Definition:
Mean pulmonary artery pressure (mPAP) > 25mmHg at rest or > 30mmHg during exercise. Can present with no apparent underlying disease (primary pulmonary hypertension) or in association with other diseases.
Causes:
1. Pre-capillary (in pulmonary arteries and arterioles)
Most severe. Examples: congenital heart disease (left-to-right shunt), pulmonary embolism.
2. Capillary
Examples: pulmonary fibrosis, COPD.
3. Post-capillary
Examples: left ventricular failure, mitral valve disease.
4. Miscellaneous
Examples: drugs, sickle-cell anaemia.
Pathophysiology:
Hypoxic vasoconstriction, decreased surface area of pulmonary vascular bed, increased right ventricular volume/pressure.
Signs and symptoms:
According to cause, signs and symptoms of right ventricular failure (eg. peripheral oedema, shortness of breath).
Investigations:
CXR - enlargement of pulmonary arteries. ECG – right ventricular hypertrophy, right atrial enlargement. Echocardiogram – enlarged right ventricle.
Management:
Treat underlying cause. Primary – warfarin, oxygen and calcium channel blockers.
Prognosis:
Mean survival of 2-3 years from diagnosis. Cause of death often right ventricular failure or sudden death.
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