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Pathology
Heart muscle is rigid and is restricted from stretching and filling properly
.Aetiology
Primary: Idiopathic, fibroelastosis
Secondary: Amyloidosis, Haemochromatosis, sarcoidosis, carcinoid, malignancy
Signs
Oedema, ascites, palpable apex, hepatomegaly, raised JVP, prominent ‘x’ and ‘y’
descents, Kussmaul’s sign, loud S3 and S4
Symptoms
Exertional shortness of breath, decreased exercise tolerance and fatigue
Investigations
ECG: P mitrale, P pulmonale, conduction delay
Echo: Assess systolic and ventricular function
Cardiac catheterisation: To exclude constrictive pericarditis
Treatment
Treat underlying cause
Treat symptoms
Cardiac Transplantation.Prognosis
Poor prognosis, most patients die within 1 year
Pulmonary hypertension, thromboembolic complications, right ventricular
hypertrophy and right heart failure
Key Facts
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Key Images
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Key References
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