Pulmonary hypertension is defined by a mean pulmonary arterial pressure above 25 mmHg. It can arise from various mechanisms, including hypoxic vasoconstriction, left heart disease, lung disease, and chronic thromboembolic events, with various classification types and symptoms such as dyspnea and fatigue. Diagnosis involves imaging and catheterization, and treatment options include medications like CCBs and prostanoids, or lung transplantation in severe cases.

1. Pulmonary hypertension
made easy
Dr/Waseem Omar, MD
Lecturer of Cardiology,
Al-Azhar University

2. Definition
• Mean pulmonary arterial pressure >25 mmHg,
or a systolic pulmonary artery pressure of >40
mmHg at rest.

3. Methods of measurement
• Invasive: pulmonary artery catheters (i.e.
Swan-Ganz catheter).
• Non invasive: assessment of TR velocity by
continuous Doppler by echo (4 X TV2+ RAP).

4. Mechanisms of Pulmonary Hypertension
• Hypoxic vasoconstriction: chronic hypoxia causes pulmonary
vasoconstriction.
causes: COPD, chronic alveolar hypoxia.
• Decreased area of pulmonary vascular bed:
causes: collagen vascular disease, HIV infection, drugs and toxins,
thrombotic or embolic disease, inflammatory, interstitial fibrosis,
• Volume and pressure overload
pulmonary artery pressure will not rise in otherwise normal lung until
pulmonary blood flow exceeds 2.5x the basal rate
causes: congenital systemic-to-pulmonary shunts (e.g. VSD, ASD,
PDA), portopulmonary HTN, left-sided heart conditions,

5. Classification
I: Pulmonary Arterial HTN:
• Idiopathic
• Collagen vascular disease (scleroderma, SLE, RA)
• Congenital systemic-to-pulmonary shunts (Eisenmenger
syndrome)
• Portopulmonary HTN
• HIV infection
• Drugs and toxins (e.g. anorexigens)
• Pulmonary veno-occlusive disease
• Schistosomiasis
• Sickle cell disease.

6. II: Pulmonary HTN due to Left Heart Disease
• Left-sided atrial or ventricular heart disease
(e.g. LV dysfunction)
• Left-sided valvular heart disease (e.g. aortic
stenosis, mitral stenosis)

7. III: Pulmonary HTN due to Lung Disease and/or
Hypoxia.
• Parenchymal lung disease (COPD, interstitial
fibrosis, cystic fibrosis)
• Chronic alveolar hypoxia (chronic high
altitude, alveolar hypoventilation disorders,
sleep-disordered breathing)

8. IV: Chronic Thromboembolic Pulmonary HTN
(CTEPH)
V: Pulmonary HTN with Unclear Multifactorial
Mechanisms
• Hematologic disorders
• Systemic disorders (e.g. sarcoidosis)
• Metabolic disorders
• Extrinsic compression of central pulmonary veins

9. Symptoms
• Symptoms of systemic congestion
• Dyspnea
• Fatigue
• Retrosternal chest pain
• Syncope
• Symptoms of underlying disease

10. Signs
• Loud, palpable P2
• RV heave
• Right-sided S4 (due to RVH)
• Systolic murmur (tricuspid regurgitation)
• If RV failure: right sided S3, increased JVP,
positive HJR, peripheral edema,

11. Investigations
• CXR: enlarged central pulmonary arteries and attenuation of peripheral Vs
(bruning), cardiac changes due to RV enlargement (filling of retrosternal air space).
• ECG: RVH/right-sided strain.
• 2-D echo doppler: assessment of right ventricular systolic pressure.
• Cardiac catheterization: direct measurement of pulmonary artery pressures
(necessary to confirm diagnosis).
• PFTs: for assessmnet of underlying lung disease:
• CT angiogram: to assess lung parenchyma and possible PE.
• Serology: ANA positive in 30% of patients with primary pulmonary HTN;

12. General rules
• Mild physical activity
• Routine influenza and pneumococcal vaccine.
• Digoxin
• Anticoagulant
• Avoid high altitude.
• Oxygen therapy to maintain SO2 ≥ 90%.
• Diuretics.
• Contraception as PHT increase mortality by 30-50%.

13. Specific treatment
I:
• No effective treatment
• CCBs.
• prostanoids,
• Endothelin receptor antagonists,
• PDE5 inhibitors
• Lung transplantation