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