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

The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group Q25 includes congenital anomalies of the great arteries, including abnormalities of the aorta, pulmonary artery, and major arterial branches such as coarctation, transposition, and interruption. These ICD-10-CM diagnosis codes are used to document congenital structural cardiovascular defects, capturing anatomic location, laterality when applicable, and clinical complexity relevant to surgical planning and long-term management. Accurate coding within the ICD-10-CM Q25 group supports proper claim adjudication, complete clinical documentation, and appropriate reimbursement.

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ICD-10-CM Q25: Congenital Malformations Of Great Arteries Overview

This group includes congenital malformations affecting the great arteries such as the aorta and pulmonary artery and their major branches. These conditions target the cardiovascular system and often present from birth with structural abnormalities that can affect circulation and organ perfusion. Accurate coding ensures clinical documentation reflects the anatomic defect and supports appropriate reimbursement for corrective and supportive care.

When to Use

Use a code from this group when a congenital malformation of a great artery is diagnosed and documented, including at birth or on subsequent evaluation. Codes should be selected to reflect the specific anatomic defect, any associated complications, and documented laterality or severity when applicable.

ICD-10-CM Codes

22 rows

ICD-10-CM Code	Description
Q25.0	Patent ductus arteriosus
Q25.1	Coarctation of aorta
Q25.21	Interruption of aortic arch
Q25.29	Other atresia of aorta
Q25.3	Supravalvular aortic stenosis
Q25.40	Congenital malformation of aorta unspecified
Q25.41	Absence and aplasia of aorta
Q25.42	Hypoplasia of aorta
Q25.43	Congenital aneurysm of aorta
Q25.44	Congenital dilation of aorta
Q25.45	Double aortic arch
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
Q25.71	Coarctation of pulmonary artery
Q25.72	Congenital pulmonary arteriovenous malformation
Q25.79	Other congenital malformations of pulmonary artery

Showing 1–20 of 22

DRG Codes

2 rows

DRG Code	Description
306	Cardiac Congenital and Valvular Disorders with MCC
307	Cardiac Congenital and Valvular Disorders without MCC

Typical Clinical Scenarios

A neonate is born with cyanosis and a harsh systolic murmur shortly after delivery; echocardiography demonstrates transposition of the great arteries with the aorta arising from the right ventricle and the pulmonary artery from the left ventricle, requiring urgent prostaglandin infusion and transfer for arterial switch repair. A congenital malformation code is assigned to document the primary structural cause of the newborn’s critical hypoxemia and to guide surgical planning. Typical codes: Q25.0, Q25.1
A school-aged child with a history of repaired ventricular septal defect presents for routine cardiology follow-up and echocardiographic surveillance; imaging now shows subvalvular and valvular pulmonary stenosis with residual right ventricular outflow tract obstruction causing exertional dyspnea. A code from this group is used to capture the chronic congenital obstruction of the pulmonary outflow tract as part of ongoing management and long-term complication monitoring. Typical codes: Q25.4, Q25.3
An adult with known congenital aortic coarctation treated in childhood is admitted for evaluation of uncontrolled systemic hypertension and lower extremity claudication; imaging reveals recoarctation and associated aneurysmal dilation of the aortic segment. A congenital malformation code is assigned to indicate the underlying congenital aortic abnormality contributing to the current complication and to inform vascular intervention planning. Typical codes: Q25.1, Q25.45

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

Q26-CONGENITAL-MALFORMATIONS-GREAT-VEINS

ICD-10-CM Code For Congenital Malformations Of Great Veins: 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.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.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.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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When to Use

ICD-10-CM Codes

22 rows

ICD-10-CM Code	Description
Q25.0	Patent ductus arteriosus
Q25.1	Coarctation of aorta
Q25.21	Interruption of aortic arch
Q25.29	Other atresia of aorta
Q25.3	Supravalvular aortic stenosis
Q25.40	Congenital malformation of aorta unspecified
Q25.41	Absence and aplasia of aorta
Q25.42	Hypoplasia of aorta
Q25.43	Congenital aneurysm of aorta
Q25.44	Congenital dilation of aorta
Q25.45	Double aortic arch
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
Q25.71	Coarctation of pulmonary artery
Q25.72	Congenital pulmonary arteriovenous malformation
Q25.79	Other congenital malformations of pulmonary artery

Showing 1–20 of 22

DRG Codes

2 rows

DRG Code	Description
306	Cardiac Congenital and Valvular Disorders with MCC
307	Cardiac Congenital and Valvular Disorders without MCC

Typical Clinical Scenarios

A neonate is born with cyanosis and a harsh systolic murmur shortly after delivery; echocardiography demonstrates transposition of the great arteries with the aorta arising from the right ventricle and the pulmonary artery from the left ventricle, requiring urgent prostaglandin infusion and transfer for arterial switch repair. A congenital malformation code is assigned to document the primary structural cause of the newborn’s critical hypoxemia and to guide surgical planning. Typical codes: Q25.0, Q25.1
A school-aged child with a history of repaired ventricular septal defect presents for routine cardiology follow-up and echocardiographic surveillance; imaging now shows subvalvular and valvular pulmonary stenosis with residual right ventricular outflow tract obstruction causing exertional dyspnea. A code from this group is used to capture the chronic congenital obstruction of the pulmonary outflow tract as part of ongoing management and long-term complication monitoring. Typical codes: Q25.4, Q25.3
An adult with known congenital aortic coarctation treated in childhood is admitted for evaluation of uncontrolled systemic hypertension and lower extremity claudication; imaging reveals recoarctation and associated aneurysmal dilation of the aortic segment. A congenital malformation code is assigned to indicate the underlying congenital aortic abnormality contributing to the current complication and to inform vascular intervention planning. Typical codes: Q25.1, Q25.45

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

Q26-CONGENITAL-MALFORMATIONS-GREAT-VEINS

ICD-10-CM Code For Congenital Malformations Of Great Veins: 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.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.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.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.

Published by

Trek Health powers OpenPayer with a leading commercial payer intelligence platform, seamlessly converting fragmented payer data into actionable insights for provider organizations.

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

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ICD-10-CM Q25: Congenital Malformations Of Great Arteries Overview

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

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ICD-10-CM Q25: Congenital Malformations Of Great Arteries Overview

When to Use

ICD-10-CM Codes

DRG Codes

Typical Clinical Scenarios

Related Codes

References

When to Use

ICD-10-CM Codes

DRG Codes

Typical Clinical Scenarios

Related Codes

References

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

Sign up for ICD-10-CM Code For Congenital Malformations Of Great Arteries: Group Overview policy alerts

ICD-10-CM Q25: Congenital Malformations Of Great Arteries Overview

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

Sign up for ICD-10-CM Code For Congenital Malformations Of Great Arteries: Group Overview policy alerts

ICD-10-CM Q25: Congenital Malformations Of Great Arteries Overview

When to Use

ICD-10-CM Codes

DRG Codes

Typical Clinical Scenarios

Related Codes

Which ICD-10-CM codes are included in the Q25 group for congenital malformations of great arteries?

How does accurate coding within the Q25 group support reimbursement and claims?

Which DRGs are related to the Q25 diagnosis group?

What types of anatomic defects are captured by the Q25 codes?

When should a coder use `Q25.40` versus a more specific Q25 code?

References

When to Use

ICD-10-CM Codes

DRG Codes

Typical Clinical Scenarios

Related Codes

Which ICD-10-CM codes are included in the Q25 group for congenital malformations of great arteries?

How does accurate coding within the Q25 group support reimbursement and claims?

Which DRGs are related to the Q25 diagnosis group?

What types of anatomic defects are captured by the Q25 codes?

When should a coder use `Q25.40` versus a more specific Q25 code?

References