2. out line:
INTRODUCTION
BRIEF HISTORY
WHAT IS PH?
WHAT CAUSES PH?
SIGNS AND SYMPTOMS
DIAGNOSIS OF PH
TREATMENT OF PH
3. Introduction
In the human body, there are two types of
circulation that enable distribution of
blood throughout the body.
The portion that pumps oxygenated blood
from the left side of the heart via the left
ventricle to all parts of the body is known
as the systemic circulation
4. Introduction
On the other hand, the portion that
pumps deoxygenated blood from the
right side of the heart via the right
ventricle into the lungs to obtain
oxygen is referred to as the
PULMONARY CIRCULATION.
5. INTRODUCTION
Millions of people are affected by a
condition known as high blood
pressure (hypertension) whereby the
blood travels through the body’s
arteries at a pressure higher than
normal.
6. Pulmonary hypertension is a less common type of
high blood pressure that affects the arteries in
the lungs.
Pressures in the lung arteries are normally
lower than the pressures in the systemic
circulation.
Pulmonary hypertension occurs when the
pressure in the pulmonary circulation becomes
abnormally elevated.
7. This disease can occur in men, women and
children of all ages. However, it is most
common in females between 20 and 40 years
old.
The condition is rare in children but is
sometimes seen in infants born with heart
defects. Pulmonary hypertension may be a
primary or secondary cause of hypoxia in
neonates.
8. Ordinarily, blood vessels in the lungs provide less
resistance to blood flow than blood vessels in the rest of
the body . And blood pressure is usually much lower in
the lungs. While pressure in general circulation is about
120/80 mm Hg, in the pulmonary arteries, it is only
around 25/15 mm Hg.
Mean (average) pulmonary artery pressure
= number between highest and lowest pressures
Normal
at rest : 14 mm Hg
Pulmonary hypertension
at rest : 25 mm Hg during exercise : 30 mm Hg
10. Primary Pulmonary Hypertension
no underlying cause for the high blood pressure
in lungs
Begin with spasm of the muscle layer in
pulmonary arteries
patients are sensitive to substances that cause
blood vessels to constrict
may have an inherited predisposition for the
disease
11. causes of Secondary Pulmonary
Hypertension
• results directly from another medical
problem
•Chronic Obstructive Pulmonary Disease,
scleroderma, pulmonary fibrosis, lung
diseases such as asbestosis in this
diseases flow of blood impedes through the
lungs.
13. Causes of secondary PH
blood clots in the lungs (pulmonary emboli)
chronic obstructive pulmonary disease such as
emphysema
Connective tissue disorders, such as scleroderma
Upper airway obstructed during sleep
obesity with reduced ability to breathe
lung diseases such as pulmonary fibrosis (causes
scarring in the tissue between the lungs’ air sacs)
14. Left-sided heart failure
• heart’s left ventricle weakens and cannot
pump out enough blood
• increase in pressure backs up blood through
pulmonary veins to arteries in lungs
Conginital heart disease
16. Abnormally high BP in pulmonary arteries
Increased pressure damages large and small pulmonary
arteries
Thickness of Blood vessel walls
Cannot transfer oxygen and carbon dioxide normally
Levels of oxygen in blood fall
Constriction of pulmonary arteries
increase in pressure in pulmonary circulation
17. Pulmonary Hypertension
right side of heart must work harder push blood through
pulmonary arteries to
lungs
cor pulmonale right ventricle thickens
and enlarges
Heart Failure
In some people, the bone marrow will produce more red blood cells to
compensate for less of oxygen in blood.
Extra RBCs cause the blood to become thicker and stickier, further increasing
the load on the heart Pulmonary Embolism
18.
19. Functional Classification
A. 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
21. Signs and symptoms
shortness of breath fatigue or light-
headed upon exertion
dizziness or fainting spells (syncope)
swelling (edema) in ankles, legs and
eventually in abdomen (ascites) – fluid leak
out of veins and into tissues
bluish colour to lips and skin (cyanosis)
22. coughing (sometimes with blood) and
wheezing
distended neck veins
enlarged liver
heart palpitations
Chest pain
feel weak – body tissues not receiving
enough oxygen
24. 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.
27. Electrocardiogram (ECG)
ECG is a record of the electrical activity produced by the
heart. Abnormal rhythms (arrhythmias) may indicate that
the heart or part of the heart is undergoing unusual
stress.
28. Pulmonary Angiogram
Used to measure circulation in the lungs and to visualize
clots in the lung on x-rays. The test involves insertion of
a thin catheter into the pulmonary artery through which
an iodine dye is injected.
Image of any blood clots present in the lung can be
observed and circulation of blood through lung’s blood
vessels can be tracked.
29. Pulmonary Function Tests
Non-invasive tests to measure how much air
your lungs can hold and the airflow in and out of
your lungs. They can also measure the amount
of gases exchanged across the membrane
between the lung wall and capillary membrane.
During the tests, the patient will be asked to
blow into a spirometer. An abnormality here may
be amongst the first indication of PH.
31. fluid restriction
diuretics to decrease fluid accumulation
cardiac glycosides (eg, digitalis) in an attempt
to improve cardiac function
calcium channel blockers for vasodilation,
and rest.
Intravenous prostacyclin helps to decrease
pulmonary hypertension by reducing
pulmonary vascular resistance and pressures
and increasing cardiac output
32. Anticoagulants such as warfarin have
been given to patients because of chronic
pulmonary emboli.
Heart— lung transplantation has been
successful in select patients with primary
hypertension who have not been responsive
to other therapies
Millions of people are affected by high blood pressure (hypertension), a condition in which blood travels through the body's arteries at a pressure too high for good health. A far less common type of high blood pressure affects only the arteries in the lungs. Known as pulmonary hypertension, it's a serious illness that becomes progressively worse. For some people, pulmonary hypertension eventually proves fatal. Pulmonary hypertension begins when tiny arteries in your lungs become narrow or blocked. This causes increased resistance to the flow of blood in the lungs, which in turn raises pressure within the pulmonary arteries. As the pressure builds, your heart's lower right chamber (right ventricle) must work harder to pump blood through your lungs, eventually causing the heart muscle to weaken and sometimes to fail completely. When a doctor or a nurse measures the blood pressure on a person's arm, he/she is measuring the pressures in the systemic circulation. When these pressures are abnormally high, the person is diagnosed as having high blood pressure (hypertension). The pressures in the lung arteries (pulmonary arteries) are normally significantly lower than the pressures in the systemic circulation. When pressure in the pulmonary circulation becomes abnormally elevated, it is referred to as pulmonary hypertension.
Millions of people are affected by high blood pressure (hypertension), a condition in which blood travels through the body's arteries at a pressure too high for good health. A far less common type of high blood pressure affects only the arteries in the lungs. Known as pulmonary hypertension, it's a serious illness that becomes progressively worse. For some people, pulmonary hypertension eventually proves fatal. Pulmonary hypertension begins when tiny arteries in your lungs become narrow or blocked. This causes increased resistance to the flow of blood in the lungs, which in turn raises pressure within the pulmonary arteries. As the pressure builds, your heart's lower right chamber (right ventricle) must work harder to pump blood through your lungs, eventually causing the heart muscle to weaken and sometimes to fail completely. When a doctor or a nurse measures the blood pressure on a person's arm, he/she is measuring the pressures in the systemic circulation. When these pressures are abnormally high, the person is diagnosed as having high blood pressure (hypertension). The pressures in the lung arteries (pulmonary arteries) are normally significantly lower than the pressures in the systemic circulation. When pressure in the pulmonary circulation becomes abnormally elevated, it is referred to as pulmonary hypertension.
Although PPH is rare, an estimated 500 to 1,000 new cases are diagnosed annually. The disease can occur in men, women and children of all ages. However, it is most common in females between 20 and 40 years old, with twice as many cases reported in women than men, according to the National Heart, Lung and Blood Institute . The condition is rare in children but is sometimes seen in infants who are born with a heart defect (congenital heart disease). Secondary pulmonary hypertension (SPH), in which the high blood pressure is caused by another underlying condition, is much more common. Pulmonary Hypertension may be a primary or secondary cause of hypoxia in the neonate incidence of about 2-3 per million per year and a prevalence of about 15 per million
Primary pulmonary hypertension When an underlying cause for high blood pressure in the lungs can't be found, the condition is called primary pulmonary hypertension (PPH). But although the exact cause of PPH isn't known, scientists believe that most people who develop the disorder are especially sensitive to substances that cause the blood vessels to constrict. Cocaine and the diet drug fenfluramine (fen-phen), which was withdrawn from the market in 1997, are two of the substances that may contribute to PPH in some people. Other people with PPH have an inherited predisposition for the disease. In these people, pulmonary hypertension is triggered by another medical condition such as chronic liver disease (cirrhosis), AIDS, sickle cell anemia, scleroderma and lupus. Secondary hypertension Pulmonary hypertension resulting directly from another medical problem is called secondary pulmonary hypertension. Medical conditions that may lead to secondary pulmonary hypertension include: Blood clots in the lungs (pulmonary emboli). Chronic obstructive pulmonary diseases, such as emphysema. Connective tissue disorders, such as scleroderma. Sleep apnea. Congenital heart disease. . In primary pulmonary hypertension, the cause is not known, but likely begins with spasm (contraction) of the muscle layer in the pulmonary arteries. Secondary pulmonary hypertension can be caused by any disease that impedes the flow of blood through the lungs or that causes sustained periods of low oxygen in the blood. One of the most common causes is chronic obstructive pulmonary disease
Primary pulmonary hypertension When an underlying cause for high blood pressure in the lungs can't be found, the condition is called primary pulmonary hypertension (PPH). But although the exact cause of PPH isn't known, scientists believe that most people who develop the disorder are especially sensitive to substances that cause the blood vessels to constrict. Cocaine and the diet drug fenfluramine (fen-phen), which was withdrawn from the market in 1997, are two of the substances that may contribute to PPH in some people. Other people with PPH have an inherited predisposition for the disease. In these people, pulmonary hypertension is triggered by another medical condition such as chronic liver disease (cirrhosis), AIDS, sickle cell anemia, scleroderma and lupus. Secondary hypertension Pulmonary hypertension resulting directly from another medical problem is called secondary pulmonary hypertension. Medical conditions that may lead to secondary pulmonary hypertension include: Blood clots in the lungs (pulmonary emboli). Chronic obstructive pulmonary diseases, such as emphysema. Connective tissue disorders, such as scleroderma. Sleep apnea. Congenital heart disease. . In primary pulmonary hypertension, the cause is not known, but likely begins with spasm (contraction) of the muscle layer in the pulmonary arteries. Secondary pulmonary hypertension can be caused by any disease that impedes the flow of blood through the lungs or that causes sustained periods of low oxygen in the blood. One of the most common causes is chronic obstructive pulmonary disease
Pulmonary hypertension resulting directly from another medical problem is called secondary pulmonary hypertension. Medical conditions that may lead to secondary pulmonary hypertension include: Blood clots in the lungs (pulmonary emboli). Chronic obstructive pulmonary diseases, such as emphysema. Connective tissue disorders, such as scleroderma. Sleep apnea. Congenital heart disease. Lung diseases such as pulmonary fibrosis — a condition that causes scarring in the tissue between the lungs' air sacs (interstitium). In this case, pulmonary hypertension may occur when the lungs' smallest arteries and capillaries are compressed and obliterated by scar tissue. obesity with reduced ability to breathe (Pickwickian syndrome), neurologic diseases involving the respiratory muscles, chronic liver disease, HIV infection
Left-sided heart failure. When your heart's left ventricle weakens and can't pump out enough blood, the increase in pressure backs up through the pulmonary veins to the arteries in your lungs. High altitude. People living above an altitude of 8,000 feet may develop pulmonary hypertension as a result of low blood oxygen (hypoxemia), which constricts the small pulmonary arteries. Especially at risk are people who climb to high elevations without first becoming acclimated. Climbers unaccustomed to high altitudes are also at risk of pulmonary edema, a condition in which the air sacs in the lungs fill with fluid instead of with air and which is always associated with pulmonary hypertension.
Left-sided heart failure. When your heart's left ventricle weakens and can't pump out enough blood, the increase in pressure backs up through the pulmonary veins to the arteries in your lungs. High altitude. People living above an altitude of 8,000 feet may develop pulmonary hypertension as a result of low blood oxygen (hypoxemia), which constricts the small pulmonary arteries. Especially at risk are people who climb to high elevations without first becoming acclimated. Climbers unaccustomed to high altitudes are also at risk of pulmonary edema, a condition in which the air sacs in the lungs fill with fluid instead of with air and which is always associated with pulmonary hypertension.
If the pressure of the blood in the pulmonary arteries is abnormally high, the condition is called pulmonary hypertension. Over time, the increased pressure damages both the large and small pulmonary arteries. The walls of the smallest blood vessels thicken and are no longer able to transfer oxygen and carbon dioxide normally between the blood and the lungs. Thus, the levels of oxygen in the blood may fall. The low oxygen level can cause narrowing (constriction) of the pulmonary arteries. These changes contribute further to the increased pressure in the pulmonary circulation.
With pulmonary hypertension, the right side of the heart must work harder to push the blood through the pulmonary arteries into the lungs. Over time, the right ventricle becomes thickened and enlarged, leading to a condition called cor pulmonale. Heart failure develops (see Heart Failure). In some people, the bone marrow produces more red blood cells to compensate for less oxygen in the blood, leading to a condition called polycythemia (see Myeloproliferative Disorders: Polycythemia Vera). The extra red blood cells cause the blood to become thicker and stickier, further increasing the load on the heart. These changes also put a person with cor pulmonale at increased risk of pulmonary embolism (see Pulmonary Embolism (PE)), because the thickened blood may clump and form clots, mainly in the veins of the legs. These clots can dislodge and travel to the lungs.
Shortness of breath (dyspnea). Initially, you may notice that you're short of breath only when you exert yourself physically, but eventually you may be short of breath most of the time, even when you're at rest. Fatigue. Dizziness or fainting spells (syncope). Chest pressure or 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. Some people feel light-headed or fatigued upon exertion, and an angina-like chest pain is common. The person is likely to feel weak because body tissues are not receiving enough oxygen.. Swelling (edema), particularly of the legs, may occur because fluid may leak out of the veins and into the tissues, but this is usually a sign that cor pulmonale has developed.
Other symptoms, such as coughing and wheezing, are usually caused by the underlying lung disease For unknown reasons, some people with primary pulmonary hypertension develop achy joints, often years before the apparent onset of the disorder. Coughing (sometimes with blood) Distended neck veins Enlarged liver Swollen abdomen
For patients with suspected PH there are several initial steps that are commonly taken to confirm the diagnosis. The history of present illnesses, past medical history, family history and any past or present medications that the patient may have taken are first discussed between the patient and the healthcare team. A thorough physical examination will also take place. Other tests used in the assessment of a patient suspected of PH may include all, or some, of the following: After the physician gives the patient a physical examination and asks about the patient's medical history , a number of tests may be ordered to aid in the diagnosis of pulmonary hypertension. These include:
Based on the symptoms, a doctor may suspect pulmonary hypertension in people who have an underlying lung disorder. A chest x-ray may show that the pulmonary arteries are enlarged.
Electrocardiogram The electrocardiogram (ECG) is a record of the electrical activity produced by the heart. An abnormal ECG may indicate that the heart is undergoing unusual stress. In addition to the usual ECG performed while the patient is at rest, the doctor may order an exercise ECG. This ECG helps the doctor evaluate the performance of the heart during exercise, for example, walking a treadmill in the doctor's office. Electrocardiogram (EKG). A test that measures the heart’s electrical activity. It is designed to detect any abnormal rhythms (arrhythmias), which may be signs that the heart or parts of the heart are overly stressed. Pulmonary angiogram . A test used to measure circulation in the lungs and to visualize clots in the lung on x-rays . The test involves the insertion of a thin catheter into the pulmonary artery through which an iodine dye is injected. The physician can then see an image of any blood clots present in the lung, and track the circulation of blood through the lung's blood vessels.
Perfusion lung scan. This test uses small amounts of radioactive tracers (radioisotopes) to study blood flow (perfusion) in your lungs. The radioisotopes are attached to substances known as radiopharmaceuticals, which are injected into a vein in your arm. Immediately afterward, a special camera (gamma camera) takes pictures of blood flow in your lungs' blood vessels. A lung scan is generally used to determine whether blood clots may be causing symptoms of pulmonary hypertension. It's usually performed in conjunction with another test known as a ventilation scan. In this test, you inhale a small amount of radiopharmaceutical while a gamma camera records the movement of air into your lungs. The two-test combination is known as a ventilation-perfusion (V/Q) scan, and in its entirety usually takes less than an hour. Only small amounts of radioactivity are used, and a V/Q scan seldom causes any side effects or complications. Pulmonary function tests. These noninvasive tests measure how much air your lungs can hold and the airflow in and out of your lungs. They can also measure the amount of gases exchanged across the membrane between your lung wall and capillary membrane. An abnormality here may be the first indication of PPH. During the tests, you'll most likely be asked to blow into a simple instrument called a spirometer.
If the pressure of the blood in the pulmonary arteries is abnormally high, the condition is called pulmonary hypertension. Over time, the increased pressure damages both the large and small pulmonary arteries. The walls of the smallest blood vessels thicken and are no longer able to transfer oxygen and carbon dioxide normally between the blood and the lungs. Thus, the levels of oxygen in the blood may fall. The low oxygen level can cause narrowing (constriction) of the pulmonary arteries. These changes contribute further to the increased pressure in the pulmonary circulation.
If the pressure of the blood in the pulmonary arteries is abnormally high, the condition is called pulmonary hypertension. Over time, the increased pressure damages both the large and small pulmonary arteries. The walls of the smallest blood vessels thicken and are no longer able to transfer oxygen and carbon dioxide normally between the blood and the lungs. Thus, the levels of oxygen in the blood may fall. The low oxygen level can cause narrowing (constriction) of the pulmonary arteries. These changes contribute further to the increased pressure in the pulmonary circulation.
If the pressure of the blood in the pulmonary arteries is abnormally high, the condition is called pulmonary hypertension. Over time, the increased pressure damages both the large and small pulmonary arteries. The walls of the smallest blood vessels thicken and are no longer able to transfer oxygen and carbon dioxide normally between the blood and the lungs. Thus, the levels of oxygen in the blood may fall. The low oxygen level can cause narrowing (constriction) of the pulmonary arteries. These changes contribute further to the increased pressure in the pulmonary circulation.