Pulmonary hypertension (PH) refers to high blood pressure in the blood vessels leading from the heart to the lungs (pulmonary arteries). When the blood pressure in the pulmonary arteries gets too high, the arteries in the lungs narrow (or constrict), reducing blood flow through the lungs and causing low levels of oxygen in the blood. Regarded as a serious condition, PH often affects the right side of the heart and causes symptoms such as shortness of breath, chest pain, and lightheadedness. If left untreated, the increased blood pressure levels can damage your heart and may often lead to serious or life-threatening complications, such as heart failure or arrhythmias, which are irregular heart rhythms. Cardiologists and other clinicians need to know the specific medical codes to report the diagnosis of PH and the treatment services they provide for it, as well as the payer reimbursement guidelines for this condition. Billing and coding tasks for pulmonary hypertension can become much easier with the support of a reliable and experienced medical billing company.
Pulmonary hypertension can occur among people of all age groups and its incidence increases with age. However, this condition happens to be more common among women in the age group of 35- 60 years. Several factors can increase the risks of suffering this condition and these include a family history of the condition, obesity, use of illegal drugs like cocaine, certain appetite-suppressant or diet medications, and living at high altitudes. The changes in the pulmonary arteries that lead to PH can be caused by several factors such as lung diseases or a shortage of oxygen in the body (hypoxia), problems with the smaller branches of the pulmonary arteries (PAH), conditions that affect the left side of the heart, and blood clots that cause narrowing or a blockage in the pulmonary arteries.
Types of Pulmonary Hypertension (PH)
The World Health Organization (WHO) has classified PH into five different groups according to its causes or associated underlying conditions –
- Group 1 – Pulmonary Arterial Hypertension (PAH) – PAH refers to high pressure in the vessels caused by obstruction in the small arteries in the lungs, for a variety of reasons. These include “idiopathic” (no identifiable cause or underlying contributing condition); drug-related, HIV infection; connective tissue/autoimmune disorders (such as scleroderma, lupus) and others.
- Group 2 – Pulmonary hypertension due to left-sided heart disease (left heart failure, valve disease) – This type is primarily caused by left-sided valvular heart disease, such as mitral valve or aortic valve disease and failure of the lower left heart chamber (left ventricle).
- Group 3 – Pulmonary hypertension caused by lung diseases – This type occurs due to lung diseases or hypoxemia (low blood oxygen conditions) such as emphysema, pulmonary fibrosis/interstitial lung disease; obstructive sleep apnea; chronic high altitude exposure; and other reasons.
- Group 4 – Chronic thromboembolic pulmonary hypertension (CTEPH) – This type is caused by chronic blood clots in the lungs that block the normal blood flow.
- Group 5 – Pulmonary hypertension from numerous other potential metabolic, systemic, or hematologic disorders like glycogen storage disease, sarcoidosis, blood disorders and tumors pressing against the pulmonary arteries.
Common Symptoms of PH
The signs and symptoms may be common across all types of PH. The symptoms during the early stages may not be noticeable for months or even years. However, as the disease progresses, symptoms become worse. Common signs and symptoms include –
- Shortness of breath (dyspnea), initially while exercising and eventually while at rest
- Swelling (edema) in the ankles, legs and eventually in the abdomen (ascites)
- Racing pulse or heart palpitations
- Fatigue
- Dizziness or fainting spells (syncope)
- Decreased appetite
- Chest pressure or pain
- Bluish color to your lips and skin (cyanosis)
- Dry cough, sometimes with blood
Diagnosing and Treating Pulmonary Hypertension (PH)
Early diagnosis of pulmonary hypertension (PH) is often difficult as it is not possible to detect it in a routine physical examination. Even when the condition rises to an advanced stage, its signs and symptoms are similar to those of other heart and lung conditions. Diagnosis of this condition may generally begin with a detailed review of the patient’s medical and family history, evaluation of signs and symptoms and physical examination.
Physicians order several tests to diagnose the exact causes of PH and determine its severity and specific stage. Diagnostic tests include Echocardiogram, Electrocardiogram (ECG), Chest X-ray, right heart catheterization and blood tests (to check for certain substances in your blood that might show you have PH or its complications). In some cases, physicians may also order additional tests like – Computerized tomography (CT) scan, Magnetic resonance imaging (MRI), Polysomnogram, Ventilation/perfusion (V/Q) scan, Pulmonary function test, Open-lung biopsy and genetic tests to check the condition of the lungs and pulmonary arteries.
There is no exact treatment for pulmonary hypertension (PH), but administering certain treatment modalities can help patients improve their symptoms and slow the progress of the disease. The treatment modalities are often complex and require extensive follow-up care. Common treatment options include medications such as Blood vessel dilators (vasodilators), Endothelin receptor antagonists, High-dose calcium channel blockers, soluble guanylate cyclase (SGC) stimulator, Anticoagulants and Diuretics. If medications don’t control the pulmonary hypertension, surgery would be considered as a last option. Surgical options include Atrial septostomy (open-heart surgery to create an opening between the upper left and right chambers of the heart (atria) to relieve the pressure on the right side of the heart) and lung or heart-lung transplantation.
Precise documentation is required for correct code assignment on the medical claims. To assign the correct diagnosis codes, medical coders need to carefully examine the medical reports to determine what specific diagnostic procedures the physicians have performed. Medical billing services offered by reliable providers ensure that the correct ICD-10 codes such as the following are used for PH medical billing purposes –
ICD-10 Codes for Pulmonary Hypertension (PH)
I27 – Other pulmonary heart diseases
- I27.0 – Primary pulmonary hypertension
- I27.1 – Kyphoscoliotic heart disease
- I27.2 – Other secondary pulmonary hypertension
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- I27.20 – Pulmonary hypertension, unspecified
- I27.21 – Secondary pulmonary arterial hypertension
- I27.22 – Pulmonary hypertension due to left heart disease
- I27.23 – Pulmonary hypertension due to lung diseases and hypoxia
- I27.24 – Chronic thromboembolic pulmonary hypertension
- I27.29 – Other secondary pulmonary hypertension
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I27.8 – Other specified pulmonary heart diseases
- I27.81- Cor pulmonale (chronic)
- I27.82 – Chronic pulmonary embolism
- I27.83 – Eisenmenger’s syndrome
- I27.89 – Other specified pulmonary heart diseases
I27.9 – Pulmonary heart disease, unspecified
Even though, medical treatment can’t fully cure PH, it can help lessen the symptoms. Incorporating certain key lifestyle changes like taking plenty of rest, quitting the habit of smoking, avoiding situations that can excessively lower blood pressure, consuming a nutritious diet and staying at a healthy body weight can help improve the heart condition in the long run.
Pulmonary medical billing and coding can be problematic. For accurate and timely medical billing and claims submission, healthcare practices can outsource their medical coding tasks to a reliable and professional medical billing company that provides the services of AAPC-certified coding specialists.