What is Pulmonary Hypertension?
Pulmonary hypertension, defined as a mean pulmonary arterial pressure greater than 25 mm Hg at rest or greater than 30 mm Hg during exercise, is often characterized by a progressive and sustained increase in pulmonary vascular resistance that eventually may lead to right ventricular failure.
Types of Pulmonary Hypertension
Pulmonary arterial hypertension (PAH):
This type of PH is caused by the changes in the walls of the small arteries of the lungs.
Pulmonary venous hypertension (PVH):
This type of PH is caused by problems related to the left side of the heart such as heart valve disease, congestive heart failure and cardiomyopathy.
Other conditions that contribute to the development of PH
- Aortic valve disease
- Chronic obstructive pulmonary disease “COPD”: a group of lung diseases that block airflow and make it difficult to breathe.
- Congenital heart disease
- Liver cirrhosis: a disease that occurs when healthy cells in the liver are damaged and replaced by scar tissue, usually as a result of alcohol abuse or chronic hepatitis.
- Autoimmune disease: a condition in which your immune system mistakenly attacks your body (e.g. lupus, rheumatoid arthritis and scleroderma).
- Mitral valve disease
- Pulmonary fibrosis: a type of lung disease that occurs when lung tissue becomes damaged and scarred.
- Sickle cell disease: a condition in which there aren't enough healthy red blood cells to carry adequate oxygen throughout your body.
- Obstructive sleep apnea: a condition in which your breathing abruptly stops and starts while sleeping.
Symptoms of Pulmonary Hypertension
The signs and symptoms of pulmonary hypertension in its early stages might not be noticeable for months or even years. As the disease progresses, symptoms become worse and begin to show. Various symptoms include:
- Abdominal bloating
- Shortness of breath during routine activity
- Heart palpitations: when you feel like your heart is racing, pounding or fluttering.
- Heart arrhythmias
- Chest pain
- Decreased appetite
- Pain in your right side of the abdomen
- Rapid heart rate (tachycardia) of more than 100 beats per minute.
- Swelling in your ankles, legs and abdomen
- Bluish lips or skin (cyanosis)
Classification of Pulmonary Hypertension
The cause of pulmonary hypertension is classified by the World Health Organization into five groups.
Group 1- Pulmonary arterial hypertension: This grouping is caused by:
- Certain drugs
- Conditions that affect veins and small blood vessels of the lungs.
- Congenital heart disease
- Autoimmune disease (e.g. lupus, rheumatoid arthritis and scleroderma) is a condition in which your immune system mistakenly attacks your body.
- Genetic tests
- HIV infection
- Liver disease
- Sickle cell disease
- Unknown cause
Group 2- Pulmonary hypertension caused by left-sided heart disease: This grouping is caused by:
- Aortic valve disease
- Congestive heart failure
- Mitral valve disease
Group 3- Pulmonary hypertension caused by lung disease: This grouping is caused by:
- Chronic obstructive pulmonary disease (COPD)
- Interstitial lung disease
- Long-term exposure to high altitudes
- Sleep apnea and other sleep disorders
Group 4- Pulmonary hypertension caused by chronic blood clots: This grouping is caused by:
- Chronic blood clots in the lungs or general clotting disorders.
Group 5- Pulmonary hypertension associated with other conditions that have unclear reasons why the pulmonary hypertension occurs: This grouping is caused by:
- Blood disorders such as polycythemia vera and essential thrombocythemia.
- Metabolic disorders such as thyroid and glycogen storage diseases.
- Systemic disorders such as sarcoidosis and vasculitis.
- Tumors pressing against pulmonary arteries.
Patients with pulmonary hypertension are normally classified into 4 symptom-based (functional) classes also described by the World Health Organization.
- Class I: Patients in this category show no limitation of physical activity. Ordinary physical activity does not cause fatigue, palpitation or shortness of breath.
- Class II: Patients in this category show slight limitation of physical activity. No symptoms at rest.
- Class III: Patients in this category show great limitation of physical activity. No symptoms at rest.
- Class IV: Patients in this category are unable to carry on any physical activity without discomfort. There are symptoms at rest.
Risk Adjustment / HCC Coding FAQs
If both pulmonary hypertension and heart failure are coded, will both diagnoses be added to the patient’s risk score?
No, both diagnoses map to HCC 85. Each
HCC category is only added to the risk score once.
Community Non-Dual, Aged
Community FB Dual, Aged
There are a total of 61 ICD-10 codes included in HCC 85. See the complete list below.
A36.81 Diphtheritic cardiomyopathy
B33.24 Viral cardiomyopathy
I09.81 Rheumatic heart failure
I11.0 Hypertensive heart disease with heart failure
I13.0 Hypertensive heart and CKD with HF and stage 1 through 4 CKD
I13.2 Hypertensive heart and CKD with HF and with stage 5 CKD, or ESRD
I26.01 Septic pulmonary embolism with acute cor pulmonale
I26.02 Saddle embolus of pulmonary artery with acute cor pulmonale
I26.09 Other pulmonary embolism with acute cor pulmonale
I27.0 Primary pulmonary hypertension
I27.1 Kyphoscoliotic heart disease
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
I27.81 Cor pulmonale (chronic)
I27.83 Eisenmenger's syndrome
I27.89 Other specified pulmonary heart diseases
I27.9 Pulmonary heart disease, unspecified
I28.0 Arteriovenous fistula of pulmonary vessels
I28.1 Aneurysm of pulmonary artery
I28.8 Other diseases of pulmonary vessels
I28.9 Disease of pulmonary vessels, unspecified
I42.0 Dilated cardiomyopathy
I42.1 Obstructive hypertrophic cardiomyopathy
I42.2 Other hypertrophic cardiomyopathy
I42.3 Endomyocardial (eosinophilic) disease
I42.4 Endocardial fibroelastosis
I42.5 Other restrictive cardiomyopathy
I42.6 Alcoholic cardiomyopathy
I42.7 Cardiomyopathy due to drug and external agent
I42.8 Other cardiomyopathies
I42.9 Cardiomyopathy, unspecified
I43 Cardiomyopathy in diseases classified elsewhere
I50.1 Left ventricular failure, unspecified
I50.20 Unspecified systolic (congestive) heart failure
I50.21 Acute systolic (congestive) heart failure
I50.22 Chronic systolic (congestive) heart failure
I50.23 Acute on chronic systolic (congestive) heart failure
I50.30 Unspecified diastolic (congestive) heart failure
I50.31 Acute diastolic (congestive) heart failure
I50.32 Chronic diastolic (congestive) heart failure
I50.33 Acute on chronic diastolic (congestive) heart failure
I50.40 Unspecified combined systolic (congestive) and diastolic (congestive) heart failure
I50.41 Acute combined systolic (congestive) and diastolic (congestive) heart failure
I50.42 Chronic combined systolic (congestive) and diastolic (congestive) heart failure
I50.43 Acute on chronic combined systolic (congestive) and diastolic (congestive) heart failure
I50.810 Right heart failure, unspecified
I50.811 Acute right heart failure
I50.812 Chronic right heart failure
I50.813 Acute on chronic right heart failure
I50.814 Right heart failure due to left heart failure
I50.82 Biventricular heart failure
I50.83 High output heart failure
I50.84 End stage heart failure
I50.89 Other heart failure
I50.9 Heart failure, unspecified
I51.4 Myocarditis, unspecified
I51.5 Myocardial degeneration
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