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Learn what separates the pulmonary hypertension codes.

Pulmonary hypertension (PH) can be defined as high blood pressure in the pulmonary arteries, which carry oxygen-rich blood from the heart to the lungs. Early symptoms are shortness of breath, lung disease, and hypoxia. Early diagnosis and treatment can lead to a better quality of life.

Refresh your knowledge of this pulmonology condition and learn how to assign the correct ICD-10-CM codes.

Get to Know Pulmonary Hypertension

PH has many different causes and is usually a complication of heart or lung disease. There are many other diseases and factors that increase a patient鈥檚 risk for PH. High blood pressure within the pulmonary arteries will cause the pulmonary arteries to narrow and results in the patient鈥檚 heart working harder to pump blood to the lungs. PH can be fatal when left untreated.

PH can cause other conditions to develop, including:

• Anemia
• Arrhythmias
• Blood clots or pulmonary embolism
• Pericardial effusion
• Pregnancy-related complications to both mother and fetus

Define the Types of Pulmonary Hypertension

PH can be divided into the following five groups based on the cause as defined by the World Health Organization (WHO):

• PH due to pulmonary arterial hypertension (PAH)
• PH due to left-sided heart disease
• PH due to lung disease or hypoxia
• PH due to blockages in the lungs
• PH due to other disorders

PH affects people of any age, but is more common among patients with heart and lung conditions such as mitral valve disease, aortic valve disease, scleroderma, sickle cell disease, and people with HIV. When PH occurs in infants, it鈥檚 called persistent pulmonary hypertension of the newborn (PPHN).

The exact cause of PH is unknown, but it affects an estimated 50-100 people per million, according to the Centers for Disease Control and Prevention (CDC). PH is most common among women ages 30-60 years old. PH is also harder to diagnosis than other conditions, such as congestive heart failure (CHF) or chronic obstructive pulmonary disease (COPD), and is sometimes missed until it progresses into the condition鈥檚 late stages. The sooner it鈥檚 diagnosed, the better the outcome for the patient.

Know What Tests Pulmonologists Perform for Pulmonary Hypertension Evaluation

Providers use multiple testing options to assist in the diagnosis and treatment of pulmonary hypertension.

Tests performed to measure the blood pressure in the pulmonary arteries include:

• Right heart catheterization (RHC)
• Doppler echocardiogram

Furthermore, physicians use the following tests to look for an underlying cause of PH:

• Blood tests
• Chest X-ray
• Chest CT scan
• Polysomnogram (PSG)
• Pulmonary ventilation/perfusion (VQ) scan
• Six-minute walk test

Treatment is based on the type of PH and the other medical conditions the patient has. There are only two types of PH that can be directly treated: pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). In other types of PH, providers treat the underlying conditions, including calcium channel blockers for high blood pressure throughout the body; diuretics are used to clear fluid out of the body; oxygen therapy is used for patients who don鈥檛 have enough oxygen in the blood; and pulmonary vasodilators relax the pulmonary arteries and open them up to improve the blood flow and lower strain on the heart.

CTEPH treatments can include anticoagulants to help prevent blood clots; balloon atrial septostomy (BAS) for babies with critical heart defects; balloon pulmonary angioplasty (BPA) to widen the pulmonary artery; medications, such as a soluble guanylate cyclase stimulator (SGCS), that can slow disease progression; and pulmonary endarterectomy (PEA), which is a surgery that removes blood clots from your lungs and is possibly the only cure for CTEPH.

Other options for PH caused by heart or lung problems will focus on managing the underlying conditions. These options include dietary changes, lifestyle changes, medication for hypertension or heart failure, oxygen therapy, and surgery for heart valve repair.

The outlook for patients with pulmonary hypertension depends on the type of PH, when it was diagnosed, the severity, and other associated medical conditions.

Evaluate Pulmonary Hypertension Diagnosis Codes

Pulmonary arterial hypertension (PAH) is the most recognized type of PH and the ICD-10-CM code book features two codes that can be reported:

• I27.0 (Primary pulmonary hypertension)
• I27.21 (Secondary pulmonary arterial hypertension)

PH due to left heart disease is caused by the failure of the left ventricle/left-sided valvular heart disease, which is also known as mitral or aortic valve disease. The condition is reported with:

• I27.22 (Pulmonary hypertension due to left heart disease)

PH occurring secondary to lung disease and hypoxia is caused by COPD/emphysema, pulmonary fibrosis, sleep apnea, and other sleep disorders as well as long-term exposure to high altitudes. You鈥檒l report the diagnosis with:

• I27.23 (Pulmonary hypertension due to lung disease and hypoxia)

CTEPH is caused by a clotting disorder or blood clots found within the lungs. The latter are also called pulmonary emboli. Assign the following code to report a CTEPH diagnosis:

• I27.24 (Chronic thromboembolic pulmonary hypertension)

The last type is secondary pulmonary hypertension, which includes causes like chronic polycythemia vera, essential thrombocytopenia, sarcoidosis, vasculitis, thyroid or glycogen storage disease, kidney disease, or things that press against the pulmonary arteries, such as a tumor. You鈥檒l also use this diagnosis when the symptoms are multifactorial. It is reported with:

• I27.29 (Other secondary pulmonary hypertension)

The ICD-10-CM official guidelines, section I.C.9.a.11 state that the condition is designated to category I27.- (Other pulmonary heart diseases). Secondary pulmonary hypertension codes I27.1 and I27.2- have Code also notes for any associated conditions or adverse effects of drugs or toxins. 鈥淭he sequencing is based on the reason for the encounter, except with adverse effects of drugs,鈥� according to the guidelines. The default code for primary pulmonary hypertension NOS is I27.20.

Cristin Robinson, CPC, CPMA, CCC, CRC, CEMC, Education Coding Consultant, Bristol, Tennessee