In this SlideShare, we provide an overview on PAH, short for pulmonary arterial hypertension, a rare and progressive disorder caused by the obstruction in the smaller arteries within the lungs. This raises the blood pressure, forcing the heart to work harder. PAH is one of 5 groups classifications by the WHO. The slides briefly covers the symptoms, diagnosis and potential treatments for PAH patients, including those by Aria CV and Liquidia.

1. Overview of PAH: A Rare & Progressive Disorder
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Pulmonary Arterial Hypertension
1 September 2021. © Xeraya Capital.

2. Pulmonary Arterial Hypertension
Contents Overview
• What is PAH?
• Causes of PAH
• Common Symptoms &
Diagnosis
• Risk Factors & Prevalence
• Treatment of PAH Patients
• Aria CV’s PH System
• Liquidia’s LIQ861 Candidate
2 September 2021. © Xeraya Capital.

3. What is PAH?
Short for pulmonary arterial
hypertension, it is a rare and
progressive disorder that
occurs when smaller arteries
in the lungs become
thickened & narrowed.
This narrowing blocks the blood
flow, raising the blood pressure
and forces the heart to work
harder. Over time, PAH leads to
heart failure & death.
Credit: Shutterstock
3 September 2021. © Xeraya Capital.
The right ventricle of the heart (that passes
blood to the lungs to pick up oxygen) ends up
becoming enlarged, while the left ventricle
suffers regurgitation.
Source: https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-
arterial-hypertension/learn-about-pulmonary-arterial-hypertension

4. Cause of PAH
The exact cause of PAH is
currently unknown, as small
arteries in the lungs can be
obstructed by a variety of
reasons.
Researchers believe PAH occurs
when there is an injury to the cells
that line the blood vessels of the
lung.
Source: https://www.mayoclinic.org/diseases-conditions/pulmonary-hypertension/
symptoms-causes/syc-20350697
Credit: MAYO Foundation
4 September 2021. © Xeraya Capital.

5. WHO Classifications of Pulmonary Hypertension (PH)
Source: https://phassociation.org/types-pulmonary-hypertension-groups/
5 September 2021. © Xeraya Capital.
Group 1 Group 2 Group 3 Group 4 Group 5
Refers to
pulmonary arterial
hypertension (PAH),
which is caused
when the arteries in
the lungs become
narrowed,
thickened or stiff.
Includes PH due to
left heart disease.
In this group of PH,
there are problems
with how the heart
squeezes or
relaxes, or
problems with the
valves on the left
side of the heart.
Includes PH due to
chronic lung
disease and/or
hypoxia (low
oxygen levels).
These lung
diseases include
obstructive lung
disease, restrictive
lung disease, sleep
apnea; and living in
high altitude area.
Chronic
thromboembolic
pulmonary
hypertension
(CTEPH), occurs
when the body is
not able to dissolve
a blood clot in the
lungs, leading to
scar tissue in the
blood vessels.
This is where PH is
regarded as
secondary to other
diseases in ways
that are not well
understood.

6. Symptoms & Diagnosis
The Challenge of Properly Identifying PAH
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7. Common Symptoms of PAH
Source: https://www.lung.org/lung-health-diseases/lung-disease-lookup/
pulmonary-arterial-hypertension/symptoms-diagnosis
7 September 2021. © Xeraya Capital.
Shortness
of Breath
Fatigue
Edema
Dizziness
& Fainting
Spells
Chest
Pain
Blue Lips
& Fingers
*Other common lung diseases exhibit
similar symptoms, making it
challenging to properly diagnose PAH.

8. Diagnosing PAH
Source: https://www.lung.org/lung-health-diseases/lung-disease-lookup/
pulmonary-arterial-hypertension/symptoms-diagnosis
8 September 2021. © Xeraya Capital.
Blood Tests
HIV, thyroid, autoimmune,
liver
Electrocardiogram
Measure electrical activity
of the heart
Lung Function Test
Detect asthma,
emphysema
6-Minute Walk Test
How far patient can walk
before oxygen levels drop
Echocardiogram
Ultrasound of the heart to
check for abnormalities
Right Heart Catheterization
Invasive procedure into the
pulmonary artery

9. Risk Factors & Prevalence
Known Conditions & Occurrences
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10. Known Conditions Associated With PAH
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10
Connective tissue disorders
Exposure to toxins & drugs
Infections (HIV, worms)
Cirrhosis of liver
Congenital heart abnormalities
Source: https://www.everydayhealth.com/pulmonary-arterial-hypertension/

11. Prevalence & Occurrence of PAH
September 2021. © Xeraya Capital.
11
Inconclusive
Data on PAH
- Reporting bias due to
better healthcare in
developed countries
- PAH patients can go
years without
diagnosis
- Approximately 15-20%
of patients have
heritable forms of PAH
- NORD
PAH is 3-5x More
Frequent in Women
Ages 30-60 are
more susceptible.
Approximately
500-1,000 New
Cases Annually
Estimated 1-2
persons per million
in the U.S.
Prevalence Range:
15-25 per million
people
Within the U.S and
Europe.
Source: https://rarediseases.org/rare-diseases/pulmonary-arterial-hypertension/

12. Treatment Options
And Potential Candidates
12 September 2021. © Xeraya Capital.

13. Current Treatment Options
Source: https://www.lung.org/lung-health-diseases/lung-disease-lookup/
pulmonary-arterial-hypertension/treating-and-managing
13 September 2021. © Xeraya Capital.
Oral &
Inhaled
Treatments
Intravenous
Portable
Infusion
Pumps
Pulmonary
Rehab
Lung
Transplant
(Severe)
*Until now, there is no specific cure for
PAH. There are medications &
procedures to slow the progression.

14. Aria CV: Pulmonary Hypertension (PH) System
The PH System is a novel
device that can be
implanted into a patient to
mimic the function of
healthy vessels.
The device works by inserting a
gas-filled balloon into the main
pulmonary artery that is
connected by a catheter to a
hollow reservoir.
Credit: Aria CV
14 September 2021. © Xeraya Capital.
The PH System enables the patient’s heart to
pump more blood for less energy. It has been
granted 'Breakthrough Device Designation' by
the FDA.
Source: https://www.investopedia.com/articles/personal-finance/102015/series-
b-c-funding-what-it-all-means-and-how-it-works.asp

15. Liquidia: LIQ861 Treprostinil Inhalation Powder
LIQ861 is an inhaled dry
powder formulation of
treprostinil that is undergoing
Phase 3 trials with the FDA.
Treprostinil is a vasodilation type
drug that widens the blood vessels.
Liquidia’s proprietary PRINT®
Technology enables the
manufacturing of highly uniform
particles, enhancing drug delivery
deep into the patient’s lungs.
Credit: Liquidia
15 September 2021. © Xeraya Capital.
LIQ861 offers a more potent (and effective) dose
of treprostinil compared to currently available
inhaled therapies.
Source: https://www.investopedia.com/articles/personal-finance/102015/series-
b-c-funding-what-it-all-means-and-how-it-works.asp

16. • Pulmonary arterial hypertension (PAH) is a rare, progressive
disorder (meaning it gets worse over time), in which the cause
is usually unknown. It is most common in women between the
ages of 30-60.
• There is no specific cure for PAH, but treatments are available
to control symptoms and improve quality of life.
• Aria CV is developing a novel implantable device that would
allow the heart to pump more blood for less energy, it has
received Breakthrough Device Designation from the FDA.
• Liquidia's inhaled dry powder formulation of treprostinil
(LIQ861) helps to widen the blood vessels and is currently in
Phase 3 of clinical trials.
Conclusion
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17. By xeraya capital
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17 September 2021. © Xeraya Capital.