Where do I fit into the pulmonary hypertension classification table?
Do I know what type of pulmonary hypertension I have?
What's my exercise capacity?
What group do I fit into?
Find out more here with the updated information from the World Health Organisation (WHO) classification table.
Do I know what type of pulmonary hypertension I have?
What's my exercise capacity?
What group do I fit into?
Find out more here with the updated information from the World Health Organisation (WHO) classification table.
Classification for Pulmonary Hypertension
Classifying Pulmonary Hypertension through Exercise Capacity and Symptoms
There are four functional classes that are used to rate how ill PH patients are.
Currently, the World Health Organization or WHO class system is used to describe these patients.
It is important to distinguish WHO functional class from WHO group. The WHO group system classifies what type of pulmonary hypertension a patient has while the WHO functional class system gives information about how affected they are by their disease.
The higher the class the more severe the disease and the greater the urgency for the patient to be accurately diagnosed and started on therapy for pulmonary hypertension.
It is important to treat all classes of pulmonary arterial hypertension (PAH) as the disease is progressive, meaning it will get worse over time without treatment.
Classifying Pulmonary Hypertension through Exercise Capacity and Symptoms
There are four functional classes that are used to rate how ill PH patients are.
Currently, the World Health Organization or WHO class system is used to describe these patients.
It is important to distinguish WHO functional class from WHO group. The WHO group system classifies what type of pulmonary hypertension a patient has while the WHO functional class system gives information about how affected they are by their disease.
The higher the class the more severe the disease and the greater the urgency for the patient to be accurately diagnosed and started on therapy for pulmonary hypertension.
It is important to treat all classes of pulmonary arterial hypertension (PAH) as the disease is progressive, meaning it will get worse over time without treatment.
WHO Class I
Class I: No symptoms of pulmonary arterial hypertension with exercise or at rest. It is rare for a patient to be diagnosed while still a class I.
Patients that are being screened because of high risk factors for developing pulmonary hypertension, such as patients with scleroderma or family history of PAH, may rarely be diagnosed as class I. More often this classification is used to describe patients that have demonstrated a substantial response to therapy that were once a class II or III but have improved to a class I.
WHO Class II
Class II: No symptoms at rest but uncomfortable and short of breath with normal activity such as climbing a flight of stairs, grocery shopping, or making the bed.
WHO Class III
Class III: May not have symptoms at rest but activities greatly limited by shortness of breath, fatigue, or near fainting. Patients in this class have a difficult time doing normal chores around the house and have to take breaks while doing activities of daily living.
WHO Class IV
Class IV: Symptoms at rest and severe symptoms with any activity. Patients in this class may faint especially while bending over with their heads lowered. Most patients in this class are also volume overloaded with edema in their feet and ankles from right heart failure.
Class I: No symptoms of pulmonary arterial hypertension with exercise or at rest. It is rare for a patient to be diagnosed while still a class I.
Patients that are being screened because of high risk factors for developing pulmonary hypertension, such as patients with scleroderma or family history of PAH, may rarely be diagnosed as class I. More often this classification is used to describe patients that have demonstrated a substantial response to therapy that were once a class II or III but have improved to a class I.
WHO Class II
Class II: No symptoms at rest but uncomfortable and short of breath with normal activity such as climbing a flight of stairs, grocery shopping, or making the bed.
WHO Class III
Class III: May not have symptoms at rest but activities greatly limited by shortness of breath, fatigue, or near fainting. Patients in this class have a difficult time doing normal chores around the house and have to take breaks while doing activities of daily living.
WHO Class IV
Class IV: Symptoms at rest and severe symptoms with any activity. Patients in this class may faint especially while bending over with their heads lowered. Most patients in this class are also volume overloaded with edema in their feet and ankles from right heart failure.
World Health Organisation (WHO) Classification
Pulmonary hypertension is a severe, rare lung disease characterized by high blood pressure in the pulmonary arteries, which deliver blood from the heart to the lungs. The high pressure causes the heart to work harder to pump blood. This, in turn, causes strain that can lead to the heart becoming larger and weaker, and eventually result in right heart failure. There is a wide range of potential causes for pulmonary hypertension, and these can affect the symptoms, treatment, and prognosis of the disease.To produce, maintain, and implement international health information standards, the World Health Organization (WHO) defined a classification for pulmonary hypertension diagnosis in 1973, based on the cause of the disease. This has seen multiple revisions, reflecting advances in disease understanding, with the most recent being presented in 2013 at the Fifth World Symposium on Pulmonary Hypertension in Nice, France.
Aims of the classification
The international classification of diseases (ICD) is created by the organization and comprises a standard diagnostic tool for physicians, nurses, other providers, researchers, health information managers and coders, health information technology workers, policy-makers, insurers, and patient organizations. To determine the classification of pulmonary hypertension, WHO analyzed the general health situation of patients, as well as the disease’s incidence and prevalence.The WHO classification is used for numerous purposes, including records, death certificates, information storage, diagnosis retrieval, national mortality and morbidity statistics, reimbursement, resource allocation, and other clinical, epidemiological and quality purposes. The organization expects the classification to help fight the disease.
The classification
In 1973, pulmonary hypertension was classified into only two groups. These were “primary pulmonary hypertension” if the cause of the disease was known, or “secondary pulmonary hypertension” if the cause could not be identified. Secondary pulmonary hypertension also was called “idiopathic pulmonary hypertension.”Now the classification has been expanded to five groups, to emphasize the importance of the underlying cause of the disease. This reflects our increased knowledge of what can cause pulmonary hypertension, along with what impact this can have on disease progression and required treatment.
Aims of the classification
The international classification of diseases (ICD) is created by the organization and comprises a standard diagnostic tool for physicians, nurses, other providers, researchers, health information managers and coders, health information technology workers, policy-makers, insurers, and patient organizations. To determine the classification of pulmonary hypertension, WHO analyzed the general health situation of patients, as well as the disease’s incidence and prevalence.The WHO classification is used for numerous purposes, including records, death certificates, information storage, diagnosis retrieval, national mortality and morbidity statistics, reimbursement, resource allocation, and other clinical, epidemiological and quality purposes. The organization expects the classification to help fight the disease.
The classification
In 1973, pulmonary hypertension was classified into only two groups. These were “primary pulmonary hypertension” if the cause of the disease was known, or “secondary pulmonary hypertension” if the cause could not be identified. Secondary pulmonary hypertension also was called “idiopathic pulmonary hypertension.”Now the classification has been expanded to five groups, to emphasize the importance of the underlying cause of the disease. This reflects our increased knowledge of what can cause pulmonary hypertension, along with what impact this can have on disease progression and required treatment.
The five groups of pulmonary hypertension today are:
Group 1: pulmonary arterial hypertension
Group 1 includes pulmonary hypertension is associated with the narrowing of the small blood vessels in the lungs.
It also is called pulmonary arterial hypertension (PAH) and includes cases where the underlying cause of the narrowing is not known (idiopathic pulmonary hypertension).
There are multiple other subgroups in group 1, including: Familial, or heritable pulmonary hypertension, PAH caused by certain drugs or toxins, PAH associated with other conditions such as connective tissue diseases like scleroderma or lupus, congenital heart problems, high blood pressure in the liver, HIV, certain infections like schistosomiasis, and sickle cell anemia. Group 1 also may be caused by rare blood conditions, like pulmonary veno-occlusive disease (PVOD) or pulmonary capillary hemangiomatosis (PCH), and a type of pulmonary hypertension present in babies called persistent pulmonary hypertension of the newborn (PPHN).
Group 1 includes pulmonary hypertension is associated with the narrowing of the small blood vessels in the lungs.
It also is called pulmonary arterial hypertension (PAH) and includes cases where the underlying cause of the narrowing is not known (idiopathic pulmonary hypertension).
There are multiple other subgroups in group 1, including: Familial, or heritable pulmonary hypertension, PAH caused by certain drugs or toxins, PAH associated with other conditions such as connective tissue diseases like scleroderma or lupus, congenital heart problems, high blood pressure in the liver, HIV, certain infections like schistosomiasis, and sickle cell anemia. Group 1 also may be caused by rare blood conditions, like pulmonary veno-occlusive disease (PVOD) or pulmonary capillary hemangiomatosis (PCH), and a type of pulmonary hypertension present in babies called persistent pulmonary hypertension of the newborn (PPHN).
Group 2: pulmonary hypertension due to left heart disease
Group 2 refers to pulmonary hypertension caused by left heart disease.
Long-term problems with the left side of the heart can lead to changes in the pulmonary arteries and cause pulmonary hypertension.
This may include: Left ventricular systolic dysfunction, when the heart cannot pump blood effectively. Left ventricular diastolic dysfunction, when the heart cannot properly relax to allow enough blood to flow in. Valvular disease, when the valves of the left side of the heart are allowing blood to leak. Congenital heart defects (heart defects from birth), which can lead to problems with blood flowing in or out of the heart.
Group 2 refers to pulmonary hypertension caused by left heart disease.
Long-term problems with the left side of the heart can lead to changes in the pulmonary arteries and cause pulmonary hypertension.
This may include: Left ventricular systolic dysfunction, when the heart cannot pump blood effectively. Left ventricular diastolic dysfunction, when the heart cannot properly relax to allow enough blood to flow in. Valvular disease, when the valves of the left side of the heart are allowing blood to leak. Congenital heart defects (heart defects from birth), which can lead to problems with blood flowing in or out of the heart.
Group 3: pulmonary hypertension due to lung disease and/or chronic hypoxia
Group 3 includes pulmonary hypertension resulting from lung diseases or shortage of oxygen in the body (hypoxia).
The common diseases associated with group 3 pulmonary hypertension are: Chronic obstructive pulmonary disease (COPD), Interstitial lung disease Sleep-disordered breathing, a group of diseases that affect breathing during sleep like obstructive sleep apnea (OSA), Chronic high-altitude exposure, Lung developmental abnormalities, Alveolar hypoventilation disorders
Group 3 includes pulmonary hypertension resulting from lung diseases or shortage of oxygen in the body (hypoxia).
The common diseases associated with group 3 pulmonary hypertension are: Chronic obstructive pulmonary disease (COPD), Interstitial lung disease Sleep-disordered breathing, a group of diseases that affect breathing during sleep like obstructive sleep apnea (OSA), Chronic high-altitude exposure, Lung developmental abnormalities, Alveolar hypoventilation disorders
Group 4: pulmonary hypertension due to blood clots in the lungs
Group 4 refers to pulmonary hypertension caused by blood clots obstructing the pulmonary arteries.
This also can be referred to as chronic thromboembolic pulmonary hypertension (CTEPH). Clots are the body’s response to bleeding and injuries, but can harm the heart and lungs when they occur without an apparent cause. Pulmonary emboli are blood clots that travel to the lungs, and pulmonary thrombosis are clots that are formed in the lungs, which can block the pulmonary arteries.
Group 4 refers to pulmonary hypertension caused by blood clots obstructing the pulmonary arteries.
This also can be referred to as chronic thromboembolic pulmonary hypertension (CTEPH). Clots are the body’s response to bleeding and injuries, but can harm the heart and lungs when they occur without an apparent cause. Pulmonary emboli are blood clots that travel to the lungs, and pulmonary thrombosis are clots that are formed in the lungs, which can block the pulmonary arteries.
Group 5: pulmonary hypertension due to blood and other disorders
Group 5 is the last category and includes other less-common causes that do not fit into any of the other four groups.
These are widely split into four categories: Blood disorders, such as some types of anemia. Systemic disorders, such as sarcoidosis (a condition that results in inflammation of different organs like the lungs and lymph nodes) and histiocytosis (a rare disorder that causes scarring). Metabolic disorders, such as glycogen storage diseases and thyroid disorders. Other disorders, such as chronic kidney failure or tumors obstructing pulmonary arteries.
Group 5 is the last category and includes other less-common causes that do not fit into any of the other four groups.
These are widely split into four categories: Blood disorders, such as some types of anemia. Systemic disorders, such as sarcoidosis (a condition that results in inflammation of different organs like the lungs and lymph nodes) and histiocytosis (a rare disorder that causes scarring). Metabolic disorders, such as glycogen storage diseases and thyroid disorders. Other disorders, such as chronic kidney failure or tumors obstructing pulmonary arteries.
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for the Classification Table in full from the (World Health Organisation - WHO)
for the Classification Table in full from the (World Health Organisation - WHO)
Shared with you through www.pulmonaryhypertensionnews.com - Information research supplied by Patricia Silva PhD and fact checked by Ozge Ozkaya - please refer to the original content online for more information.
