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Pathology

Right heart failure due to chronic pulmonary hypertension

Aetiology

Any cause of pulmonary hypertension. 
Pulmonary vascular disease: acute pulmonary embolism, primary pulmonary hypertension, chronic pulmonary embolism, parasitic infection 
Lung disease: COPD, asthma, pulmonary fibrosis, bronchiectasis 
Chronic hypoventilation: motor neurone disease, myasthenia gravis, kyphosis, scoliosis, OSA 

Signs

Peripheral oedema, raised JVP, right ventricular heave, cyanosis, tricuspid regurgitation, hepatomegaly, cyanosis

Symptoms

Dyspnoea, fatigue, anorexia, weight loss.

Investigations

ECG: Right ventricular hypertrophy and right axis deviation, P pulmonale. 
Chest X-Ray: Right atrial enlargement, cardiomegaly 
FBC: Polycythaemia. 
ECHO: Right ventricular dysfunction, tricuspid regurgitation and estimation of right 
sided pressures

Treatment

Treat the underlying cause, oxygen if hypoxic, diuretics, heart-lung transplantation

Prognosis

Poor, 50% 5 year mortality
Arrhythmia, liver failure

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