Pulmonary Hypertension

Question 1

What is the definition of pulmonary hypertension?

Answer 1

An mPAP of more than 25mmHg at rest, as measured on right heart catheterisation.

Question 2

What is the difference between primary and secondary pulmonary hypertension?

Answer 2

Primary: disease of the pulmonary arteries/arterioles
Secondary: occurs due to underlying disease or known risk factors, most commonly heart disease and lung fibrosis

Question 3

What are some symptoms of pulmonary hypertension?

Answer 3

Dyspnoea
Fatigue
Weakness
Angina
Syncope
Abdominal distension

Question 4

What are some signs of pulmonary hypertension?

Answer 4

Left parasternal heave
Loud P2 heart sound
Soft pansystolic murmur with tricuspid regurgitation
Early diastolic murmur with pulmonary regurgitation

Question 5

What are some investigations for pulmonary hypertension?

Answer 5

Routine bloods: FBC, renal and liver biochemistry, TFTs, serological assays

CXR: shows enlargement of pulmonary arteries and major branches, may be right atrial and left ventricular enlargement

ECG: P pulmonale, right ventricular hypertrophy, right axis deviation

Echocardiogram: to determine PAP

Right heart catheterisation: shows mPAP >25mmHg (GOLD STANDARD)

Question 6

What is the management of pulmonary hypertension?

Answer 6

Treat underlying condition
Reduction of pulmonary vascular resistance: long term oxygen therapy, nifedipine, sildenafil, prostacyclin analogues, bosentan
Manage heart failure
Heart-lung transplant for selected cases