Pulmonary arterial hypertension (PAH) is abnormally high blood pressure in the arteries of the lungs. It can be caused by genetic factors or other conditions. PAH occurs when pressure in the pulmonary arteries is greater than 25 mmHg at rest or 30 mmHg with exercise. Diagnosis involves assessing medical history and tests like echocardiograms and heart catheterization. Treatment includes medications to dilate blood vessels, diuretics, oxygen therapy, and potentially lung transplantation for severe cases. The document provides details on the types, risk factors, symptoms, diagnosis, and treatment of PAH.

1. PULMONARY ARTERIAL
HYPERTENSION(PAH)
R. Anusha,
Pharm D intern
170514882007
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2. INTRODUCTION
 In the human body, there are two types of circulation that
enable distribution of blood through out 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.
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3.  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.
 Normal pulmonary artery pressure is 8-20 mm Hg at rest.
 Pulmonary hypertension is pressure in the pulmonary
artery that is greater than 25 mm Hg at rest or 30
mmHg during physical activity.
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5. 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)
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7. RISK FACTORS
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 Congestive heart failure
 Blood clots in the lungs
 Family history
 Lupus
 Scleroderma
 Emphysema
 Chronic bronchitis
 Pulmonary fibrosis
 Sleep apnea
 Cirrhosis
 HIV/AIDs
 Sex (young female)
 Drug induced

8. PATHOPHYSIOLOGY
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 It is high blood pressure (BP) in the arteries that go
from the heart to the lungs.
 Can be genetic or caused by another condition.
 Platelets, fibroblasts, and circulating cells are involved
in the progression of PAH
 The phenotypical change of pulmonary arterial smooth
muscle cells (PASMCs) and pulmonary arterial
endothelial cells (PAECs) results from multiple genetic
and acquired defects and is probably the major cause for
the onset of the disease.

9.  Increased PASMC contraction, increased PASMC
proliferation and inhibited PASMC apoptosis,
monoclonal PAEC proliferation, and endothelial injury
all are involved in the development of sustained
pulmonary vasoconstriction, lumen obliteration of small
pulmonary arteries with plexiform lesions, and
pulmonary vascular wall thickening due to medial
hypertrophy.
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12. FUNCTIONAL CLASSIFICATION
A. Class I - Patients with pulmonary hypertension but
without resulting limitation of physical activity.
Ordinary physical activity does not cause undue
dyspnea 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 dyspnea or fatigue, chest pain, or near
syncope.
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13. 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 dyspnea, 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. Dyspnea and /or fatigue may be present even at
rest. Discomfort is increased by any physical activity
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14. SIGNS AND SYMPTOMS
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15. DIAGNOSIS
 Hard to spot/diagnose because it mimics those of other
similar conditions.
 Will assess medical history and use one or more tests (ie.
blood test, chest X-ray, CT scan, MRI,
electrocardiogram, echocardiogram, heart
catheterization, pulmonary function test, lung biopsy)
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16. COMPLICATIONS
 Right-sided heart enlargement and heart failure
 Blood clots(Pulmonary Embolism)
 Arrhythmia
 Bleeding
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17. TREATMENT: NON-PHARMACOLOGIC
 Salt and volume management
 Supervised exercise (not too strenuous)
 Immunization - Specifically, influenza and
pneumococcal
 Improving diet
 Psycho-social support
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18. TREATMENT:PHARMACOLOGIC
 Fluid restriction
 Diuretics to decrease fluid accumulation
 Cardiac glycosides (eg, digitalis) in an attempt to
improve cardiac function
 Calcium channel blockers for vasodilation
 Intravenous prostacyclin helps to decrease pulmonary
hypertension by reducing pulmonary vascular resistance
and pressures and increasing cardiac output
 Anticoagulants such as warfarin have been given to
patients because of chronic pulmonary emboli.
 Long-term oxygen therapy is suggested to maintain
arterial blood O2 pressure at 60 mmHg
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19.  All: Anticoagulants ± Diuretics ± Digoxin ± Oxygen
 Acute vasoreactive testing (IPAH or APAH, ONLY)
 Positive: use high dose oral CCB (ie. nifedipine, diltiazem,
or amlodipine)
 Negative: depends on risk
• Low/High risk: Endothelin receptor antagonist (ERA) or
Phosphodiesterase Type 5 (PDE5) Inhibitors (oral),
[Prostanoids: epoprostenol (IV) or treprostinil (IV/SC),
Iloprost (inhaled)], Soluble guanylate cyclase (sGC)
stimulators
• Low or intermediate risk: monotherapy or oral combination
therapy
• High risk: combination therapy including IV prostacyclin
analogs
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21. Prostanoids:
 Epoprostenol (Flolan, Veletri): 2 ng/kg/min IV infusion every 15
min (initial)
 Treprostinil (Remodulin, Tyvasco):
• Remodulin (IV) = 1.25 ng/kg/min continuous SC or central line
IV infusion (initial)
• Tyvasco: QID inhalation
 Iloprost (Ventavis): 2.5 -5 mcg/inhalation 6- 9x/day
• Class AE: D/N/V (dose limiting), flushing, hypotension, anxiety,
chest pain, tachycardia, edema
• Chronic use: anxiety, flu-like symptoms, and jaw pain (lockjaw
with Iloprost)
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22. ERAs:
 Bosentan: 62.5 mg PO bid x 4 wks and then 125 mg bid
• BBW: hepatotoxicity and Cat X
• CI: cyclosporin and glyburide
• AE: HA, decrease Hgb, anemia, increase LFTs, upper
respiratory tract infections, edema, male infertility
• Monitor: LFTs
 Ambrisentan: 5 or 10 mg PO qd
• BBW: Cat X
• AE: peripheral edema, HA, decrease Hgb, flushing,
palpitations, congestion
• Monitor: Hgb and haematocrit 22

23. PDE-5 Is:
 Sildenafil (Revatio): 10 mg IV tid or 20 mg PO tid (4-6
hr apart)
 Tadalafil (Adcirca): 40 mg PO qd or 20 mg PO qd with
mild – moderate renal/hepatic impairment (avoid with
CrCl < 30 mL/min)
• CI: nitrates or sildenafil with PI based regimen, severe
hepatic impairment
• AE: dizziness, sudden drop in BP, HA, flush, dyspepsia,
back pain (Adcirca), and epistaxis
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24. sGC stimulant:
 Riociguat (Adempas): 1 mg PO TID (initial)
• Antacids decrease absorption and should not be taken
within 1 hr of taking medication
• BBW: Cat X
• CI: nitrates or NO donors, PDE inhibitors
• AE: HA, dyspepsia and gastritis, dizziness, N/D/V,
hypotension
• Monitor: BP (baseline and every 2 weeks)
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25.  If fail therapy, add another agent
 If fail that, lung transplant or atrial septostomy (Last
option)
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26. REFERNCES
 Pulmonary Arterial Hypertension,Gandharba Ray, L Ravi
Kumar,Pg:126-130.
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