Pulmonary Hypertension

Question 1

Define pulmonary hypertension

Answer 1

Increased pulmonary arterial pressure (>20mmHg) at rest

Vascular proliferation and remodelling -> increased pulmonary vascular resistance

Question 2

What are the causes/risk factors of pulmonary hypertension?

Answer 2

Primary
• Idiopathic

Secondary
• Left heart disease e.g. mitral valve disease, left ventricular failure, left atrial myxoma
• Chronic lung disease e.g. COPD
• Recurrent pulmonary emboli
• Increased pulmonary blood flow e.g. ASD, VSD, patent ductus arteriosus
• Connective tissue disease e.g. SLE, scleroderma
• Drugs e.g. amiodarone

Question 3

What are the symptoms of pulmonary hypertension?

Answer 3

• SOB
• Peripheral oedema
• Fatigue
• Chest pain
• Syncope

Question 4

What are the signs of pulmonary hypertension?

Answer 4

• Cyanosis
• Raised JVP with prominent a wave
• Parasternal heave (RVH)
• Accentuated P2 (may hear S3/4)
• Tricuspid regurgitation – pansystolic murmur at left lower sternal edge, large cv wave
• Graham-Steell murmur – early diastolic murmur of pulmonary regurgitation

Question 5

What investigations are carried out for pulmonary hypertension?

Answer 5

• CXR - attenuated peripheral vascular markings (‘pruning’), enlarged pulmonary artery shadows, and opacification of the retrosternal space on the lateral view
• ECG - RVH, right axis deviation, right atrial enlargement
• transthoracic Doppler echocardiography - TR; estimated RVSP >35 mmHg; right ventricular and right atrial dilation; pericardial effusion
• right heart catheterisation - mean pulmonary arterial pressure >25 mmHg at rest or >30 mmHg with exercise, with pulmonary capillary wedge pressure ≤15 mmHg and pulmonary vascular resistance (PVR) >3 Wood units
• ANA- negative or low titre positive (≤1:80)
• PFTs - normal or mild reductions in lung volumes; mild reduction in diffusion capacity
• nocturnal oximetry ->10% of sleep time with oxygen saturation by pulse oximetry <90%
• ventilation-perfusion (V/Q) scintigraphy -normal or low probability
• 6-minute walk test
• B-type natriuretic peptide (BNP) or N-terminal proBNP (NT-proBNP) - elevated (>99 nanograms/L or >99 picograms/mL)
• full blood count - normal
• LFTs - normal
• thyroid function tests - normal
• HIV serology - negative