Pulmonary Hypertension

Question 1

Define pulmonary hypertension.

Answer 1

Idiopathic pulmonary arterial hypertension (IPAH) is a disease of the small pulmonary arteries characterised by vascular proliferation and remodelling. It results in a progressive increase in pulmonary vascular resistance (PVR) and, ultimately, right ventricular failure and death.

Question 2

Explain the aetiology/risk factors of pulmonary hypertension.

Answer 2

Bone morphogenetic protein receptor type 2 (BMPR2) mutations
Appetite suppressants

Question 3

Summarise the epidemiology of pulmonary hypertension.

Answer 3

While pulmonary artery hypertension remains a rare disease, it is being increasingly recognised, with an estimated prevalence of 15.0 cases per million adult inhabitants and incidence of 2.4 cases per million adult inhabitants per year.

Question 4

Recognise the presenting symptoms of pulmonary hypertension.

Answer 4

SOB
Fatigue

Question 5

Recognise the signs of pulmonary hypertension on physical examination.

Answer 5

Peripheral oedema
Cyanosis
Accentuated pulmonic component (P2) to the second heart sound
Tricuspid regurgitation murmur

Question 6

Identify appropriate investigations for pulmonary hypertension and interpret the results.

Answer 6

CXR
ECG
Transthoracic Doppler
BNP
FBC
LFT