Coding Changes for Pulmonary Hypertension

What is Pulmonary Hypertension?

Pulmonary hypertension is a certain type of high blood pressure that increases the pressure in the pulmonary arteries/lungs and the right side of the heart. Some of the forms of pulmonary hypertension can be serious and over time even fatal. Some forms of pulmonary hypertension are not curable but there are medications that can help lessen the symptoms and improve quality of life. Pulmonary hypertension is different than systemic high blood pressure/hypertension. Pulmonary blood pressure reflects the pressure the heart exerts to pump blood from the heart through the lung arteries. So, this is pressure that is focused on the blood flow in the lungs. Secondary PH is always caused by something else or due to another condition.

Symptoms of Pulmonary Hypertension

Early stages of pulmonary hypertension may have minimal or no symptoms. As the disease progresses, the symptoms become worse.

Common symptoms of Pulmonary Hypertension

  • Dyspnea/Shortness of breath
  • Fatigue
  • Dizziness/syncopal episodes/feeling of being lightheaded
  • Chest pain or pressure
  • Swelling of ankles, legs and eventually the abdomen
  • Cyanosis/bluish color to lips and/or skin
  • Palpitation of the heart or racing pulse
  • Decreased appetite
  • Upper right side abdominal pain

Groups of Pulmonary Hypertension

Pulmonary hypertension is classified into five groups, depending on the cause of the disease.

Group 1: Pulmonary arterial hypertension:

This is the most recognized category of pulmonary hypertension. This type includes both primary and secondary causes. This can be idiopathic (cause unknown) or due to a variety of other conditions/factors. This group can be inherited, drug or toxin induced, caused by connective tissue disease, HIV, liver disease, sickle cell disease, congenital heart disease or by conditions that affect the veins and small blood vessels of the lungs to name a few. There are two ICD-10-CM codes to report for this type of pulmonary hypertension. I27.0 (primary pulmonary hypertension) and I27.21 (secondary pulmonary arterial hypertension).

Group 2: Pulmonary hypertension due to left heart disease:

This category of pulmonary hypertension is caused by failure of the left ventricle or left sided valvular heart disease (mitral and/or aortic valve disease). The most common cause of pulmonary hypertension is left heart disease. ICD-10-CM code I27.22 (pulmonary hypertension due to left heart disease) is reported for this type.

Group 3: Pulmonary hypertension occurring secondary to lung disease and/or hypoxia:

This category of pulmonary hypertension is caused by COPD/emphysema, pulmonary fibrosis, sleep apnea or other sleep disorders and long-term exposure to high altitudes. ICD-10-CM code I27.23 (pulmonary hypertension due to lung disease and hypoxia) is reported for this type.

Group 4: Chronic thromboembolic hypertension:

This category is caused by clotting disorders or blood clots in the lung also called pulmonary emboli. ICD-10-CM code I27.24 (chronic thromboembolic pulmonary hypertension) is reported for this type.

Group 5: Other secondary pulmonary hypertension:

This would include causes such as polycythemia vera, essential thrombocytopenia, sarcoidosis, vasculitis, thyroid or glycogen storage disease, kidney disease, anything that presses on the pulmonary artery (like a tumor) or multifactorial. ICD-10-CM code I27.29 (other secondary pulmonary hypertension) is reported for this type.

Unspecified pulmonary hypertension is reported with ICD-10-CM code I27.20.

Sequencing is based on the circumstances of admission or reason for the encounter. When reporting a code from subcategory I27.2, be sure and report the associated conditions or the adverse effect of the drug/toxin.

As stated above, there is no cure for the disease pulmonary hypertension. But medication/treatment can lessen the symptoms and improve quality of life.

Here are a few lifestyle changes that can improve the symptoms:

  • Discuss with your MD before taking over the counter medications
  • Quit smoking or do not start to smoke
  • Follow a healthy diet-low in fat, cholesterol, sodium and sugar
  • Record your weight. If there is a rapid weight gain it may be a sign of worsening
  • Stay active
  • Avoid the hot tub/sauna

Authored by Kim Boy, RHIT, CDIP, CCS, CCS-P

ICD-10-CM Index
AHA Coding Clinic, Fourth Quarter 2017 Pages: 14-15

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The information contained in this coding advice is valid at the time of posting. Viewers are encouraged to research subsequent official guidance in the areas associated with the topic as they can change rapidly.

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