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Distinguish repaired defects from residual lesions and late complications.

Advances in congenital heart disease treatment mean more patients are reaching adulthood with conditions that once limited life expectancy. Today, adults with congenital heart disease (ACHD) may receive routine cardiology care decades after childhood surgery, return for additional procedures, or develop complications related to their original defect or its repair.

For coders, these encounters can create a challenge: Should you report the original congenital defect, a personal history code, or a code for a current complication?

The answer depends on what remains clinically present: A repaired defect does not automatically mean you should continue reporting its original congenital diagnosis. However, a patient who has undergone partial repair or still has a residual defect may need a congenital diagnosis code throughout adulthood. Here’s the first part of two for navigating ICD-10-CM guidelines to code congenital heart defects.

Determine Whether the Congenital Defect Is Still Present

Start by reviewing the cardiologist’s assessment, surgical history, imaging findings, and current treatment plan. Do not assume that a congenital heart defect has been completely corrected just because the patient underwent surgery as a child.

The ICD-10-CM Official Guidelines, Chapter 17, provide two important instructions:

• Codes from Q00-QA1 (Congenital malformations, deformations, chromosomal abnormalities, and genetic disorders) may be used throughout the patient’s life when the congenital malformation remains present.
• When a congenital malformation has been corrected, use a personal history code to identify the history of the condition.

For cardiac conditions, Z87.74 (Personal history of (corrected) congenital malformations of heart and circulatory system) is the applicable history code. AAPC’s Codify listing for Z87.74 also includes an important Excludes2 note: Congenital malformations that have been partially corrected or repaired but still require medical treatment should be coded to the condition.

This means you need to distinguish three situations:

• The congenital malformation remains present. Assign the appropriate Q code when the condition meets the reporting criteria for the encounter. Active treatment is not a prerequisite for using a Q code when the malformation remains present.

• The defect has been partially repaired, but a residual congenital malformation remains and requires treatment. Continue to code the condition rather than substituting Z87.74.
• The defect has been fully corrected. Use Z87.74 to identify the history of the corrected congenital condition when relevant to the encounter.

Do not use a patient’s age or the number of years since surgery to determine whether the congenital condition remains present. The provider’s documentation and current clinical findings should guide your code selection.

Identify the Specific Congenital Heart Defect

ICD-10-CM classifies congenital heart defects primarily within categories Q20-Q28, Congenital malformations of the circulatory system.

Some commonly encountered codes include the following:

• Q20.0 (Common arterial trunk)
• Q20.3 (Discordant ventriculoarterial connection)
• Q21.0 (Ventricular septal defect)
• Q21.3 (Tetralogy of Fallot)
• Q21.20 (Atrioventricular septal defect, unspecified as to partial or complete)
• Q22.5 (Ebstein’s anomaly)
• Q23.0 (Congenital stenosis of aortic valve)
• Q23.4 (Hypoplastic left heart syndrome)
• Q25.0 (Patent ductus arteriosus)
• Q25.1 (Coarctation of aorta)

Also distinguish congenital defects from acquired conditions. For example, Q21 (Congenital malformations of cardiac septa) includes an Excludes1 note for I51.0 (Cardiac septal defect, acquired). A septal defect that develops after a myocardial infarction is not the same diagnosis as a congenital ventricular or atrial septal defect.

Consider Your Coding Approach

Example: A 38-year-old patient underwent repair of tetralogy of Fallot (TOF) during childhood. The patient now has pulmonary regurgitation and right ventricular enlargement. The adult congenital cardiologist documents persistent hemodynamic abnormalities related to the congenital condition and recommends additional imaging and evaluation for pulmonary valve intervention.

TOF is a complex congenital heart defect involving a ventricular septal defect, pulmonary stenosis or atresia, an overriding aorta, and right ventricular hypertrophy. ICD-10-CM provides Q21.3 (Tetralogy of Fallot) to identify the condition.

If the provider documents that the original congenital defect remains partially corrected and requires ongoing treatment, report Q21.3. Also report separately documented manifestations that are not inherent components of the congenital diagnosis, such as pulmonary regurgitation, when appropriate.

If the original TOF has been fully corrected and no longer exists as a current malformation, do not automatically continue reporting Q21.3 solely because the patient has a history of TOF. Consider Z87.74 and the specific current residual condition or manifestation based on the provider’s documentation.

This distinction matters because the original congenital defect and a late hemodynamic consequence are not necessarily the same diagnosis. The provider’s documentation must establish whether a congenital malformation remains present or whether the current finding is a separate late manifestation after correction.

Check back next month for more tips on coding adult congenital heart defects.

Suzanne Burmeister, BA, MPhil, Medical Writer and Editor