Pulmonary Hypertension

Question 1

What is the diagnostic definition of Pulmonary Hypertension?

Answer 1

Resting mean Pulmonary Artery Pressure at or above 25mmHg (normal 18-25) - gold-standard measure : right heart catheterisation.

Question 2

Pathophysiology of Pulmonary Hypertension.

Answer 2

Increased resistance and pressure of blood in the pulmonary arteries.

Question 3

Aetiology of Pulmonary Hypertension (5).

Answer 3

1. Primary Pulmonary Hypertension or Connective Tissue Disease (e.g. SLE).
2. Left Heart Failure (e.g. MI, HTN).
3. Chronic Lung Disease (e.g. COPD).
4. Pulmonary Vascular Disease (e.g. PE).
5. Miscellaneous e.g. Haematological Disorders, Glycogen Storage Disease, Sarcoidosis.

Question 4

Clinical Presentation of Pulmonary Hypertension (3).

Answer 4

1. Shortness of Breath.
2. Syncope, Tachycardia.
3. HF : Raised JVP, Hepatomegaly, Peripheral Oedema.

Question 5

ECG Changes in Pulmonary Hypertension (3).

Answer 5

Right-Sided Strain :

1. RV Hypertrophy - Larger R waves on Right-Sided Leads (V1-V3) and S waves on Left-Sided Leads (V4-V6).
2. Right Axis Deviation.
3. RBBB.
4. P-Pulmonale.

Question 6

CXR Changes in Pulmonary Hypertension (2).

Answer 6

1. Dilated Pulmonary Arteries.

2. Right Ventricular Hypertrophy.

Question 7

Other Investigations in Pulmonary Hypertension.

Answer 7

1. Echo - Estimate Pulmonary Artery Pressure.
2. Raised NT-pro-BNP : RV Failure.
3. Right Heart Catheterisation (Gold-Standard).

Question 8

Management of Primary Pulmonary Hypertension (3).

Answer 8

1. IV Prostanoids e.g. Epoprostenol.
2. Endothelin Receptor Antagonists e.g. Macitentan, Bosnian.
3. PDE-5 Inhibitors e.g. Sildenafil.

Question 9

Management of Secondary Pulmonary Hypertension.

Answer 9

Treatment of underlying cause e.g. PE, SLE.

Question 10

Prognosis of Pulmonary Hypertension.

Answer 10

30-40% 5 Year Survival (60-70% with specific treatment).