PULMONARY

HYPERTENSION
 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.

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.
 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.

PULMONARY HYPERTENSION is a far less common type of

high blood pressure that affects specifically the arteries in the
lungs. Pressures in the lung arteries are normally significantly
lower than the pressures in the systemic circulation. Pulmonary
hypertension occurs when the pressure in the pulmonary
circulation becomes abnormally elevated. It is a serious condition
that becomes progressively worse and eventually proves fatal.
 INTRODUCTION

An estimated of 500 – 1000 new cases are diagnosed annually.

There is an incidence of about 2-3 per million per year and a
prevalence of 15 per million.

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, with twice as many cases reported in women then men.

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.
 WHAT IS
PULMONARY
HYPERTENSION?
Ordinarily, blood vessels in the lungs provide less resistance to blood
flow than blood vessels in the rest of the body do. Hence, 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
Primary Pulmonary Hypertension
• no underlying cause for the high blood pressure in lungs
• likely to begin with spasm of the muscle layer in pulmonary arteries
• patients are rather sensitive to substances that cause blood vessels
to constrict
• may have an inherited predisposition for the disease

Secondary Pulmonary Hypertension

• results directly from another medical problem
• most probable from diseases that impedes flow of blood through
lungs or that causes periods of low oxygen in blood
• eg. Chronic Obstructive Pulmonary Disease, scleroderma, sleep
apnea, pulmonary fibrosis, lung diseases such as asbestosis
 Abnormally high BP in pulmonary arteries

Increased pressure damages large and small pulmonary arteries

Blood vessel walls thicken

Cannot transfer oxygen and carbon dioxide normally

Levels of oxygen in blood fall

Constriction of pulmonary arteries

Further increase in pressure in pulmonary circulation

 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 leading Polycythemia

Extra RBCs cause the blood to become thicker and stickier, further increasing
the load on the heart Pulmonary Embolism
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
 WHAT CAUSES
PULMONARY
HYPERTENSION?
the exact cause of primary pulmonary hypertension is unknown,
scientists believe that most people who develop the disorder are
especially sensitive to substances that cause blood vessels to
constrict.

Cocaine and fenfluramine (fen-phen), which was withdrawn from

the market in 1997, are two of the substances that may contribute
to PH in many people.

Other people with PH have an inherited predisposition for the

disease. In these people, PH is triggered by another medical
condition such as chronic liver disease (cirrhosis), AIDS, sickle
cell anemia, scleroderma and lupus.
Pulmonary hypertension resulting directly from another medical
problem is called secondary pulmonary hypertension. Medical
conditions that may lead to secondary PH include :

 blood clots in the lungs (pulmonary emboli)

 chronic obstructive pulmonary disease such as emphysema
 connective tissue disorders, such as scleroderma
 sleep apnea – upper airway obstructed during sleep
 congenital heart disease
 obesity with reduced ability to breathe (Pickwickian syndrome)
 neuromuscular diseases involving respiratory muscles
 HIV infection
 lung diseases such as pulmonary fibrosis (causes scarring in
the tissue between the lungs’ air sacs)
Left-sided heart failure
• heart’s left ventricle weakens and cannot pump out enough blood
• increase in pressure backs up through pulmonary veins to
arteries in lungs

High Altitude
• above altitude of 8000 feet - may develop PH
• low blood oxygen (hypoxemia)
• constricts small pulmonary arteries
• climb to high elevations without first becoming acclimated
• risk of pulmonary edema too – air sacs filled with fluid instead of
with air, always associated with PH
The overall rise in blood pressure in PH is the end result of a
process which begins with changes in the endothelial cells that line
the lungs’ arteries.

Changes → causes formation of extra tissue → blockage in vessels

Scarring (fibrosis) usually also occurs → arteries stiff and narrow

These causes increased resistance to blood flow which raises

pressure in the pulmonary arteries.

Less often, PH is caused by extensive loss of lung tissue from

surgery/trauma.
SIGNS AND
SYMPTOMS
 shortness of breath (dyspnea)
Initially, only short of breath when exert oneself physically but
eventually may be short of breath most of the time, even when
at rest.
 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)
 coughing (sometimes with blood) and wheezing
 distended neck veins
 enlarged liver
 racing pulse or heart palpitations
 angina-like chest pain
 feel weak – body tissues not receiving enough oxygen
 achy joints (often developed years before apparent onset of
disorder)
 Signs and Symptoms in Children
The symptoms of PH for children are similar to that of an adult,
though children are more likely to experience tiredness, dizziness
and breathlessness and for many, fainting is common.

 fail to put on weight like a normal child

 slowed growth
DIAGNOSIS OF PH
 Chest X-Ray

. A chest x-ray may show that the pulmonary arteries are enlarged.
the right side of the heart is enlarged.
 Echocardiography (Ultrasonic Cardiography)
This test uses sound waves to track the structure and function of the
heart. It can compose images of a beating heart on a monitor and
detects:
• heart’s thickness, size and function
• motion pattern and structure of the four heart valves
→ revealing any potential leakage (regurgitation)
• thickening of right ventricle, enlarged right heart
• reversal of blood through tricuspid valve
• extent of lung damage

A specific echocardiogram, Doppler ultrasound, is sometimes used to

determine pulmonary artery pressure.

Exercise echocardiogram – determine how well heart works under stress

 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.

Exercise ECG helps evaluation of performance of the heart during

exercise, for example, walking on a treadmill in the examination room.
 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.
 Perfusion Lung Scan

Uses small amounts of radioactive tracers (radioisotopes) to study blood

flow in the lungs. Radioisotopes are attached to radiopharmaceuticals
which are then injected into a vein the arm. A gamma camera takes
pictures of blood flow in the lungs’ blood vessels. It is generally used to
determine whether blood clots may be causing symptoms of PH.

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.
 Computerized Tomography (CT)

Organs can be scanned in two-dimensional ‘slices’. Split-second

computer processing creates images as a series of very thin x-ray beams
pass through the body. A contrast medium is used to help visualization.
The fast CT machine can scan arteries in less than 20 seconds as
opposed to 20 minutes for a standard CT. Speed is important because it
allows the dye to be visualized while still in the arteries.

Magnetic Resonance Imaging (MRI)

Uses no x-rays but instead, a computer creates tissue ‘slices’ from data
generated by a powerful magnetic field and radio waves. Although not yet
routinely used to diagnose PH, it is showing great value in assessing the
pulmonary arteries. It cannot, however, measure artery pressure.
 Other screening or diagnostic methods

 exercise testing
 ventilation-perfusion (V/O) scanning
 arterial blood gas studies
 central hematocrit count
 serum glucose and calcium levels count
 platelet count
 hyperoxia (100% oxygen) challenge test
TREATMENT OF PH
 Calcium Channel Blockers

 blocks entry of calcium into muscle cells of heart & arteries

 improve ability of heart to pump blood
 relaxes smooth muscle in walls of heart and blood vessels
 amlodipine (Norvasc), diltiazem (Cardizem, Tiazac),
nifedipine (Adalat, Procardia), nicardipine (Cardene) etc.
 only small number of people with PH respond to them
 side effects – constipation, nausea, headache, rash, edema,
drowsiness, dizziness, low blood pressure
 Blood Vessel Dilators

Prostacyclin

 substance that acts like a hormone (prostaglandin)

 imitates behaviour of natural prostacyclin
 powerful vasodilator and anti-clotting agent
 prevent blood clots from forming
 given intravenously through catheter
 bridge to help those waiting for transplant
 Epoprostenol (Flolan) - 1st vasodilator approved by FDA
 Ilopost - inhaled through nebulizer
 Treprostinil - injected under skin
 side effects - jaw pain, nausea, leg cramps etc
 need comprehensive follow-up care
 Endothelin Receptor Antagonists

 available in pill form

 reverse effect of endothelin (blood vessels constriction)
 Bosentan (Tracleer) - may improve stamina of people with PH
 not for pregnant women
 need monthly liver monitoring - risk of liver complications

Phosphodiesterase Inhibitors

 Revatio – contains sildenafil

 same active ingredient used in Viagra
 blocks the enzyme phosphodiesterase
 accentuates actions of nitric oxide
 opens blood vessels in the lungs - dilation
 side effect - vision problems
 Anticoagulant

 warfarin (Coumadin)
 prevent formation of blood clots within pulmonary arteries
 risk of bleeding complications – prevent normal blood coagulation
 periodic blood tests – check how well the drug is working
 more than 100 drugs can interact with anticoagulants

Diuretics

 water pills
 eliminate excess fluid from body
 reduces amount of work heart has to do
 limit fluid buildup in the lungs
 improve exchange of gases in lungs
 Oxygen

 oxygen therapy
 especially for those who live in high altitude
or have sleep apnea
 continuous use of oxygen through
nasal prongs/oxygen mask
 relieve shortness of breath

Cardiotonics

 strengthen the contractions of the heart

 heart does not need to beat as often to circulate adequate
blood for body
 Transplants

 surgical interventions – considered only in extreme cases

 treatment for severe secondary PH if treatment of the underlying
disorder fails
 surgically replace damage organs with healthy donated organs
 lung and/or heart transplantation
 most common : single-lung transplant, fewer complications than
double-lung or heart-lung transplant
 lung transplant - improvement in structure and functioning
of right ventricle
 major risks : rejection of transplanted organ, serious infection
 take immunosuppressant drugs for life – help reduce chance of
rejection
 survival rate is about 60% per year and 37% per 5 years
 Other treatment procedures

Dilation Atrial Septostomy

 experimental procedure
 use in patient with severe PH
 makes a small hole in the heart, slowly enlarging it to
relieve some of the pressure in the heart’s right side
 shunts blood across the atrial septum and into the left side
of the heart
 similar to balloon atrial septostomy – naturally occurring
hole present at birth is enlarged to help those with
congenital heart defects

Other areas of research for treatment of PH includes gene

therapy and stem cell research.