Pulmonary hypertension is high blood pressure in the lungs and right side of the heart. It is classified into 5 groups based on cause. Symptoms include shortness of breath, fatigue, dizziness, and swelling. Diagnosis involves echocardiogram, chest X-ray, CT/MRI scans, and right heart catheterization. Treatment includes medications like diuretics, prostacyclin, and endothelin receptor antagonists as well as surgical procedures like atrial septostomy and lung/heart-lung transplants. Lifestyle changes and oxygen therapy can also help manage symptoms.

1. Pulmonary Hypertension

2. Definition

a type of high blood pressure that affects the arteries
in your lungs and the right side of your heart.

Usually pressure in the lung arteries are normally
lower than the pressures in the systemic circulation

PH occurs when pressure in the pulmonary circuit
becomes abnormally elevated

3. Classifications of Pulmonary
Hypertension
according to WHO 1998

Group 1: Pulmonary arterial hypertension
− Cause unknown, known as idiopathic pulmonary
arterial hypertension
− A specific gene mutation that can cause pulmonary
hypertension to develop in families, also called heritable
pulmonary arterial hypertension
− Certain drugs — such as certain prescription diet
drugs or illegal drugs such as methamphetamines — or
certain toxins
− Heart abnormalities present at birth (congenital heart
disease)
− Other conditions, such as connective tissue disorders
(scleroderma, lupus, others), HIV infection or chronic

4. Classifications of Pulmonary
Hypertension
according to WHO 1998

Group 2: Pulmonary hypertension caused by left-
sided heart disease
− Left-sided valvular heart disease, such as mitral
valve or aortic valve disease
− Failure of the lower left heart chamber (left
ventricle)

5. Classifications of Pulmonary
Hypertension
according to WHO 1998

Group 3: Pulmonary hypertension caused by lung
disease
− Chronic obstructive pulmonary disease, such as
emphysema
− Lung disease such as pulmonary fibrosis, a
condition that causes scarring in the tissue between
the lungs' air sacs (interstitium)
− Sleep apnea and other sleep disorders
− Long-term exposure to high altitudes in people
who may be at higher risk of pulmonary
hypertension

6. Classifications of Pulmonary
Hypertension
according to WHO 1998

Group 4: Pulmonary hypertension caused by chronic
blood clots
− Chronic blood clots in the lungs (pulmonary
emboli)

7. Classifications of Pulmonary
Hypertension
according to WHO 1998

Group 5: Pulmonary hypertension associated with
other conditions that have unclear reasons why the
pulmonary hypertension occurs
− Blood disorders
− Disorders that affect several organs in the body,
such as sarcoidosis
− Metabolic disorders, such as glycogen storage
disease
− Tumors pressing against pulmonary arteries

8. Risk Factors

Family history. If two or more members of your family have PH or if a
family member in your lineage is known to have a PH-causing gene
mutation, the risk of getting PH is more likely.

Obesity and obstructive sleep apnea. In isolation, obesity is not a risk
factor. However, if obesity is combined with obstructive sleep apnea
(meaning that oxygen levels fall while a person is sleeping), mild PH
may occur.

Gender. Idiopathic PAH and heritable PAH (also known as familial
PAH) are at least two-and-a-half times more common in women than in
men. Females of childbearing age are also more susceptible.

Pregnancy. Pregnancy is a possible risk factor suggested by registries
and expert opinion.

Altitude. Living at a high altitude for years can make you more
predisposed to PH. When travelling to high altitudes, your PH
symptoms can be aggravated by the altitude.

9. Risk Factors

Other diseases. Other diseases, including congenital heart disease, lung
disease, liver disease and connective tissue disorders like scleroderma
and lupus, can lead to the development of pulmonary hypertension.

Drugs and toxins. Certain drugs, such as methamphetamines and the
diet drug “fen phen,” are known to cause pulmonary hypertension.

10. Pathophysiology of Pulmonary
Hypertension

11. Abnormally high BP in pulmonary arteries
Increased pressure damages large and small pulmonary
arteries
Thickness of Blood vessel walls
Inability to transfer oxygen and carbon dioxide
normally
Levels of oxygen in blood fall
Constriction of pulmonary arteries
increase in pressure in pulmonary circulation

12. Complications of Pulmonary
Hypertension

Right-sided heart enlargement and heart failure
(cor pulmonale). In cor pulmonale, your heart's
right ventricle becomes enlarged and has to pump
harder than usual to move blood through narrowed
or blocked pulmonary arteries.

At first, the heart tries to compensate by thickening
its walls and expanding the chamber of the right
ventricle to increase the amount of blood it can hold.
But this thickening and enlarging works only
temporarily, and eventually the right ventricle fails
from the extra strain.

13. Complications of Pulmonary
Hypertension

Blood clots. Clots help stop bleeding after you've
been injured. But sometimes clots form where
they're not needed. A number of small clots or just a
few large ones dislodge from these veins and travel
to the lungs, leading to a form of pulmonary
hypertension that can generally be reversible with
time and treatment.

Having pulmonary hypertension makes it more
likely you'll develop clots in the small arteries in
your lungs, which is dangerous if you already have
narrowed or blocked blood vessels.

14. Complications of Pulmonary
Hypertension

Arrhythmia. Irregular heartbeats (arrhythmias)
from the upper or lower chambers of the heart are
complications of pulmonary hypertension. These
can lead to palpitations, dizziness or fainting and
can be fatal.

Bleeding. Pulmonary hypertension can lead to
bleeding into the lungs and coughing up blood
(hemoptysis). This is another potentially fatal
complication.

16. Signs and Symptoms

Shortness of breath during routine activity, such as climbing a flight of stairs

Fatigue

Dizziness or fainting spells (syncope)

Chest pain

Swelling (edema) in your ankles, legs and eventually in your abdomen (ascites)

Bluish color to your lips and skin (cyanosis)

Racing pulse or heart palpitations

Decreased appetite

coughing (sometimes with blood) and wheezing

distended neck veins

enlarged liver

17. Functional Classification of PH
according to WHOFunctional Classification
• Class I- Patients with pulmonary hypertension but without resulting limitation of
physical activity. Ordinary physical activity does not cause undue dyspnoea or
fatigue, chest pain, or near syncope.
B. Class II- patients with pulmonary hypertension resulting in slight limitation of
physical activity. They are comfortable at rest. Ordinary physical activity causes
undue dyspnoea or fatigue, chest pain, or near syncope.
C. Class III- patients with pulmonary hypertension resulting in marked. Limitation of
physical activity. They are comfortable at rest. Less than ordinary activity causes
undue dyspnoea, fatigue, and chest pain or near syncope.
D. Class IV -patients with pulmonary hypertension with inability to carry out any
physical activity without symptoms. these patients manifest signs of right heart
failure. Dyspnoea and /or fatigue may be present even at rest. Discomfort is
increased by any physical activity
This is done by either a 6-minute walk test or a cardiopulmonary exercise test

18. Diagnostic Examinations

Assessment/Interview
− history of present illnesses
− past medical history
− Family history
− Any past or present medications that
the patient may have taken
− physical examination will also take
place.

19. Diagnostic Examinations

Echocardiography. or echo, uses sound waves
to create a moving picture of your heart. This
test can estimate the pressure in your
pulmonary arteries. Echo also can show the
size and thickness of your right ventricle and
how well it's working.

20. Diagnostic Examinations

Chest x ray. takes pictures of the structures in
your chest, such as your heart, lungs, and
blood vessels. This test can show whether your
pulmonary arteries and right ventricle are
enlarged.

The pulmonary arteries and right ventricle
may get larger if the right ventricle has to
work hard to pump blood through the
pulmonary arteries.

A chest x ray also may show signs of an
underlying lung disease that's causing or
contributing to PH.

21. Diagnostic Examinations

22. Diagnostic Examinations

EKG (electrocardiogram). An EKG is a simple,
painless test that records the heart's electrical
activity. This test also shows whether your heart's
rhythm is steady or irregular. An EKG may show
whether your right ventricle is enlarged or strained.

23. Diagnostic Examinations

Right heart catheterization. This procedure
measures the pressure in your pulmonary arteries. It
also shows how well your heart is pumping blood to
the rest of your body. Right heart catheterization
can find any leaks between the left and right side of
the heart.

During this procedure, a thin, flexible tube called a
catheter is put into a blood vessel in your groin
(upper thigh) or neck. The tube is threaded into the
right side of your heart and into the pulmonary
arteries. Through the tube, your doctor can do tests
and treatments on your heart.

24. Diagnostic Examinations, others

Chest CT scan. A chest computed tomography (to-MOG-ra-fee) scan, or chest CT scan, creates pictures of
the structures inside your chest, such as your heart, lungs, and blood vessels. These pictures can show
signs of PH or a condition that may be causing PH.

Chest MRI. Chest magnetic resonance imaging, or chest MRI, shows how your right ventricle is working.
The test also shows blood flow in your lungs. Chest MRI also can help detect signs of PH or an underlying
condition causing PH.

Lung function tests. Lung function tests measure how much air you can breathe in and out, how fast you
can breathe air out, and how well your lungs deliver oxygen to your blood. These tests can help detect a
lung disease that may be causing PH.

Polysomnogram (PSG). This test records brain activity, eye movements, heart rate, and blood pressure
while you sleep. A PSG also measures the level of oxygen in your blood. A low oxygen level during sleep is
common in PH, and it can make the condition worse.

A PSG usually is done while you stay overnight at a sleep center. For more information about this test, go
to the Diseases and Conditions Index Sleep Studies article.

Lung ventilation/perfusion (VQ) scan. A lung VQ scan measures air and blood flow in your lungs. This
test can help detect blood clots in your lung's blood vessels.

Blood tests. Blood tests are used to rule out other diseases, such as HIV, liver disease, and autoimmune
diseases (such as rheumatoid arthritis).

25. Treatments for
Pulmonary Hypertension

Pharmacological Treatments
− Diuretics to decrease fluid accumulation
− Cardiac Glycosides (digitalis) to improve cardiac function
− Calcium Channel Blockers – vasodilation
− Intravenous prostacyclin - decrease pulmonary
hypertension by reducing pulmonary vascular resistance
and pressures and increasing cardiac output
− Anticoagulants - to help prevent the formation of blood
clots within the small pulmonary arteries.

26. Treatments for
Pulmonary Hypertension

Pharmacological Treatments
− Endothelin receptor antagonists. These medications reverse
the effect of endothelin, a substance in the walls of blood
vessels that causes them to narrow. These drugs may
improve your energy level and symptoms. However, these
drugs shouldn't be taken if you're pregnant. Also, these
drugs can damage your liver and you may need monthly
liver monitoring.
− Phosphodiesterase-5 inhibitors (Sildenafil and tadalafil.)
are sometimes used to treat pulmonary hypertension. These
drugs work by opening the blood vessels in the lungs to
allow blood to flow through more easily. Side effects can
include an upset stomach, headache and vision problems.

27. Treatments for
Pulmonary Hypertension

Pharmacological Treatments
− Soluble guanylate cyclase (SGC) stimulator. Soluble
guanylate cyclase (SGC) stimulators (Adempas) interact
with nitric oxide and help relax the pulmonary arteries and
lower the pressure within the arteries. These medications
should not be taken if you're pregnant. They can sometimes
cause dizziness or nausea.

28. Treatments for
Pulmonary Hypertension

Surgical Interventions
− Atrial septostomy (sep-TOS-toe-me). For this procedure, a
thin, flexible tube called a catheter is put into a blood vessel
in your leg and threaded to your heart. The tube is then put
through the wall that separates your right and left atria (the
upper chambers of your heart). This wall is called the
septum. A tiny balloon on the tip of the tube is inflated.
This creates an opening between the atria. This procedure
relieves the pressure in the right atria and increases blood
flow.
− Lung transplant. A lung transplant is surgery to replace a
person's diseased lung with a healthy lung from a deceased
donor. This procedure may be used for people who have
severe lung disease that's causing PAH.

29. Treatments for
Pulmonary Hypertension

Surgical Interventions
− Atrial septostomy (sep-TOS-toe-me). For this procedure, a thin,
flexible tube called a catheter is put into a blood vessel in your
leg and threaded to your heart. The tube is then put through the
wall that separates your right and left atria (the upper chambers
of your heart). This wall is called the septum. A tiny balloon on
the tip of the tube is inflated. This creates an opening between the
atria. This procedure relieves the pressure in the right atria and
increases blood flow.
− Lung transplant. A lung transplant is surgery to replace a
person's diseased lung with a healthy lung from a deceased
donor. This procedure may be used for people who have severe
lung disease that's causing PAH.
− Heart–lung transplant. A heart–lung transplant is surgery in
which both the heart and lung are replaced with healthy organs
from a deceased donor.

30. Heart–lung transplant

31. Treatments for
Pulmonary Hypertension

Nursing Interventions
− Fluid restriction
− Periods of Rest and Physical Activity/ Minimize Sedentary
Lifestyle
− Oxygen, especially if you live at a high altitude or have
sleep apnea. Some people who have pulmonary
hypertension eventually require continuous oxygen therapy.
− A low-sodium diet that is both low in calories and high in
healthy carbohydrates is recommended
− Anxiety or depression affect approximately one-third of PH
patients. An interdisciplinary team approach is best as
appropriate medications can be dispersed for distressing
symptoms, and psychological, social, and spiritual support
can be provided.