Congenital malformations of great veins (Q26)

The Q26 ICD-10 code section covers congenital malformations of great veins, which are structural abnormalities present at birth affecting major veins such as the vena cava and pulmonary veins. These codes help identify specific congenital vein conditions for accurate diagnosis and treatment planning.

This section includes codes like Q26.0, for congenital stenosis of the vena cava; commonly called inferior or superior vena cava stenosis; and Q26.1 for persistent left superior vena cava, an unusual vein connection often documented as “left superior caval vein persisting to coronary sinus.” Codes Q26.2 and Q26.3 identify total and partial anomalous pulmonary venous connections, respectively, which refer to abnormal drainage of pulmonary veins to systemic veins rather than the left atrium. Q26.4 specifies unspecified anomalous pulmonary venous connections, including terms like “Scimitar syndrome” or “pulmonary vein atresia.” Other less common malformations such as anomalous portal venous connections (Q26.5), portal vein-hepatic artery fistula (Q26.6), and various other great vein anomalies are coded under Q26.8 and Q26.9 for unspecified cases. Thus, the ICD-10 code for congenital malformations of great veins guides clinicians and coders in classifying diverse anatomical vein anomalies present from birth.