Congenital tricuspid stenosis
Incl.:
Tricuspid atresia
Q22.5 Ebstein 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
Guidance

Q23.0 Congenital stenosis of aortic valve
Incl.:
Congenital aortic:
  • atresia
  • stenosis
Excl.:
congenital subaortic stenosis ( Q24.4)
that in hypoplastic left heart syndrome ( Q23.4)
Q23.1 Congenital insufficiency of aortic valve
Incl.:
Bicuspid aortic valve
Congenital aortic insufficiency
Q23.2 Congenital mitral stenosis
Incl.:
Congenital mitral atresia
Q23.3 Congenital mitral insufficiency
Q23.4 Hypoplastic left heart syndrome
Additional Text

Atresia, or marked hypoplasia of aortic orifice or valve, with hypoplasia of ascending aorta and defective development 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
Guidance

Excl.:
endocardial fibroelastosis ( I42.4)
Q24.0 Dextrocardia
Excl.:
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
Definition

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
Incl.:
Congenital coronary (artery) aneurysm
Q24.6 Congenital heart block
Q24.8 Other specified congenital malformations of heart
Incl.:
Congenital:
  • diverticulum of left ventricle
  • malformation of:
    • —  myocardium
    • —  pericardium
Malposition of heart
Uhl disease
Q24.9 Congenital malformation of heart, unspecified
Incl.:
  • Congenital:
    • anomaly
    • disease
  • NOS of heart

Q25 Congenital malformations of great arteries
Guidance

Q25.0 Patent ductus arteriosus
Incl.:
Patent ductus Botalli
Persistent ductus arteriosus
Q25.1 Coarctation of aorta
Incl.:
Coarctation of aorta (preductal)(postductal)
Q25.2 Atresia of aorta
Q25.3 Stenosis of aorta
Incl.:
Supravalvular aortic stenosis
Excl.:
congenital stenosis of aortic valve ( Q23.0)
Q25.4 Other congenital malformations of aorta
Incl.:
  • Absence
  • Aplasia
  • Congenital:
    • aneurysm
    • dilatation
  • of aorta
Aneurysm of sinus of Valsalva (ruptured)
Double aortic arch [vascular ring of aorta]
Hypoplasia of aorta
Persistent:
  • convolutions of aortic arch
  • right aortic arch
Excl.:
hypoplasia of aorta in hypoplastic left heart syndrome ( Q23.4)
Q25.5 Atresia of pulmonary artery
Q25.6 Stenosis of pulmonary artery
Incl.:
Supravalvular pulmonary stenosis
Q25.7 Other congenital malformations of pulmonary artery
Incl.:
Aberrant pulmonary artery
  • Agenesis
  • Aneurysm, congenital
  • Anomaly
  • Hypoplasia
  • 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
Guidance

Q26.0 Congenital stenosis of vena cava
Incl.:
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
Incl.:
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
Incl.:
Anomaly of vena cava (inferior)(superior) NOS

Q27 Other congenital malformations of peripheral vascular system

Excl.:
anomalies of:
congenital retinal aneurysm ( Q14.1)
haemangioma and lymphangioma ( D18.-)
Q27.0 Congenital absence and hypoplasia of umbilical artery
Incl.:
Single umbilical artery
Q27.1 Congenital renal artery stenosis
Q27.2 Other congenital malformations of renal artery
Incl.:
Congenital malformation of renal artery NOS
Multiple renal arteries
Q27.3 Peripheral arteriovenous malformation
Incl.:
Arteriovenous aneurysm
Excl.:
acquired arteriovenous aneurysm ( I77.0)
Q27.4 Congenital phlebectasia
Q27.8 Other specified congenital malformations of peripheral vascular system
Incl.:
Aberrant subclavian artery
  • Absence
  • Atresia
  • of artery or vein NEC
Congenital:
  • aneurysm (peripheral)
  • stricture, artery
  • varix
Q27.9 Congenital malformation of peripheral vascular system, unspecified
Incl.:
Anomaly of artery or vein NOS

Q28 Other congenital malformations of circulatory system

Excl.:
congenital aneurysm:
ruptured:
  • cerebral arteriovenous malformation (I60.8)
  • malformation of precerebral vessels (I72.-)
Q28.0 Arteriovenous malformation of precerebral vessels
Incl.:
Congenital arteriovenous precerebral aneurysm (nonruptured)
Q28.1 Other malformations of precerebral vessels
Incl.:
Congenital:
  • malformation of precerebral vessels NOS
  • precerebral aneurysm (nonruptured)
Q28.2 Arteriovenous malformation of cerebral vessels
Incl.:
Arteriovenous malformation of brain NOS
Congenital arteriovenous cerebral aneurysm (nonruptured)
Q28.3 Other malformations of cerebral vessels
Incl.:
Congenital:
  • cerebral aneurysm (nonruptured)
  • malformation of cerebral vessels NOS
Q28.8 Other specified congenital malformations of circulatory system
Incl.:
Congenital aneurysm, specified site NEC
Q28.9 Congenital malformation of circulatory system, unspecified