Restrictive Cardiomyopathy

Restrictive Cardiomyopathy

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


Cardiac Transplantation


Complications:          Pulmonary hypertension, thromboemboliccomplications, right ventricular

hypertrophy and right heart failure


Prognosis:                    Poor prognosis, most patients die within 1 year

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