June 30, 2026

Fontan-Associated Conditions: ICD-10-CM Coding and Documentation Guidance

Overview of the Fontan Procedure and Fontan Physiology

Reporting Fontan-Associated Conditions


The Fontan procedure (Fontan circulation) is performed in pediatric patients with single-ventricle congenital heart defects such as Tricuspid atresia, Hypoplastic left heart syndrome, and Double outlet right ventricle.


The altered “Fontan physiology” (chronic venous hypertension and low cardiac output) can result in long-term systemic complications affecting the liver, lymphatic system, and overall circulation.


ICD-10-CM Codes for Fontan-Associated Conditions

ICD-10-CM Codes for Fontan-Associated Conditions


Use category I27.84- to report complications specific to Fontan physiology:

  • I27.840 – Fontan-associated liver disease (FALD)
  • I27.841 – Fontan-associated lymphatic dysfunction
  • I27.848 – Other Fontan-associated condition
  • I27.849 – Fontan-related circulation, unspecified


Coding Guidance for Fontan-Associated Conditions

Coding Guidance

Code the specific Fontan-associated complication when documented.

• I27.840 (FALD)
 FALD may include:
 o Liver fibrosis or cirrhosis
 o Regenerative arterialized liver nodules
 o Hepatocellular carcinoma
 Code additional diagnoses (e.g., cirrhosis, hepatocellular carcinoma) as applicable.

• I27.841 (Lymphatic dysfunction)
 Manifestations may include:
 o Plastic bronchitis
 o Protein-losing enteropathy
 Report additional codes for the specific manifestations when documented.

• I27.848 Other Fontan-associated condition
 Examples may include:
 o Arrhythmias
 o Ventricular dysfunction
 o Organ fibrosis

• I27.849 Fontan related circulation, unspecified
 Use when documentation states “Fontan-related circulation” without further detail.




Lori Amende, RHIA, CCS, Education and Quality Manager at UASI

Lori Amende, RHIA, CCS 

Education and Quality Manager at UASI


Works Cited

American Hospital Association. (2025). Fontan-associated conditions coding guidance. AHA Coding Clinic for ICD-10-CM/PCS, Fourth Quarter 2025, p. 19.


Children’s Hospital of Philadelphia. (n.d.). Hypoplastic left heart syndrome (HLHS). Available at: https://www.chop.edu/conditions-diseases/hypoplastic-left-heart-syndrome-hlhs


Leeds Teaching Hospitals NHS Trust. (n.d.). Fontan circulation. Available at: https://www.leedsth.nhs.uk/patients/resources/fontan-circulation/


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