Chapter 17

Q00–Q99: Congenital Malformations, Deformations and Chromosomal Abnormalities

Congenital Malformations, Deformations and Chromosomal Abnormalities diagnostic classification updated for 2026.

Chapter Instructions

Excludes2(Not included here. Code separately if applicable.)

inborn errors of metabolism (E70-E88)

All Codes Billable Only

Internal Codes

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Q25.46|
Tortuous aortic arch
Q25.47|
Right aortic arch
Q25.48|
Anomalous origin of subclavian artery
Q25.49|
Other congenital malformations of aorta
Q25.5|
Atresia of pulmonary artery
Q25.6|
Stenosis of pulmonary artery
Q25.7|
Other congenital malformations of pulmonary artery
Q25.71|
Coarctation of pulmonary artery
Q25.72|
Congenital pulmonary arteriovenous malformation
Q25.79|
Other congenital malformations 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
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
Q26.9|
Congenital malformation of great vein, unspecified
Q27|
Other congenital malformations of peripheral vascular system
Q27.0|
Congenital absence and hypoplasia of umbilical artery
Q27.1|
Congenital renal artery stenosis
Q27.2|
Other congenital malformations of renal artery
Q27.3|
Arteriovenous malformation (peripheral)
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
Q27.9|
Congenital malformation of peripheral vascular system, unspecified
Q28|
Other congenital malformations of circulatory system
Q28.0|
Arteriovenous malformation of precerebral vessels
Q28.1|
Other malformations of precerebral vessels
Q28.2|
Arteriovenous malformation of cerebral vessels
Q28.3|
Other malformations of cerebral vessels
Q28.8|
Other specified congenital malformations of circulatory system
Q28.9|
Congenital malformation of circulatory system, unspecified
Q30|
Congenital malformations of nose
Q30.0|
Choanal atresia
Q30.1|
Agenesis and underdevelopment of nose
Q30.2|
Fissured, notched and cleft nose
Q30.3|
Congenital perforated nasal septum
Q30.8|
Other congenital malformations of nose
Q30.9|
Congenital malformation of nose, unspecified

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