Congenital malformations of great veins (Q26) ICD-10-CM
The Q26 code range covers congenital malformations of great veins with 10 ICD-10-CM diagnosis codes. 9 of them are billable and valid for claim submission in fiscal year 2027, and the category headers group them but cannot themselves be billed.
Codes in the Q26 Range 10 codes · 9 billable
- Q26 Congenital malformations of great veinsNon-billable
- 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
Clinical Terms in This Code Range
Definitions from the National Library of Medicine for conditions coded in the Q26 range.
Partial Anomalous Pulmonary Venous Return
A congenital heart disorder in which one or two pulmonary veins are not connected to the left atrium and drain into the right atrium instead. It may lead to arrhythmias and pulmonary hypertension.
Persistent Left Superior Vena Cava
Finding of the SUPERIOR VENA CAVA on the left instead of the usual right side of the ASCENDING AORTA. In bilateral superior vena cava it is found on both sides.
Scimitar Syndrome
An anomalous pulmonary venous return in which the right PULMONARY VEIN is not connected to the LEFT ATRIUM but to the INFERIOR VENA CAVA. Scimitar syndrome is named for the crescent- or Turkish sword-like shadow in the chest radiography and is often associated with hypoplasia of the right lung and right pulmonary artery, and dextroposition of the heart.
About the Q26 Code Range
These are abnormalities of the great veins present at birth, within the circulatory system.
The subdivisions distinguish the vein involved and the type of abnormality. Q26.0 describes a narrowed vena cava, while Q26.1 names a persistent left superior vena cava. Pulmonary venous connections are divided into total, partial and unspecified forms under Q26.2, Q26.3 and Q26.4. Q26.5 concerns a portal venous connection; Q26.6 names a connection between the portal vein and hepatic artery. Q26.8 and Q26.9 distinguish other specified malformations from an unspecified malformation.
Questions About This Page
How many billable codes are in the Q26 range?
Of the 10 codes in this range, 9 are billable and valid for claim submission from October 1, 2026 through September 30, 2027. Category header codes group them but cannot be reported on claims.
What does the Q26 range classify?
The range classifies congenital malformations of great veins. Each code links to its own reference page with billing status, MS-DRG grouping, coding notes, and clinical information.
Related References
Source: CMS FY 2027 ICD-10-CM Tabular List and order file, effective October 1, 2026 through September 30, 2027.
