Pulmonary hypertension
DEFINITION
A consistently increased pulmonary arterial pressure (>20 mmHg) under resting conditions.
AETIOLOGY
Primary:
Idiopathic.
Secondary:
Left heart disease (mitral valve disease, left ventricular failure, left atrial myxoma/ thrombosis), chronic lung disease (COPD), recurrent pulmonary emboli, " pulmonary blood flow (ASD, VSD, patent ductus arteriosus), connective tissue disease (e.g. SLE, systemic sclerosis), drugs (e.g. amiodarone).
EPIDEMIOLOGY
Primary pulmonary hypertension is usually seen in young females.
HISTORY Dyspnoea (on exertion), chest pain, syncope, tiredness.
Symptoms of the underlying cause (e.g. chronic cough).
EXAMINATION
Elevated JVP (Prominent a wave in the JVP waveform).
Palpation:
Left parasternal heave (right ventricular hypertrophy).
Auscultation:
Loud pulmonary component of S2 (S3/S4 may be heard), an early diastolic murmur (Graham–Steell murmur) caused by pulmonary regurgitation may be present, if tricuspid regurgitation develops (large cv wave and pansystolic murmur).
Signs of the underlying condition, or right heart failure in severe cases.
INVESTIGATIONS
CXR:
Cardiomegaly (right ventricular enlargement, right atrial dilation), prominent main pulmonary arteries (which taper rapidly), signs of the cause (e.g. COPD, calcified mitral valves).
ECG:
Right ventricular hypertrophy (right-axis deviation, prominent R wave in V1, T inversion in V1, V2), right atrial enlargement (peaked P wave in II, called ‘P pulmonale’); limb leads exhibit low voltage (R < 5 mm) in COPD.
Echocardiography:
To visualize right ventricular hypertrophy or dilation and possible
underlying cause.
Lung function tests:
To assess for chronic lung disease.
VQ scan:
To assess for pulmonary embolism.
Cardiac catheterization:
To assess severity, right heart pressures and response to vasodilators.
High resolution
CT-thorax:
Images pulmonary arteries and to diagnose lung disease.
Lung biopsy:
Assesses structural lung changes.
MANAGEMENT
Medical:
Treat secondary cause. For primary pulmonary hypertension, consider:
- . anticoagulation (warfarin);
- .calcium channel antagonists (e.g. verapamil, nifedipine);
- . prostacycline analogues (e.g. iloprost);
- . endothelial receptor antagonist (bosentan) blocks vasoconstriction, but teratogenic, so only indicated in NYHA III or IV patients;
- . phosphodiesterase inhibitor (e.g. sildenafil) promotes pulmonary smooth muscle relaxation reducing pulmonary hypertension.
Surgical:
Heart and lung transplantation may be an option for younger patients.
COMPLICATIONS
Right heart failure, arrhythmias (AF, VT, VF), sudden death.
PROGNOSIS
Chronic and incurable with unpredictable survival rate. Length of survival has improved to up to 15–20 years.
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