ICD10 code of Hypoplastic Left Heart Syndrome and ICD9 code

The International Classification of Diseases (ICD) codes for Hypoplastic Left Heart Syndrome (HLHS) are Q23.4 in the ICD-10-CM classification system and 746.7 in the ICD-9-CM system. These codes are utilized globally by healthcare providers and insurance systems to accurately identify and track cases of this complex congenital heart defect.

What is Hypoplastic Left Heart Syndrome?

Hypoplastic Left Heart Syndrome is a severe congenital heart defect characterized by the underdevelopment of the left side of the heart, including the left ventricle, mitral valve, aortic valve, and ascending aorta. Because the heart cannot effectively pump oxygen-rich blood to the rest of the body, Hypoplastic Left Heart Syndrome requires immediate medical intervention shortly after birth. Currently, 28 individuals within the DiseaseMaps community are living with or navigating the care journey associated with Hypoplastic Left Heart Syndrome.

How is Hypoplastic Left Heart Syndrome classified?

In clinical practice, coding is essential for medical records and research. Hypoplastic Left Heart Syndrome is uniquely identified to ensure patients receive appropriate specialized care. The following codes are standard:

• ICD-10-CM Code Q23.4: Congenital mitral stenosis and congenital aortic stenosis (includes Hypoplastic Left Heart Syndrome).


• ICD-9-CM Code 746.7: Hypoplastic left heart syndrome.

Is Hypoplastic Left Heart Syndrome hereditary?

Most cases of Hypoplastic Left Heart Syndrome occur sporadically, meaning there is no clear family history. However, genetic factors may contribute to the risk. Research suggests that parents who have had one child with Hypoplastic Left Heart Syndrome have a slightly increased risk of approximately 2-4% of having another child with the same or a related congenital heart defect.

How is Hypoplastic Left Heart Syndrome managed?

Management of Hypoplastic Left Heart Syndrome involves a staged surgical approach, typically beginning with the Norwood procedure in the first days of life, followed by the Glenn and Fontan procedures. Long-term cardiac follow-up is mandatory for all patients diagnosed with Hypoplastic Left Heart Syndrome to monitor heart function and rhythm.

Next steps

• Consult a pediatric cardiologist or a specialized congenital heart surgeon to discuss your specific care plan.


• Join the DiseaseMaps community to connect with others who have personal experience with Hypoplastic Left Heart Syndrome.


• Maintain copies of all diagnostic reports, including echocardiograms, using the appropriate ICD-10 code (Q23.4) for your medical records.

Medical disclaimer: This information is for educational purposes only and does not constitute medical advice, diagnosis, or treatment; always seek the advice of a qualified healthcare provider with any questions regarding a medical condition.

References

• NIH Genetic and Rare Diseases (GARD) Information Center: Hypoplastic Left Heart Syndrome.


• Orphanet: Rare Disease Database (ORPHA: 2095).


• Centers for Disease Control and Prevention (CDC): Congenital Heart Defects - HLHS.


• OMIM (Online Mendelian Inheritance in Man): Hypoplastic Left Heart Syndrome (Entry #241550).