New and Revised ICD-10 Codes for Pulmonary Hypertension in 2018

by | Published on Apr 5, 2018 | Resources, Medical Coding News (A) | 0 comments

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ICD-10 2018 expands the code choices for pulmonary hypertension (PHT). Reporting PHT correctly, as experienced coders in medical coding companies know, depends on having a proper understanding of hypertension and hypertension groupings.

Pulmonary Hypertension

According to the American Heart Association, PHT refers to high blood pressure in the arteries of the lungs, occurring when the vessels of the pulmonary arterial system constrict and their walls thicken. The slowing of the flow of blood through the pulmonary arteries to the lungs causes the pressure in the arteries to rise as the heart works harder to try to pump the blood through. Heart failure occurs when the heart becomes too weak to pump enough blood to the lungs.

Pulmonary hypertension is classified as primary (without an apparent cause) and secondary (due to another condition). Secondary pulmonary hypertension is often associated with congenital hear disorders, liver disease, HIV collagen vascular disease and emphysema, and other forms of chronic constructive pulmonary disease (COPD).

New Pulmonary Hypertension ICD-10 Codes

The 2018 ICD-10 code changes for pulmonary hypertension came into effect on October 1, 2017. In 2018, the code selection for “other secondary pulmonary hypertension” has been expanded to include the group 1 through group 5 classification of PHT. This classification, as provided by the World Health Organization (WHO) is as follows:

Group 1 – Pulmonary arterial hypertension
Group 2 – Pulmonary hypertension due to left heart disease
Group 3 – Pulmonary hypertension due to lung disease
Group 4 – Chronic thromboembolic pulmonary hypertension
Group 5 – Pulmonary hypertension with unclear or multifactorial mechanism

These groups make it possible to classify and select the specific ICD-10 code for pulmonary hypertension due to an underlying condition.
The new pulmonary hypertension ICD-10 codes that came into effect for claim submission starting October 1, 2017 are listed under cardiology diseases as 127 Other pulmonary heart diseases:

I27.0 Primary pulmonary hypertension

  • Primary Group 1 pulmonary arterial hypertension
  • Primary pulmonary hypertension
  • Excludes secondary pulmonary hypertension<//li>

I27.2 Other secondary pulmonary hypertension (Code 127.2 is unchanged but excludes Eisenmenger’s syndrome which will be listed under a new code, I27.83)

    • 127.20 Pulmonary hypertension unspecified
      Pulmonary hypertension NOS


    • 127.21 Secondary pulmonary arterial hypertension
      Drug, toxin-induced pulmonary arterial hypertension NOS
      Drug, toxin-induced secondary Group 1 pulmonary hypertension
      Also code: associated conditions if applicable, or adverse effects of drugs or toxins Adverse effect of appetite depressants (T50.5X5) Congenital heart disease (Q20-Q28) HIV (B20) Portal hypertension (K76.6) Collagen vascular disease (M33.2-, M34.-, M05.-) Schistosomiasis (B65.-)


    • I27.22 Pulmonary hypertension due to left heart disease
      Group 2 Pulmonary hypertension
      New code 127.22 has been added for Group 2 pulmonary hypertension. Associated left heart diseases such as multiple valve diseases (I08.-), rheumatic mitral valve diseases (I05.-), and rheumatic aortic valve diseases (I06.-) should also be coded, if present.


    • I27.23 Pulmonary hypertension due to lung diseases and hypoxia
      Group 3 Pulmonary hypertension
      Code also associated lung disease, if known, such as: Bronchiectasis (J47.-) Cystic fibrosis with pulmonary manifestations (E84.0) Interstitial lung disease (J84.-) Pleural effusion (J90) Sleep apnea (G47.3-).


    • I27.24 Chronic thromboembolic pulmonary hypertension
      Group 4 Pulmonary hypertension
      Code also associated pulmonary embolism, if applicable (I26.-, I27.82)


  • I27.29 Other secondary pulmonary hypertension
    Group 5 pulmonary hypertension
    Pulmonary hypertension with unclear multifactorial mechanisms
    Pulmonary hypertension due to hematologic disorders
    Pulmonary hypertension due to metabolic disorders
    Pulmonary hypertension due to other systemic disorders

Code also other associated disorders, if known, such as: Chronic myeloid leukemia (C92.10-C92.22) Essential thrombocythemia (D47.3) Gaucher disease (E75.22) Hypertensive chronic kidney disease with end stage renal disease (I12.0, I12.11, I13.2) Hyperthyroidism (E05.-) Hypothyroidism (E00-E03) Polycythemiavera (D45) Sarcoidosis (D86.-)

127.8 Other specified pulmonary heart diseases

    • 127.83 Eisenmenger’s syndrome
      Eisenmenger’s complex
      (Irreversible) Eisenmenger’s disease
      Pulmonary hypertension with right to left shunt related to congenital heart disease
      Code also underlying heart defect, if known, such as, Atrialseptal defect (Q21.1) Eisenmenger’s defect (Q21.8) Patent ductusarteriosus (Q25.0) Ventriculatseptal defect (Q21.0)


  • 127.89 Other specified pulmonary heart diseases
    Eisemnmenger’s complex
    Eisenmenger’s syndrome
    Excludes Eisenmenger’s defect (Q21.8)

Medical coding service providers can ensure accurate coding only the physician’s documentation is accurate and describes the patient’s condition to the highest level of specificity. As an ACP Hospitalist report notes, if the documentation does not allow correct code assignment, it will lead to inaccurate capture of the patient’s for quality metrics and reimbursement programs including Medicare’s pay for performance programs.

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