Pulmonary Hypertension

Question 1

Defintion

Answer 1

Mean pulmonary arterial pressure (mPAP) greater than 20 mmHg at rest

Question 2

Epidemiology

Answer 2

Female

Question 3

Pulmonary arterial hypertension (PAH)
Aetiology

Answer 3

• Idiopathic
• Familial BMPR2 and EIF2AK4 mutations
• Connective tissue disorders or CHD
• Drugs/toxins
• Persistent pulmonary hypertension of new-born babies

Question 4

Pulmonary hypertension due to left heart disease Aetiology

Answer 4

• Heart failure
• Valvular heart disease
• Hypertrophic obstructive cardiomyopathy (HOCM)

Question 5

Pulmonary hypertension due to lung disease and/or hypoxia aetiology

Answer 5

• COPD
• Interstitial lung disease
• Obstructive sleep apnoea
• Chronic high altitude
• Chronic hypoxia (e.g. myasthenia)

Question 6

Pulmonary hypertension due to pulmonary artery obstruction aetiology

Answer 6

• Pulmonary embolism
• Pulmonary artery obstruction e.g. due to tumour or sickle cell disease

Question 7

Pulmonary hypertension with unclear and/or multifactorial mechanisms aetiology

Answer 7

• Haematological disorders
• Metabolic disorders
• Sarcoidosis

Question 8

Pathophysiology

Answer 8

In pulmonary hypertension:
The increased resistance in the pulmonary vasculature leads to increased pressure in the right ventricle
This results in increased ventricular filling and stroke volume, which further increases pulmonary arterial pressure
Over time, right ventricular hypertrophy develops

Question 9

Signs (SLRRP)

Answer 9

Right parasternal heave
Loud second heart sound
Pulmonary or tricuspid regurgitation
Raised JVP
Signs of underlying conditions

Question 10

Symptoms

Answer 10

Progressive breathlessness
Exertional dizziness/ syncope
Fatigue
Haemoptysis (chronic thromboembolic pulmonary HTN)

Question 11

Investigations

Answer 11

FIRST LINE = Echo
ECG =
- right-sided abnormalities such as P pulmonale, right axis-deviation,
right ventricular strain,
right bundle-branch block,
QTc prolongation
Chest imaging
Pulmonary function tests
GOLD STANDARD = Right heart catheterisation

Question 12

Management (FIRST LINE)

Answer 12

FIRST LINE = for patients who have idiopathic PAH and positive vasoreactivity test = CCB (NIFEDIPINE and DILTIAZEM)
Pulmonary vasodilators = for patients who are non-vasoreactive or not responding to CCB after 4 months
- Prostacyclin (prostaglandin I2)
Diuretics = for right ventricular failure + overload
O2 therapy = PO2 is consistently less than 60mmHg
Consider anticoagulation = warfarin or a novel oral anticoagulant (NOAC)

Question 13

Management (SECOND LINE)

Answer 13

Lung transplantation: can be considered if there is inadequate response to combination therapy with pulmonary vasodilators
Balloon atrial septostomy

Question 14

Complications

Answer 14

Right heart failure: due to increasing pulmonary pressures
Pericardial effusion and tamponade
Hepatic congestion: due to worsening right heart failure