ICD-10-CM Codes for PAH

Helpful ICD-10-CM codes for patients with PAH

Use the following ICD-10-CM codes to classify diagnoses, symptoms, and procedures performed on patients with PAH.

ICD-10-CM

Description

Hypertension, hypertensive (accelerated) (benign) (essential) (idiopathic) (malignant) (systemic)

126.09

With cor pulmonale (acute)
With right heart ventricular strain/failure (acute)

127.1

Due to kyphoscoliotic disease

127.20

Pulmonary hypertension

127.21

Group 1 (associated) (drug-induced) (toxin-induced)
Primary (idiopathic)
Secondary arterial
Arterial (associated) (drug-induced) (toxin-induced)

127.22

Group 2
Due to left heart disease

127.23

Group 3
Due to lung disease and hypoxia

127.24

Group 4
Chronic thromboembolic

127.29

Group 5
Due to hematologic disorders, metabolic disorders
Secondary specified NEC
With cor pulmonale (chronic)
With right heart ventricular strain/failure
With unclear multifactorial mechanisms

127.83

With right to left shunt related to congenital heart disease

ICD-10-CM=International Classification of Diseases, Tenth Revision, Clinical Modification; NEC=not elsewhere classifiable.

Reference: Centers for Disease Control and Prevention. Accessed March 17, 2025. https://icd10cmtool.cdc.gov/?fy=FY2025&query=pulmonary%20hypertension

Disclaimers:
This document provides an overview of International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) diagnosis codes that have been associated with pulmonary arterial hypertension (PAH) and pulmonary hypertension (PH). This list was developed in consultation with coding experts, providers, and third-party claims data analyses of patients with these conditions. This document is presented for informational purposes only and does not constitute reimbursement or legal advice. As third-party reimbursement is affected by many factors, United Therapeutics Corporation does not promise or guarantee coverage, levels of reimbursement, or payment.
Reimbursement policy through third-party payers is complex and updated frequently; therefore, the information presented in this document may not be current, accurate, or comprehensive. United Therapeutics Corporation strongly recommends consulting each patient’s third-party payer for the most current coding, coverage, and reimbursement policies. In no event shall United Therapeutics Corporation, or their employees or agents, be liable for the damages resulting from or relating to any information provided on this document.