June 9, 2022

According to the Cleveland Clinic, “Pulmonary hypertension is a rare lung disorder in which the arteries that carry blood from the heart to the lungs become narrowed, making it difficult for blood to flow through the vessels”.

As a result, the blood pressure in these arteries, called pulmonary arteries, rises far above normal levels. This abnormally high pressure strains the right ventricle of the heart, causing it to expand in size. Overworked and enlarged, the right ventricle gradually becomes weaker and fails to pump enough blood to the lungs. This process can lead to the development of right heart failure.

Pulmonary hypertension occurs in individuals of all ages, races and ethnic backgrounds. Although, it is more common in young adults and is approximately twice as common in women as in men. The symptoms of pulmonary hypertension range in severity, and not every patient has every symptom. Usually, the symptoms do not occur in a patient until the disease has progressed.

Symptoms include:

  • Bluish lips and skin
  • Swelling in the ankles, abdomen, or legs
  • Chest pain
  • Difficulty breathing at rest
  • Passing out or dizziness
  • Irregular heartbeat (palpitations or strong throbbing sensation)
  • Racing pulse
  • Progressive shortness of breath during exercise or activity

There are 5 pulmonary hypertension groups:

  • Stage 1 is the same as secondary pulmonary arterial hypertension: ICD-10-CM code I27.21.
    • Examples: high blood pressure due to congenital heart disease, connective tissue disease, CAD or liver disease.
  • Stage 2 is the same as pulmonary hypertension due to left heart disease: ICD-10-CM code I27.22.
    • Examples: systolic of diastolic heart failure.
  • Stage 3 is the same as pulmonary hypertension due to lung diseases and hypoxia: ICD-10-CM code I27.23.
    • Examples: COPD, Emphysema, interstitial lung disease or pulmonary fibrosis.
  • Stage 4 is the same as chronic thromboembolic pulmonary hypertension: ICD-10-CM code I27.24.
    • Example: due to single or multiple pulmonary embolisms.
  • Stage 5 is other secondary pulmonary hypertension: ICD-10-CM code I27.25.
    • Example: due to other causes documented by the physician, but not listed in stages 1-4.

Historically, coders most often assigned code I27.20 pulmonary hypertension unspecified or NOS due to lack of further specification from the physician. However, we are seeing a new trend in physician documentation where they are starting to document the groups of pulmonary hypertensions. Therefore, when coding pulmonary hypertension always make sure to not default to I27.20 and take the extra time to review the documentation for specificity of the stage.



Alicia R. Blamble, RHIA

Managing Auditor, Excite Health Partners