ICD-10-CM 2027
ICD-10 code for portal vein anomaly
From the official ICD-10-CM alphabetic index entry “Anomaly, anomalous (congenital) (unspecified type) › portal vein”. Page updated September 29, 2026.
About coding portal vein anomaly
The ICD-10-CM code for portal vein anomaly is Q26.5 (Anomalous portal venous connection). The official index lists 1 more specific code, so check the documentation for details such as type, cause, site or severity before settling on Q26.5.
Within Q26 (congenital malformations of great veins), Q26.5 is specifically for anomalous portal venous connection. Related codes cover congenital stenosis of vena cava (Q26.0), persistent left superior vena cava (Q26.1), total anomalous pulmonary venous connection (Q26.2), partial anomalous pulmonary venous connection (Q26.3), anomalous pulmonary venous connection, unspecified (Q26.4), portal vein-hepatic artery fistula (Q26.6), other congenital malformations of great veins (Q26.8) and congenital malformation of great vein, unspecified (Q26.9).
It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.
In MS-DRG v44.0, it is part of the grouping logic for DRG 299 (Peripheral Vascular Disorders with MCC, relative weight 1.5842), DRG 300 (Peripheral Vascular Disorders with CC, relative weight 1.0464) and DRG 301 (Peripheral Vascular Disorders without CC/MCC, relative weight 0.7133), in MDC 05 (Diseases and Disorders of the Circulatory System), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
More specific portal vein anomaly codes (official index)
- connection Q26.5 — Anomalous portal venous connection
Common questions about portal vein anomaly ICD-10 codes
What is the ICD-10 code for portal vein anomaly?
- Q26.5 — Anomalous portal venous connection. More specific codes apply when the documentation supports them: Q26.5.
What is the ICD-10 code for connection portal vein anomaly?
- Q26.5 — Anomalous portal venous connection.
Is Q26.5 billable?
- Yes. Q26.5 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can Q26.5 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict Q26.5 as a principal diagnosis.
Is Q26.5 a CC or MCC?
- No. Q26.5 is neither a CC nor an MCC under MS-DRG v44.0.