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

The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group Q28 includes congenital malformations of peripheral and central blood vessels and other circulatory anomalies not classified elsewhere, such as anomalies of the pulmonary artery, systemic veins, and unspecified vascular malformations. These ICD-10-CM diagnosis codes are used to document structural vascular anomalies present at birth that may affect circulation, organ perfusion, or require surgical or interventional management. Accurate coding within the ICD-10-CM Q28 group supports proper claim adjudication, complete clinical documentation, and appropriate reimbursement.

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ICD-10-CM Q28: Other Congenital Malformations Of Circulatory System Overview

The ICD-10-CM Q28 diagnoses represent a set of congenital malformations affecting the circulatory system beyond the more common cardiac septal and outflow tract defects. These conditions involve structural abnormalities of blood vessels and related circulatory components and can impact hemodynamics and organ perfusion. Accurate coding matters for complete clinical documentation and appropriate reimbursement for the complexity of care required.

When to Use

Use a code from this group when documenting a congenital vascular anomaly that does not fit into more specific cardiac or vascular categories, such as atypical pulmonary artery or systemic venous malformations. Select the most specific code available to reflect the exact anatomical site and nature of the malformation for accurate clinical records and billing.

ICD-10-CM Codes

6 rows

ICD-10-CM Code	Description
Q28.0	Arteriovenous malformation of precerebral vessels
Q28.1	Other malformations of precerebral vessels
Q28.2	Arteriovenous malformation of cerebral vessels
Q28.3	Other malformations of cerebral vessels
Q28.8	Other specified congenital malformations of circulatory system
Q28.9	Congenital malformation of circulatory system, unspecified

DRG Codes

6 rows

DRG Code	Description
091	Other Disorders of Nervous System with MCC
092	Other Disorders of Nervous System with CC
093	Other Disorders of Nervous System without CC/MCC
299	Peripheral Vascular Disorders with MCC
300	Peripheral Vascular Disorders with CC
301	Peripheral Vascular Disorders without CC/MCC

Typical Clinical Scenarios

An infant is evaluated in the neonatal unit for cyanosis and differential blood pressures between the upper and lower extremities shortly after birth; echocardiography reveals a persistent patent ductus arteriosus with abnormal pulmonary artery branching consistent with congenital subpulmonary stenosis. The neonatologist documents a congenital malformation of the pulmonary artery/arterial tree as the cause of the hemodynamic findings and orders surgical consultation. Typical codes: Q28.1, Q28.2
An adult presents to cardiology clinic with progressive exertional dyspnea and a history of a congenital vascular ring diagnosed in childhood; imaging now shows tracheoesophageal compression from an aberrant subclavian artery and associated chronic respiratory symptoms. The cardiologist records a long-standing congenital vascular anomaly of the aortic arch/major branches as the underlying diagnosis contributing to the patient’s chronic cardiopulmonary symptoms. Typical codes: Q28.3, Q28.8
A child admitted from the emergency department with acute limb ischemia after a previously diagnosed congenital arteriovenous malformation in a limb develops thrombosis and localized ischemic pain; vascular surgery is consulted and the admission note attributes the acute complication to the congenital vascular malformation. Typical codes: Q28.8, Q28.9

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

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

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

6 rows

ICD-10-CM Code	Description
Q28.0	Arteriovenous malformation of precerebral vessels
Q28.1	Other malformations of precerebral vessels
Q28.2	Arteriovenous malformation of cerebral vessels
Q28.3	Other malformations of cerebral vessels
Q28.8	Other specified congenital malformations of circulatory system
Q28.9	Congenital malformation of circulatory system, unspecified

DRG Codes

6 rows

DRG Code	Description
091	Other Disorders of Nervous System with MCC
092	Other Disorders of Nervous System with CC
093	Other Disorders of Nervous System without CC/MCC
299	Peripheral Vascular Disorders with MCC
300	Peripheral Vascular Disorders with CC
301	Peripheral Vascular Disorders without CC/MCC

Typical Clinical Scenarios

An infant is evaluated in the neonatal unit for cyanosis and differential blood pressures between the upper and lower extremities shortly after birth; echocardiography reveals a persistent patent ductus arteriosus with abnormal pulmonary artery branching consistent with congenital subpulmonary stenosis. The neonatologist documents a congenital malformation of the pulmonary artery/arterial tree as the cause of the hemodynamic findings and orders surgical consultation. Typical codes: Q28.1, Q28.2
An adult presents to cardiology clinic with progressive exertional dyspnea and a history of a congenital vascular ring diagnosed in childhood; imaging now shows tracheoesophageal compression from an aberrant subclavian artery and associated chronic respiratory symptoms. The cardiologist records a long-standing congenital vascular anomaly of the aortic arch/major branches as the underlying diagnosis contributing to the patient’s chronic cardiopulmonary symptoms. Typical codes: Q28.3, Q28.8
A child admitted from the emergency department with acute limb ischemia after a previously diagnosed congenital arteriovenous malformation in a limb develops thrombosis and localized ischemic pain; vascular surgery is consulted and the admission note attributes the acute complication to the congenital vascular malformation. Typical codes: Q28.8, Q28.9

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

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

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 Other Congenital Malformations Of Circulatory System: Group Overview

Sign up for ICD-10-CM Code For Other Congenital Malformations Of Circulatory System: Group Overview policy alerts

ICD-10-CM Q28: Other Congenital Malformations Of Circulatory System Overview

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

Sign up for ICD-10-CM Code For Other Congenital Malformations Of Circulatory System: Group Overview policy alerts

ICD-10-CM Q28: Other Congenital Malformations Of Circulatory System 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 Other Congenital Malformations Of Circulatory System: Group Overview

Sign up for ICD-10-CM Code For Other Congenital Malformations Of Circulatory System: Group Overview policy alerts

ICD-10-CM Q28: Other Congenital Malformations Of Circulatory System Overview

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

Sign up for ICD-10-CM Code For Other Congenital Malformations Of Circulatory System: Group Overview policy alerts

ICD-10-CM Q28: Other Congenital Malformations Of Circulatory System Overview

When to Use

ICD-10-CM Codes

DRG Codes

Typical Clinical Scenarios

Related Codes

Which diagnoses are included in the ICD-10-CM Q28 group?

How does accurate coding within `Q28` affect reimbursement and claims?

What types of vascular anomalies are represented by `Q28.0` through `Q28.3`?

When should `Q28.8` or `Q28.9` be used in documentation?

Which related DRGs might be applicable for cases coded with Q28 diagnoses?

References

When to Use

ICD-10-CM Codes

DRG Codes

Typical Clinical Scenarios

Related Codes

Which diagnoses are included in the ICD-10-CM Q28 group?

How does accurate coding within `Q28` affect reimbursement and claims?

What types of vascular anomalies are represented by `Q28.0` through `Q28.3`?

When should `Q28.8` or `Q28.9` be used in documentation?

Which related DRGs might be applicable for cases coded with Q28 diagnoses?

References