Pulmonary Hypertension ICD-10-CM Coding

Earlier this year, the pulmonary hypertension community of medical professionals and advocates banded together with ATS to create a marked difference for the world of PH.

New and revised ICD-10-CM codes will become effective for claims submitted with dates of service on or after October 1, 2017. phaware® is pleased to share this important information as it positively affects our entire community.   

Dr. Manaker discusses the new and revised ICD-10-CM codes, which creates a marked difference for the world of PH.

Starting in October, there will be revised ICD-10-CM codes for pulmonary hypertension… The previous PH codes used outdated descriptions of the disease and did not support diagnostic or clinical treatment realities. The ATS led the effort to update the PH code family. While the revised code family is not exactly what we had requested from the ICD Update Committee, it represents a marked improvement over the previous codes.
— ATS October 2017 Issue of Coding & Billing Quarterly

Why is Proper Coding Important?

Nelly Leon-Chisen, RHIA, director of coding and classification at the American Hospital Association says, “Medical coding is there to help present a picture…” And with the breadth of use of the coding system today, improved data will support a host of health care needs including:

  • Measuring the quality, safety, and efficacy of care

  • Designing payment systems and processing claims for reimbursement

  • Conducting research, epidemiological studies, and clinical trials

  • Setting health policy

  • Operational and strategic planning and designing healthcare delivery systems

  • Monitoring resource utilization

  • Improving clinical, financial, and administrative performance

  • Preventing and detecting healthcare fraud and abuse

  • Tracking public health and risks

ICD-10-CM has these intrinsic benefits:

  • Greater level of specificity and clinical detail, and improvements in the capture of advances in medical technology

  • Medical terminology and classification of diseases have been updated to be consistent with current clinical practice

  • Comparability between mortality and morbidity data

Article citation: Fuller, Sandra R.. "Importance of ICD-10" ICD-TEN (May 2009).

New ICD-10-CM Coding Changes

New and revised ICD-10-CM coding effective for claims submitted with dates of service on or after October 1, 2017

I27.0 Primary pulmonary hypertension

Primary Group 1 pulmonary arterial hypertension Primary pulmonary hypertension
Excludes secondary pulmonary hypertension

I27.2 Other secondary pulmonary hypertension

  1. I27.20  Pulmonary hypertension, unspecified

    Pulmonary hypertension NOS

  2. I27.21  Secondary pulmonary arterial hypertension

    Drug, toxin-induced pulmonary arterial hypertension NOS
    Drug, toxin-induced secondary Group 1 pulmonary hypertension

    Code also: associated conditions if applicable, or adverse e ects of drugs or toxins

    Adverse effect of appetite depressants (T50.5X5) Congenital heart disease (Q20-Q28)
    HIV (
    Portal hypertension (

    Collagen vascular disease (M33.2-, M34.-, M05.-)

    Schistosomiasis (B65.-)

  3. I27.22  Pulmonary hypertension due to left heart disease

    Group 2 pulmonary hypertension
    Code also associated left heart disease, if known, such as:

    Multiple valve disease (I08.-) Rheumatic mitral valve diseases (I05.-) Rheumatic aortic valve diseases (I06.-)

  4. 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 (
    Sleep apnea (

  5. 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 (

Hypertensive chronic kidney disease with end stage renal disease (I12.0, I12.11, I13.2) Hyperthyroidism (E05.-)
Hypothyroidism (
Polycythemia vera (

Sarcoidosis (D86.-)
I27.8 Other specified pulmonary heart diseases

I27.83 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:

Atrial septal defect (Q21.1) Eisenmenger’s defect (Q21.8) Patent ductus arteriosus (Q25.0) Ventricular septal defect (Q21.0)

I27.89 Other speci ed pulmonary heart diseases

Eisenmenger’s complex
Eisenmenger’s syndrome
Excludes Eisenmenger’s defect (

phaware® believes in empowering our community. We are pleased to keep you informed. Stay tuned for an ICD-10 centric episode of I’m Aware That I’m Rare: the phaware® podcast regarding these important changes. Subscribe to the podcast here or on iTunes.