Types of PH

I know its been a while for me writing on my blogs, and for that I apologize. I just made this statement to another family member and friend, sometimes life gets in the way of life and you forget to let the one's you love know you are still alive and well.

So today's blog is going to be about the WHO GROUP's of Pulmonary Hypertension, the difference between PAH and PH, and the Stages or "CORRECT" terminology "Functional Class".

PAH vs PH ~ PAH mainly relates to those in WHO GROUP 1 any other category that you would fall under for the WHO GROUP would be PH as it is caused by an underlying condition that needs to be treated.   All patients that fall withing WHO GROUP 1 are able to take most all of the medications to treat the condition. Whereas those in the other WHO GROUPS are treated for their underlying disease as the PH medications can be more harmful for you. Please see the remaining article to get a better understanding of what I am stating here.

GROUPS

1. Pulmonary Arterial Hypertension

2. Pulmonary Hypertension due to left heart disease

3. Pulmonary Hypertension due to lung disease

4. Pulmonary Hypertension due to blood clots in the lungs

5. Blood and other rare disorders that lead to Pulmonary Hypertension

Pulmonary Arterial Hypertension:  PAH ~ WHO GROUP I

1. Idiopathic PAH

2. Heritable (genetic, can be passed down)

a. BMPR2 (the most common cause of PAH that runs in families)

b. ALK1, endoglin, SMAD9, CAV1, KCNK3 (less common genetic causes of PAH that runs in families)

3. Drug and Toxin induced

      a. Amphetamines and Methamphetamines

      b. Cocaine

      c. Fenfluramine-Phentermine (prescription diet pills)

4. Associated with other systemic diseases

     a. Connective tissue diseases such as scleroderma, systemic lupus erythematosis, mixed                connective tissue disease and rheumatoid arthritis

     b. HIV infection

     c. Portal hypertension (a type of liver disease that leads to high pressures within the liver)

     d. Congenital heart disease

All available medications are able to assist in slowing down the progression of the disease. 

Pulmonary Hypertension: PH ~ WHO GROUP 2

1. Left ventricular systolic dysfunction (the squeeze of the left ventricle is impaired)

2. Left ventricular diastolic dysfunction (stiffness of the left ventricle leads to pressure elevation in the pulmonary arteries)

3. Valvular heart disease (either narrowing or leakiness of the left heart valves)

4. Left heart due to valvular disease and congenital cardiomyopathies (problems with blood flowing in or out of the left side of the heart not due to a valve problem)

Treatment of Group 2 PH (caused by left heart disease) focuses on the underlying problems. In patients with impaired squeeze of the left ventricle we have a group of medications that improves the left heart function over time. Patients with stiffness of the left side of the heart are treated with diuretics, blood pressure medications if their blood pressure is elevated, weight loss if they are over weight, and CPAP if they have sleep apnea. Patients with valvular heart disease are often treated with surgery to replace the diseased heart valve. Medicines are used to treat fluid retention.

Pulmonary Hypertension: PH ~ WHO GROUP III

1. Chronic obstructive pulmonary disease (COPD)

2. Interstitial lung diseases (scarring and inflammation in the lungs)

3. Sleep-disordered breathing (sleep apnea)

4. Alveolar hypoventilation disorders (diseases that lead to inadequate breathing and increased levels of carbon dioxide in the blood)

5. Chronic high altitude exposure

6. Developmental abnormalities of the lung

For cases of this nature, some of the PH medications can be used, but under proper care from your PH Team, as they still have to provide beneficial ways to treat the underlying condition as well as the PH. 

Pulmonary Hypertension: PH ~ WHO GROUP IV

Chronic Thromboembolic Pulmonary Hypertension otherwise known as CTEPH

Chronic blood clots in the arteries of the lungs. You can have PTE treatment to rid the clots, if you are medically able to do so. This is normally treated with blood thinners and you can also take Adempas.  Some are able to take other forms of the PH medication and is best left to the PH Specialist you are in care with, to determine what will work best.  There are patients in my FB group that you can reach out to and discuss their treatments and how they are dealing with this.  Join us today:  https://www.facebook.com/groups/304235876377617/

Pulmonary Hypertension: Unclear Multifactural Mechanisms WHO GROUP V

1. Hematologic Disorders (certain types of anemia, splenectomy)

2. Systemic Disorders that have lung involvement (Sarcoidosis, Langerhan cell histiocytosis, neurofibromatosis, vasculitis, and lymphangioleimyomatosis)

3. Metabolic Disorders (rare diseases of impaired cell metabolism, thyroid disease)

4. Other diseases not well classified elsewhere (chronic renal failure, tumors obstructing the pulmonary arteries and other rare diseases)

Functional Classification

We DO NOT go by a stage process with our disease like many other diseases out there. We are in a classification of functionality.   A stage of a disease is determined to be the worst case with less likely prognosis of being cured/corrected.  Whereas for PH all Functionality is treatable, but NEVER Curable unless of course you have certain surgeries to correct the PH, like PTE surgery or Transplant of lungs/heart if needed. 

Our Functionality is as follows: 

Class I ~ No symptoms of pulmonary arterial hypertension with exercise or at rest

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.

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.

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.

Information obtained in this article was derived from PHAssociation and PulmonaryHypertensionRN websites and the World Health Organization. For more information regarding this disease please visit either website at: 

www.phassociation.org

www.pulmonaryhypertensionrn.com

http://www.who.int/en/

Thanks for reading and be on the lookout for my next blog: Traveling with PH 💜😊