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.
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.
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.
WHAT IS PULMONARY
HYPERTENSION?

PH is an elevation in the mean pulmonary

artery pressure greater than 25 mmHg at
rest. (Egan’s 10th ed.)
PH is an elevation in the mean pulmonary
artery pressure greater than 25 mmHg at
rest or 30 mmHg during exercise. (Egan’s 8th
and 9th ed.)
 Clinical Classification of Pulmonary Hypertension (Dana Point,
2008)
Group 1 Pulmonary Arterial Hypertension (PAH)
a. Idiopathic (IPAH)
b. Heritable (HPAH)
• Bone morphogenetic protein receptor type 2 (BMPR2)
• Activin receptor-like kinase gene
c. Drug- and toxin-induced: fenfluramin
d. Associated with (APAH)
• Connective tissue diseases
• HIV infection
• Portal hypertension
• Congenital heart disease (CHD)
• Schistosomiasis
• Chronic haemolytic anemia
e. Persistent PH of the newborn (PPHN)
Group 2 PH due to left heart diseases
a. Systolic dysfunction
b. Diastolic dysfunction
c. Valvular disease
Group 3 PH due to lung diseases and/or hypoxemia
a. COPD
b. ILD
c. Other pulmonary diseases with mixed restrictive and obstructive
pattern
d. Sleep-disordered breathing
e. Alveolar Hypoventilation Disorders
f. Chronic exposure to high altitude
g. Developmental abnormalities

Group 4 Chronic thromboembolic PH (CTEPH)

Group 5 PH with unclear multifactorial mechanisms
a. Hematologic al disorders, spenectomy
b. Systemic disorders:sarcoidosis, neurofibromatosis
c. Metabolic disorders: glycogen storage disease, thyroid disorders
d. Others: tumoral obstruction, fibrosing mediastinitis
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
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.
PATHOGENESIS
• Genetic predisposition
• Damage to the endothelium after balance between:
 Vasoconstrictive mediators
• thromboxane
• endothelin
 Vasodilators
• nitric oxide
• prostacyclin
 Vasoconstriction results
• Inflammation
• thrombosis
• cell proliferation
• apoptosis
• fibrosis
Most recent research suggests the
presence of other potential airways
involved in the pathogenesis of PAH:
• Down regulation of potassium channels
• Increase matrix metalloproteinases
• Decrease vasoactive intestinal peptide
• Elevated serotonin
• Transforming growth factor beta
EPIDEMIOLOGY AND
CLINICAL FINDINGS
 Prevalence
• IPAH is more common in women than among
men, with a ratio of 3:1.
 Prevalence
• Approximately 7% of all cases are
heritable.
• Can occur at any age, although it is more
common from ages 20 – 50 years old.
 Epidemiology
• On average, the diagnosis of IPAH is
delayed for 2 years after the onset.

• The condition is frequently misdiagnosed

as anxiety or depression because it is
characterized by onset of vague
symptoms and hyperventilation.
 Symptoms
• Dyspnea – most common initial symptom
• Angina - caused by under perfusion of the
right ventricle or stretching of the large
pulmonary arteries
• Syncope - early symptom
• Other symptoms:
– Cough, hemoptysis, hoarseness, and Raynaud
phenomenon
 Physical Findings
• Loud second heart sound
• Right-sided third or fourth heart sound
• Signs of right ventricular failure are
common
• Cyanosis often is present as a result of
low cardiac output or the presence of an
intracardiac right-to-left shunt that
occurs as cor pulmonale develops
 Physical Findings
• Other common signs are:
– A palpable right ventricular heave
– An impulse of the pulmonary artery
and both pulmonary ejection
– Pulmonary tricuspid regurgitation
murmurs
 Chest Radiograph Findings
• Enlargement of the main and hilar
pulmonary arteries
• “Pruning” (or narrowing) of the
peripheral arteries
• Enlargement of the right ventricle and
atrium
• Pleural effusion
 Pulmonary Venoocclusive Disease
• It is one of the less commonly encountered
causes of pulmonary hypertension
• Some reports suggest that PVOD accounts for 5-
20% of cases classified as idiopathic pulmonary
arterial hypertension 
• It is the occlusion or narrowing of the
pulmonary veins and venules by sometimes
loose, sometimes more dense and collagen-rich,
fibrous tissue, leading to clinical manifestations
that are, in many ways, similar to IPAH
Pulmonary Venoocclusive Disease
• There is an increase in vascular markings in
the chest Xray
• Kerley B lines may be present on the chest
radiograph
DIAGNOSIS OF PH
DIAGNOSIS
• Tests commonly ordered to establish the
diagnosis include:
- Blood tests
- ECG
- Pulmonary function testing
- Echocardiogram
- V­/Q­scan
- Pulmonary artery catheterization
1. Blood tests

-Laboratory tests include complete

blood count, HIV, rheumatologic panel
and liver function testing.
-These tests help identify conditions
associated with PAH
2. ECG

-Traces the heart's activity and can

show whether the right side of the
heart is under strain or enlarged.
That's a warning sign of pulmonary
hypertension.
3. Pulmonary function testing

- measures 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
useful to rule out the presence of
significant restrictive or obstructive airway
disease
 
4. Echocardiogram

- uses sound waves to create a

moving picture of your heart
show dilation of the right ventricle
and right atrium and tricuspid
regurgitation (leaking heart valves)
5. V/Q scan

- One of the most important non invasive

tests for IPAH
- 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.
6. Right heart catheterization

- required to confirm the diagnosis and determine

the degree of hemodynamic impairment
(functioning in the mechanics of blood
circulation) and presence of vasoreactivity.

- measures the pressure in the pulmonary

arteries and right ventricle

- shows how well the heart is pumping blood to

the rest of the body
 References
Egan’s 8th, 9th, and 10th edition

http://www.nhlbi.nih.gov/healthtopic/diagnosis

http://www.webmd.com/lung/hypertension

http://
www.nhlbi.nih.gov/condition/pulmonary-arteri
al-pressure

http://www.emedicine.medscape.com/articles
/PMC2853883