Pulmonary hypertension is abnormal elevation of pulmonary artery pressure that can be caused by several factors. It occurs when the arteries in the lungs become narrowed, blocking blood flow and overworking the heart. There are five groups of pulmonary hypertension distinguished by their causes, which include left heart disease, lung diseases that cause hypoxia, chronic thromboembolic pulmonary hypertension, rare diseases with unclear causes, and pulmonary arterial hypertension. The latter involves thickening of pulmonary arteries and is the most severe form, sometimes caused by genetic mutations. Symptoms include shortness of breath and right heart failure over time if left untreated.

1. Pulmonary Hypertension
By: Nirjhari Makwana

2. Pulmonary Circulation
Low Pressure System
Systemic - 120/80
Pulmonary Artery - 24/12
Pulmonary artery has very thin walls
• Little smooth muscles
• Low resistance to flow
• Very distensible

3. Resistance to blood flow as blood moves to the lungs.
Two Types of Vessels
1. Alveolar Vessels - Capillaries ( surrounds alveoli )
2. Extra Alveolar Vessels - Arteries & Veins ( supplies to
capillaries )
Increased Lung Volume
• Crushes Alveolar Vessels —> High Resistance
• Pulls Extra Alveolar Vessels open —> Low Resistance
Resistance goes high & low while inhalation &
exhalation during respiratory cycle.
Pulmonary Vascular Resistance

5. • Pulmonary Hypertension is an abnormal elevation
in Pulmonary Artery Pressure ( PAP )
Normal PA Pressure
24/12
Mean 10-14 mmHg
Pulmonary Hypertension
Mean Pressure >25 mmHg
Loud / Accentuated P2 Sound - at upper sterna
border.
PAP & PVR Progressively rises, leading to Right Heart
Failure & Death.
Pulmonary
Hypertension

6. Pulmonary hypertension (PH) may be post-capillary, a result of an increase in
pulmonary venous pressure in left-sided heart diseases, or pre-capillary, caused
by pulmonary vascular remodelling leading to increased pulmonary vascular
resistance.

8. Group:
1
Pulmonary arterial hypertension (PAH) is a specific type
of pulmonary hypertension that is caused when the tiny
arteries in your lung become thickened and narrowed.
This blocks the blood flow through your lungs which raises
the blood pressure in the lungs and causes your heart to
work harder to pump your blood through those narrowed
arteries.
Over time, your heart loses the ability to effectively pump
blood throughout your body.
Idiopathic
Affects young women
High Pulmonary Vascular Resistance
Increased activity by Vasoconstrictors - Endothelin.
Decreased Activity by Vasodilators - Nitric Oxide.
Pulmonary Arterial Hypertension

9. Plexiform Lesions
Unique to Idiopathic PAH.
Endothelial proliferation forms
multiple lesions.
Small arteries branch points
from medium arteries.

10. BMPR2 Mutation
Bone morphogenetic protein receptor
type II
• Inhibits smooth muscle proliferation.
• Mutations —> abnormal growth (
endothelium, smooth muscle )
25% - idiopathic cases
80% - familial cases
Others : activin receptor kinase-like1
(ALK1), Endoglin (ENG), Smad8
Heritable

11. Associated with:
• Connective Tissue Disease
• Human immunodeficiency virus
(HIV) infection
• Portal hypertension
• Congenital Heart Disease
• Schistosomiasis
• Drugs - Amphetamines, Cocaine

12. • Epoprostenol: Prostacyclin (IV)
PGI2
Potentvasodilator
• Bosentan:
Antagonist endothelin-1 receptors (PO)
• Sildenafil:
Inhibits PDE-5 in smooth muscle of lungs
Treatment

14. Group:2
Elevation in PAP with elevation in PCWP
includes:
Left Ventricular Systolic Dysfunction
Left Ventricular Diastolic Dysfunction
Valvular Disease
Congenital/ acquired left heart inflow/outflow
tract obstruction & congenital
cardiomyopathies.
Congenital / acquired pulmonary vein stenosis.
Pulmonary Venous Hypertension

15. Group:3
• Includes medial hypertrophy and intimal
obstructive proliferation of distal pulmonary
arteries.
• A variable degree of obstruction of vascular
bed in emphysematous or fibrotic areas may
by present.
• Chronic obstructive pulmonary disease
• Interstitial lung disease
• Sleep disordered breathing, alveolar
hypoventilation disorders
• Hornier exposure to high altitude
Pulmonary Hypertension due to Lung Disease or
Hypoxia

16. Group:4
• Characterized by organised thrombi
tightly attached to medial layer in
pulmonary arteries, replacing the
normal intima.
• These may completely occlude the
lumen or form different grade of
stenosis, webs & bands.
• Angiosarcoma, congenital pulmonary
arteries stenoses
Chronic Thromboembolic Pulmonary
Hypertension

17. Group:5
PH with Unclear &/or Multifactorial Mechanisms

18. Symptoms of PH

20. Clinical Presentation
• The physical signs of PH include, left parasternal lift,
• an accentuated pulmonary component of the second heart sound,
• an RV third heart sound,
• a pan-systolic murmur of tricuspid regurgitation and,
• a diastolic murmur of pulmonary regurgitation.
• Elevated jugular venous pressure,
• hepatomegaly, ascites,
• peripheral oedema and
• cool extremities characterise patients with advanced disease.
• Wheeze and crackles are usually absent

22. Functional classification of PH according to WHO

23. Chest Radiograph
• In 90% of patients with IPAH the chest radiograph is abnormal at the time
of diagnoses
• patients with PAH include central pulmonary arterial dilatation, which
contrasts with ‘pruning’ (loss) of the peripheral blood vessels.
• Right atrium (RA) and RV enlargement may be seen in more advanced
cases

24. Pulmonary function tests and arterial blood
gases
• Pulmonary function tests and arterial blood gases identify the contribution
of underlying airway or parenchymal lung disease. Patients with PAH have
usually mild to moderate reduction of lung volumes related to disease
severity
• The prevalence of nocturnal hypoxaemia and central sleep ap- noeas are
high in PAH (70 – 80%).

25. • Echo is the most important screening tool for
determining the possibility of PH
• Transthoracic echocardiography is used to
image the effects of PH on the heart and
estimate PAP from continuous wave Doppler
measurement
• Evaluating RV function, including right atrial
area and RV ejection fraction (RVEF)
• Transthoracic Doppler echo can detect chronic
thromboembolic PH (CTEPH) in patients who
have had acute pulmonary embolism
Echocardiography

26. EPIDEMIOLOGY
• Pulmonary hypertension is a major
global health issue
• All age groups are affected
• Present estimates suggest a PH
prevalence of ∼1% of the global
population
• higher in individuals aged >65 years
• In UK, prevalence has doubled in the
last 10 years and is currently 125
cases/million inhabitants