PULMONARY

HYPERTENSION

Presented by:
Christine A. Patriarca
BSN-4A
• Pulmonary hypertension (PH) 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 increased pressure in the blood
vessels of the lungs means that your heart has to work
harder to pump blood into the lungs.
• This, in turn, causes strain that can lead to the heart
becoming larger, weaker, and and cannot do its job and
eventually result in right heart failure.

• Pulmonary blood pressure is normally a lot lower than

systemic blood pressure. Normal pulmonary artery
pressure is 8-20 mm Hg at rest. If the pressure in the
pulmonary artery is greater than 25 mm Hg at rest or
30 mmHg during physical activity, it is abnormally high
and is called pulmonary hypertension.
• Pulmonary hypertension (PH) is a serious health
condition that results when the arteries carrying
blood from the right side of the heart to the lungs
are constricted, disrupting blood flow. Blood must
travel through the lungs for air exchange in order
to pick up oxygen that it delivers to all the organs,
muscles, and tissue in the body.
• When the arteries between the heart and lungs
become narrowed and flow is constricted, the
heart has to work extra hard to pump blood to the
lungs. Over time, the heart can grow weak and
proper circulation can diminish throughout the
body.
 STATISTICS:
-Approximately 10% of patients diagnosed with pulmonary arterial
hypertension (PAH) have a family history of the disease.
- referred to as having familial PAH (FPAH)
-Idiopathic PAH (IPAH) has an annual incidence of 1-2 cases per million
people
Pulmonary hypertension, also known as pulmonary arterial hypertension, is
a rare condition affecting 1 to 2 people in every 1 million over the US and
Europe. Older women are at higher risk of the condition.
- While the exact frequency of the condition is unknown, it is estimated that
about 1,000 new cases occur a year in the United States.[3][2] Females are
more often affected than males.[2] Onset is typically between 20 and 60
years of age
There’s currently no readily available cure for PH. It’s a progressive disease,
which means it can advance over time, sometimes much faster for some
people than others. If left untreated, the disease can become life-
threatening within a couple of years.
If you have PH and systemic scleroderma, an autoimmune skin disease that
also affects the small arteries and internal organs, your two-year survival
odds are estimated to be 40 percent, according to a 2008 study. Survival
rates with PH depend on the cause of the condition.
• Pulmonary hypertension classifications
• Once you've been diagnosed with pulmonary
hypertension, your doctor might classify the severity of
your disease into one of several classes, including:
• Class I. Although you've been diagnosed with
pulmonary hypertension, you have no symptoms with
normal activity.
• Class II. You don't have symptoms at rest, but you
experience symptoms such as fatigue, shortness of
breath or chest pain with normal activity.
• Class III. You're comfortable at rest, but have symptoms
when you're physically active.
• Class IV. You have symptoms with physical activity and
while at rest.
• 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.
 TYPES:
• Group 1 PAH (Pulmonary arterial
hypertension)
• Group 2 PH (left heart disease)
• Group 3 PH (lung disease)
• Group 4 PH (thromboembolic disease)
• Group 5 PH (multifactorial)
 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 (FPAH) or (HPH)
• PAH caused by certain drugs or toxins including some recreational drugs
and diet medications
• PAH associated with other conditions such as connective tissue diseases
like :
• scleroderma or lupus (connective tissue diseases certain autoimmune
diseases)
• congenital heart problems
• high blood pressure in the liver
• HIV
• certain infections like schistosomiasis (a type of parasitic infection)
• sickle cell anemia
• liver disease
• 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 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 (such as pulmonary fibrosis),
which can cause scarring on lung tissue
• 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 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
( polycythemia vera and thrombocythemia)
• 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
 PREDISPOSING FACTORS:
-Family history & Genetics
Certain genetic disorders, such as Down syndrome, congenital heart
disease, and Gaucher disease, can increase your risk of developing
pulmonary hypertension.
A family history of blood clots or pulmonary embolism also increases your
risk of developing Pulmonary hypertension.
Age
Your risk of pulmonary hypertension goes up as you get older, although it
may occur at any age. The condition is typically diagnosed between ages
30 and 60.
Sex
Pulmonary hypertension is more common in women than in men.
Pulmonary hypertension with certain types of heart failure is also more
common in women.
 PRECIPITATING FACTORS:
• -Lifestyle habits
• Unhealthy lifestyle habits can increase the risk of
pulmonary hypertension.
• -Illegal drugs
• such as cocaine and amphetamines
• -Smoking
• You're overweight
• You take certain appetite-suppressant
medications
• You have one of various conditions that
can increase your risk of developing
pulmonary hypertension
PATHOPISIOLOGY:
 CLINICAL MANIFESTATIONS:
• Early symptoms include:
• Shortness of breath during routine activity and eventually
while at rest
• Fatigue
• Chest pain (angina)
• Racing heartbeat
• Pain in upper right side of abdomen
• Decreased appetite
• Later symptoms include:
• Feeling light-headed, especially during physical activity
• Dizziness or fainting (syncope)
• Swelling in the ankles or legs
• Bluish color to lips or skin (cyanosis)
 LABORATORY & DIAGNOSTIC
• TESTS:
Pulmonary hypertension is hard to diagnose early because it's not often
detected in a routine physical exam. Even when the condition is more
advanced, its signs and symptoms are similar to those of other heart and
lung conditions.
• Echocardiogram. Sound waves can create moving images of the beating
heart. An echocardiogram can help your doctor to check the size and
functioning of the right ventricle, and the thickness of the right ventricle's
wall. An echocardiogram can also show how well your heart chambers and
valves are working. Doctors may also use this to measure the pressure in
your pulmonary arteries.
• In some cases, your doctor will recommend an exercise echocardiogram to
help determine how well your heart and lungs work under stress. In this
test, you'll have an echocardiogram before exercising on a stationary bike or
treadmill and another test immediately afterward. This could be done as an
oxygen consumption test, in which you may have to wear a mask that
assesses the ability of your heart and lungs to deal with oxygen and carbon
dioxide.
• Other exercise tests may also be done. These tests can help determine the
severity and cause of your condition. They may also be done at follow-up
appointments to check that your treatments are working.
• Chest X-ray. A chest X-ray can show images of your heart, lungs
and chest. This test can show enlargement of the right ventricle of
the heart or the pulmonary arteries, which can occur in pulmonary
hypertension. This test can also be used to identify other
conditions that may be causing pulmonary hypertension.
• Electrocardiogram (ECG). This noninvasive test shows your heart's
electrical patterns and can detect abnormal rhythms. Doctors may
also be able to see signs of right ventricle enlargement or strain.
• Right heart catheterization. After you've had an echocardiogram,
if your doctor thinks you have pulmonary hypertension, you'll
likely have a right heart catheterization. This test can often help
confirm that you have pulmonary hypertension and determine the
severity of your condition.
• During the procedure, a cardiologist places a thin, flexible tube
(catheter) into a vein in your neck or groin. The catheter is then
threaded into your right ventricle and pulmonary artery.
• Right heart catheterization allows your doctor to directly measure
the pressure in the main pulmonary arteries and right ventricle.
It's also used to see what effect different medications may have on
your pulmonary hypertension.
• Blood tests. Your doctor might order blood
tests to check for certain substances in your
blood that might show you have pulmonary
hypertension or its complications. Blood tests
can also test for certain conditions that may
be causing your condition.
• Cardiac MRI. to get detailed pictures of the
structure and functioning of the heart and
surrounding blood vessels
 COMPLICATIONS:
• Pulmonary hypertension can lead to a number of complications, including:
• 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.
• 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.
• 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.
 NURSING MANAGEMENT:
• Although medical treatment can't cure pulmonary hypertension, it
can lessen symptoms. Lifestyle changes also can help improve your
condition. Consider these tips:

• Get plenty of rest. Resting can reduce the fatigue that might come
from having pulmonary hypertension.

• Stay as active as possible. Even the mildest forms of activity might

be too exhausting for some people who have pulmonary
hypertension. For others, moderate exercise such as walking might
be beneficial — especially when done with oxygen. Discuss the
level of activity with your doctor about specific exercise
restrictions. Avoid straining or lifting heavy weights. Rest when you
need to.
• Don't smoke. If you smoke, the most important thing you can
do for your heart and lungs is to stop. If you can't stop
smoking by yourself, ask your doctor to prescribe a treatment
plan to help you quit. Also, avoid secondhand smoke if
possible.
• Avoid pregnancy and birth control pills. If you're a woman of
childbearing age, avoid pregnancy. Pregnancy can be life-
threatening for both you and your baby. Also avoid using birth
control pills, which can increase your risk of blood clots. Talk
to your doctor about alternative forms of birth control. If you
do become pregnant, it's important to consult with your
doctor as pulmonary hypertension can cause serious
complications to both you and the fetus.
• Avoid traveling to or living at high altitudes. High altitudes
can worsen the symptoms of pulmonary hypertension. If you
live at an altitude of 8,000 feet (2,438 meters) or higher, your
doctor might recommend that you move to a lower altitude.
Be cautious about air travel or high-altitude locales. You may
need to travel with extra oxygen.
• Get vaccines. Your doctor may recommend getting an
influenza and pneumonia vaccine, as these conditions
can cause serious issues for people with pulmonary
hypertension.
• Get support. If you're feeling stressed or worried due
to your condition, get support from family or friends.
Or, consider joining a support group with others who
have pulmonary hypertension
• Heart-healthy eating, which includes eating less salt, to
lower blood pressure or cholesterol if high levels of
these contributed to the cause of your pulmonary
hypertension. Eating less salt will help control your
body fluids and may improve heart function. Aim to eat
a healthy diet of whole grains, a variety of fruits and
vegetables, lean meats and low-fat dairy products.
Avoid saturated fat, trans fat and cholesterol. Aim to
maintain a healthy weight.
• Physical rehabilitation to improve your ability
to exercise and also boost your quality of life
• Ask your doctor about medications. Take all
your medications as prescribed. Ask your
doctor about any other medications before
taking them, as some can interfere with your
medication or worsen your condition.
• Watch your weight. A daily record of your
weight can help you be aware of rapid weight
gain, which may be a sign that your pulmonary
hypertension is worsening.
 MEDCAIL MANAGEMENT:
• Blood vessel dilators (vasodilators). Vasodilators open
narrowed blood vessels. To relax blood vessels and
lower blood pressure in the pulmonary artery most
affected in people who have pulmonary arterial
hypertension. One of the most commonly prescribed
vasodilators for pulmonary hypertension is
epoprostenol (Flolan, Veletri). The drawback to
epoprostenol is that its effects last only a few minutes.
• This includes calcium channel blockers such as
nifedipine and diltiazem, as well as newer groups of
medicines called endothelin receptor antagonists and
phosphodiesterase type 5 inhibitors.
• Anticoagulation or blood thinners to prevent
blood clots in people whose pulmonary
hypertension is caused by chronic blood clots
in the lungs. These thinners also can help
some people who have pulmonary arterial
hypertension, heart failure, or other risk
factors for blood clots.
• Digitalis, or digoxin to control the rate blood
is pumped throughout the body.
 SURGICAL MANAGEMENT:
• Atrial septostomy. If medications don't control your pulmonary
hypertension, this open-heart surgery might be an option. In an
atrial septostomy, a surgeon will create an opening between the
upper left and right chambers of your heart (atria) to relieve the
pressure on the right side of your heart.
• Atrial septostomy can have serious complications, including heart
rhythm abnormalities (arrhythmias).
• Transplantation. In some cases, a lung or heart-lung transplant
might be an option, especially for younger people who have
idiopathic pulmonary arterial hypertension.
• Major risks of any type of transplantation include rejection of the
transplanted organ and serious infection, and you must take
immunosuppressant drugs for life to help reduce the chance of
rejection.
• Oxygen therapy if oxygen levels in the blood are too low.
• Balloon atrial septostomy to decrease pressure in the right
heart chambers and improve the output of the left heart and
oxygenation of the blood. In this procedure, a small hole is
made in the wall between the right and left atria to allow
blood to flow from the right to the left atrium.
• Balloon pulmonary angioplasty to lower the blood pressure
in your pulmonary artery and improve heart function in
people who cannot have a pulmonary endarterectomy.
• Pulmonary endarterectomy surgery to remove blood clots
from the inside of the blood vessels of the lungs.
• Blood pressure medicines such as angiotensin-converting
enzymes inhibitors, beta blockers, or calcium channel blockers
when left heart disease is the cause
• Blood transfusions or hydroxyurea to treat sickle cell disease
• Heart valve repair
 NURSING DIAGNOSIS:
• Decreased Cardiac Output related to inadequate
blood pumped by the heart to meet metabolic
demands of the body.
• Ineffective Tissue Perfusion related to impaired
circulation.
• Acute pain related to increased cerebral vascular
pressure.
• Decreased cardiac output related to increased
afterload, vasoconstriction, myocardial ischemia,
ventricular hypertrophy.
• Knowledge Deficit related to lack of information
about the disease process and self-care.
 RESEARCH ABSTRACT:
• Clinical trial design and new therapies for pulmonary arterial hypertension
• Olivier Sitbon, Mardi Gomberg-Maitland, John Granton, Michael I. Lewis, Stephen
C. Mathai, Maurizio Rainisio, Norman L. Stockbridge, Martin R. Wilkins, Roham
T. Zamanian, Lewis J. Rubin
• European Respiratory Journal 2018; DOI: 10.1183/13993003.01908-2018
• Until 20 years ago the treatment of pulmonary arterial hypertension (PAH) was
based on case reports and small series, and was largely ineffectual. As a deeper
understanding of the pathogenesis and pathophysiology of PAH evolved over the
subsequent two decades, coupled with epidemiological studies defining the clinical
and demographic characteristics of the condition, a renewed interest in treatment
development emerged through collaborations between international experts,
industry and regulatory agencies. These efforts led to the performance of robust,
high-quality clinical trials of novel therapies that targeted putative pathogenic
pathways, leading to the approval of more than 10 novel therapies that have
beneficially impacted both the quality and duration of life. However, our
understanding of PAH remains incomplete and there is no cure. Accordingly, efforts
are now focused on identifying novel pathogenic pathways that may be targeted,
and applying more rigorous clinical trial designs to better define the efficacy of
these new potential treatments and their role in the management scheme. This
article, prepared by a Task Force comprised of expert clinicians, trialists and
regulators, summarises the current state of the art, and provides insight into the
opportunities and challenges for identifying and assessing the efficacy and safety of
new treatments for this challenging condition.
 REFERENCES:
• https://pulmonaryhypertensionnews.com
• https://www.mayoclinic.org
• https://www.heart.org
• https://phassociation.org
• https://erj.ersjournals.com
• https://medlineplus.gov
• https://www.nhlbi.nih.gov
• https://www.thoracic.org
• https://nanda-nursinginterventions.blogspot.com
• https://www.slideshare.net
• http://www.aboutpulmonaryhypertension.com