Pulmonary hypertension

Question 1

Pulmonary HTN: definition

Answer 1

increase in mean pulmonary arterial pressure (normally around 15mmHg) which is associated with a number of conditions.

Question 2

Pulmonary HTN: features

Answer 2

SOB, fatigue, syncope

Raised JVP
parasternal heave, loud P2, presence of an S3 sound
R-sided murmurs –> pansystolic murmur (tricuspid regurgitation) and an end-diastolic murmur (pulmonary regurgitation).

Question 3

Causes of pulmonary hypertension:

Answer 3

1. Parenchymal lung disease: COPD, Chronic Asthma, Interstitial lung disease, bronchiectasis, cystic fibrosis
2. Pulmonary vascular disease: Idiopathic pulmonary hypertension, pulmonary vasculitis, pulmonary embolism, portal hypertension
3. Hypoventilation: Sleep apnoea, kyphosis/scoliosis, neuromuscular conditions (e.g. myasthenia gravis)
4. Left Heart Disease: Mitral stenosis, mitral regurgitation, left ventricular failure

Question 4

Pulmonary hypertension: investigations

Answer 4

ECG: P pulmonale, right ventricular hypertrophy, right axis deviation
Echocardiogram
GOLD STANDARD - Right heart catheterisation shows mean pulmonary artery pressure >25mmHg

Question 5

Pulmonary hypertension: treatment

Answer 5

1. Treat underlying condition
2. Reduction of pulmonary vascular resistance: Long term oxygen therapy, Nifedipine, Sildenafil, Prostacycline analogues, Bosentan (endothelin receptor antagonist)
3. Manage heart failure
4. Heart-lung transplant for selected cases