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    v1.0.28
    OpenPayerBilling CodesCongenital malformations of the circulatory systemICD-10-CM q26-congenital-malformations-great-veins
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    ICD-10-CM Code For Congenital Malformations Of Great Veins: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group Q26 includes congenital malformations of the great veins such as atresia, hypoplasia, duplication, or abnormal connections of the superior and inferior vena cava and major tributaries. These ICD-10-CM diagnosis codes are used to document congenital venous anomalies that affect venous return, may require surgical or interventional management, and capture anatomic detail and clinical complexity. Accurate coding within the ICD-10-CM Q26 group supports proper claim adjudication, complete clinical documentation, and appropriate reimbursement.

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    ICD-10-CM Q26: Congenital Malformations Of Great Veins Overview

    This code group represents congenital malformations affecting the major systemic veins, including anomalies of the superior vena cava, inferior vena cava, and their major tributaries. These conditions target the circulatory system and often present in neonatal or pediatric settings but may be identified later in life. Accurate coding matters for appropriate clinical documentation and reimbursement because it captures the anatomic abnormality and potential complexity of care required.

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    When to Use

    Use codes from this group when a congenital malformation of a great vein is documented by clinical evaluation, imaging, or operative findings. Codes are selected to reflect the specific anatomic site and nature of the venous anomaly to capture severity and guide care documentation.

    ICD-10-CM Codes

    9 rows

    ICD-10-CM CodeDescription
    Q26.0Congenital stenosis of vena cava
    Q26.1Persistent left superior vena cava
    Q26.2Total anomalous pulmonary venous connection
    Q26.3Partial anomalous pulmonary venous connection
    Q26.4Anomalous pulmonary venous connection, unspecified
    Q26.5Anomalous portal venous connection
    Q26.6Portal vein-hepatic artery fistula
    Q26.8Other congenital malformations of great veins
    Q26.9Congenital malformation of great vein, unspecified

    DRG Codes

    5 rows

    DRG CodeDescription
    299Peripheral Vascular Disorders with MCC
    300Peripheral Vascular Disorders with CC
    301Peripheral Vascular Disorders without CC/MCC
    306Cardiac Congenital and Valvular Disorders with MCC
    307Cardiac Congenital and Valvular Disorders without MCC

    Typical Clinical Scenarios

    • A newborn in the neonatal intensive care unit with cyanosis and a continuous murmur is found on echocardiography to have an abnormal connection between the pulmonary veins and the systemic venous circulation causing right-to-left shunting and hypoxemia; the infant was admitted emergently for oxygen support and surgical planning, and a congenital malformation of the great veins is assigned to describe the anomalous pulmonary venous drainage. Typical codes: Q26.2, Q26.3
    • A young adult presents to cardiology for evaluation of longstanding exertional dyspnea and recurrent respiratory infections; imaging and cardiac catheterization identify a partial anomalous pulmonary venous return with an atrial-level shunt causing right heart enlargement, a chronic congenital venous malformation that is being monitored and considered for elective repair. Typical codes: Q26.3, Q26.4
    • A patient with known congenital malformation of systemic venous return develops atrial arrhythmia and congestive heart failure; during hospitalization for decompensated heart failure, the congenital venous anomaly is documented as a comorbidity contributing to volume overload and guiding management decisions. Typical codes: Q26.1, Q26.6

    Related Codes

    Q20-CONGENITAL-CARDIAC-CHAMBERS-AND-CONNECTIONS

    ICD-10-CM Code For Congenital Malformations Of Cardiac Chambers And Connections: Group Overview

    Q21-CONGENITAL-CARDIAC-SEPTAL-DEFECTS

    ICD-10-CM Code For Congenital Malformations Of Cardiac Septa: Group Overview

    Q22-CONGENITAL-PULMONARY-TRICUSPID-VALVE

    ICD-10-CM Code For Congenital Malformations Of Pulmonary And Tricuspid Valves: Group Overview

    Q23-CONGENITAL-AORTIC-AND-MITRAL-VALVE-MALFORMATIONS

    ICD-10-CM Code For Congenital Malformations Of Aortic And Mitral Valves: Group Overview

    Q24-OTHER-CONGENITAL-MALFORMATIONS-OF-HEART

    ICD-10-CM Code For Other Congenital Malformations Of Heart: Group Overview

    Q25-CONGENITAL-MALFORMATIONS-OF-GREAT-ARTERIES

    ICD-10-CM Code For Congenital Malformations Of Great Arteries: Group Overview

    Q27-OTHER-CONGENITAL-PERIPHERAL-VASCULAR-MALFORMATIONS

    ICD-10-CM Code For Other Congenital Malformations Of Peripheral Vascular System: Group Overview

    Q28-OTHER-CONGENITAL-CIRCULATORY-MALFORMATIONS

    ICD-10-CM Code For Other Congenital Malformations Of Circulatory System: Group Overview

    Frequently Asked Questions

    References

    1. 1.CPT® Codes and Descriptions. AAPC. Retrieved Aug 11, 2026.Maintains the CPT code set's official descriptions and guidelines, licensed from the American Medical Association, used as the definitional source for CPT codes on this page.
    2. 2.HCPCS Quarterly Update. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's quarterly release of HCPCS Level II codes and short/long descriptors, the source for HCPCS code definitions and update history.
    3. 3.ICD-10-CM Full Code List. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's complete ICD-10-CM diagnosis code list, used to cross-reference diagnosis codes associated with this procedure or service.
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