Shone's Syndrome

What is the appropriate ICD-9-CM diagnosis code for Shone’s syndrome? This syndrome seems to have many of the same characteristics as hypoplastic left heart syndrome. Are Shone’s syndrome and hypoplastic left heart syndrome the same condition with the same code assigned for both conditions? If both conditions are present, what code should be assigned as the principal diagnosis? ...

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Note:  The following article synopsis was NOT provided by the AHA. It was created by Find-A-Code/innoviHealth.

Article Overview

This premium article explains how a specific congenital heart syndrome is discussed in ICD-9-CM coding terms and clarifies its relationship to another congenital heart condition. It is intended for coders and clinical documentation professionals who need help understanding the diagnosis coding context, including which code set is involved and how the topic is framed in relation to principal diagnosis questions.

Why This Topic Matters

Accurate diagnosis coding for rare congenital heart conditions depends on understanding how overlapping conditions are described and differentiated in the coding literature. This article helps readers identify the relevant ICD-9-CM context for reviewing documentation and coding guidance.

What You Will Learn

  • How the article frames Shone's syndrome in ICD-9-CM coding terms
  • How the topic is presented in relation to hypoplastic left heart syndrome
  • What types of diagnosis coding questions the article addresses
  • How principal diagnosis questions are discussed at a high level

Who Should Read This

  • Medical coders
  • Coding auditors
  • Clinical documentation integrity specialists
  • Revenue cycle professionals

Codes Discussed

  • ICD-9-CM: 746.5
  • ICD-9-CM: 746.81
  • ICD-9-CM: 747.10

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