Coding/Billing

Relieve Pulmonary Hypertension

Coding Pressures
2018 ICD-10-CM coding updates expand your options for specificity.
By Artemio Castillejos, CPC, CPMA, CCVTC, CCS, CL6SGB

For 2018, ICD-10-CM Chapter 9: Diseases of the Circulatory System (I00-I99) includes
expanded code choices for pulmonary hypertension. Let’s review what you need to know to
report these conditions appropriately.

Understand Hypertension
Hypertension (high blood pressure) is when the force of the blood against the artery wall is too
high. In ICD-10-CM, hypertension (I10 Essential (primary) hypertension) is synonymous with
arterial, benign, essential, malignant, primary, or systemic hypertension.

Pulmonary hypertension, also called pulmonary arterial hypertension (PAH), refers specifically
to high blood pressure in the arteries that supply the lungs. This occurs when the blood vessels
carrying oxygen-poor blood to your lungs from your heart become hard and narrow. As blood
pressure builds, the heart is forced to work harder to pump blood to the lungs. The heart weakens
over time and can develop heart failure. Primary pulmonary hypertension develops without a
known cause. Secondary pulmonary hypertension develops because of other disease. Secondary
pulmonary hypertension is often associated with congenital heart disorders, liver disease, HIV,
collagen vascular disease, and emphysema and other forms of chronic obstructive pulmonary
disease (COPD).

Note New Codes for Pulmonary Hypertension

ICD-10-CM 2017 had a limited number of pulmonary hypertension codes: one for primary
pulmonary hypertension and another for secondary pulmonary hypertension. ICD-10-CM 2018
expands the code selection for “other secondary pulmonary hypertension” to include the Group I
through Group V classifications (see Hypertension Groupings section). These groups allow you
to classify and select the specific ICD-10-CM code for a patient admitted with pulmonary
hypertension due to an underlying condition.

Hypertension Groupings
The World Health Organization (WHO) classifies pulmonary hypertension within five diagnostic
groups:

Group 1 - Pulmonary Arterial Hypertension: This group includes all causes that lead to structural
narrowing of the pulmonary vessel and PAH. This classification is subdivided into four smaller
groups: idiopathic PAH, heritable, drug and toxin induced, and associated with other systemic
diseases.

Group 2 - Pulmonary Hypertension Due to Left Heart Disease: This group of classification is
characterized by left heart disease, including valve disorder, diastolic dysfunction, and
cardiomyopathy. Specific subgroups include left ventricular systolic dysfunction, left ventricular
diastolic dysfunction, congenital cardiomyopathies, inflow and outflow obstruction of left heart
not due to valvular disease, and valvular heart disease.

Group 3 - Pulmonary Hypertension Due to Lung Disease: This classification includes problems
related to shortage of oxygen in the body (hypoxia). COPD that includes emphysema, chronic
bronchitis, scarring in the lungs, and pulmonary fibrosis caused by lung disease are common
diseases within this classification.

Group 4 - Chronic Thromboembolic Pulmonary Hypertension (CTEPH): This group

classification refers to pulmonary hypertension secondary to blood clot that blocks the flow of
blood through the pulmonary arteries.

Group 5 - Pulmonary Hypertension with Unclear or Multifactorial Mechanism: This group

classification includes rare disorders that do not fit into any of the other four groups. Most of the
diseases in this group include hematologic disorders, systemic disorders with lung involvement,
metabolic disorders, and other disease not classified elsewhere.

Primary pulmonary hypertension — also called heritable PAH, idiopathic PAH, primary group 1
pulmonary hypertension, and primary PAH — is reported using I27.0 Primary pulmonary
hypertension. The physician or other qualified healthcare professional must document primary or
idiopathic hypertension to report this condition.

Note that I27.0 excludes persistent pulmonary hypertension of newborn, pulmonary hypertension
not otherwise specified (NOS), secondary PAH, and secondary pulmonary hypertension. Do not
report these conditions using I27.0.

To report secondary pulmonary hypertension and NOS diagnoses, use the secondary code I27.2
Other secondary pulmonary hypertension. Note that I27.2 instructs you to code also any
associated underlying condition, but does not provide sequencing direction.

As shown in Table A, code I27.2 is expanded to report more detail for other secondary
pulmonary hypertension.

Table A: New ICD-10-CM codes allow you to report pulmonary hypertension due to an
underlying condition.

New Codes Description

I27.20 Pulmonary hypertension, unspecified

Pulmonary hypertension NOS
I27.21 Secondary pulmonary arterial hypertension
(Associated) (drug-induced) (toxin-induced) pulmonary arterial hypertension NOS
(Associated) drug-induced) (toxin-induced) (secondary) group 1 pulmonary
hypertension

I27.22 Pulmonary hypertension due to left heart disease

Group 2 pulmonary hypertension

I27.23 Pulmonary hypertension due to lung diseases and hypoxia

Group 3 pulmonary hypertension

I27.24 Chronic thromboembolic pulmonary hypertension

Group 4 pulmonary hypertension

I27.29 Other secondary pulmonary hypertension

Group 5 pulmonary hypertension

Examples Illustrate Proper Coding

The below scenarios illustrate the impact of code assignment against documentation, and are not
intended to represent any official advice or official sequencing instruction.

Scenario 1:

A 50-year-old male patient presents with congestive heart failure. He has persistent pulmonary
hypertension despite normalization of the LV filling pressure.

I27.22 Pulmonary hypertension due to left heart

• Group 2 pulmonary hypertension

Code also:

• associated left heart disease, if known, such as:

o multiple valve disease (I08.-)
o rheumatic mitral valve disease (I05.-)
o rheumatic aortic valve disease (I06.-)

Scenario 2:

A 63-year-old female patient is seen today for pulmonary hypertension secondary to interstitial
lung disease.

J84.9 Interstitial pulmonary disease, unspecified

• Interstitial pneumonia NOS

I27.23 Pulmonary hypertension due to lung disease and hypoxia

• Group 3 pulmonary hypertension

Code also:

• associated lung disease, if known, such as:

o bronchiectasis (J47.-)
o cystic fibrosis with pulmonary manifestation (E84.0)
o interstitial lung disease (J84.-)
o pleural effusion (J90)
o sleep apnea (G47.3-)

Note that ICD-10-CM guideline I.C.9.a.11 Pulmonary Hypertension provides instruction for
coding primary and secondary pulmonary hypertension and associated conditions, or adverse
effects of drugs or toxins.

For new guidelines on a lower threshold for stage 1 and stage 2 hypertension, read the
accompanying sidebar “Guidelines Make 30 Million More Americans Hypertensive.”

Resources
American Heart Association, Pulmonary Hypertension, May 23, 2017:
www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/TheImpactofCongenitalHeart
Defects/Pulmonary-Hypertension_UCM_307044_Article.jsp#.VqrgL1MrKqA

University of Wisconsin Foundation, Primary and Secondary Hypertension:

www.uwhealth.org/thoracic-surgery/primary-and-secondary-pulmonary-hypertension/11358

National Center for Biotechnology Information, U.S. National Library of Medicine. The WHO
classification of pulmonary hypertension: A case-based imaging compendium:
www.ncbi.nlm.nih.gov/pmc/articles/PMC3342739/

2018 ICD-10-CM Code Set and ICD-10-CM Official Guidelines for Coding and Reporting:
www.cdc.gov/nchs/icd/icd10cm.htm

Bio:

Artemio Castillejos, CPC, CPMA, CCVTC, CCS, CL6SGB, is a healthcare information

management (HIM) professional and an AHIMA World Congress Board Member with more
than four years of extensive experience in outsourced or offshored projects. He is a medical
coding trainer at UST Global Manila Philippines. Castillejos is specialized in cardiology,
cardiothoracic and vascular, evaluation and management (E/M), and surgical coding. He is a
member of the Makati, Philippines, local chapter.

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Code I27.2 is expanded to report more detail for other secondary pulmonary hypertension.