Restrictive cardiomyopathy is characterised by decreased right atrial compliance, which manifests as a significantly elevated jugular venous pressure and prominent right heart failure.
- Causes
- Idiopathic
- Infiltration
- Sarcoidosis
- Amyloidosis
- Hemocromatosis
- Scleroderma
- Fabry’s disease
- Eosinophilia
- Metastatic cancer
- Radiation for chest malignancy
- Signs and symptoms
- Fatigue
- Dyspnoea
- Shortness of breath
- Jugular venous distension
- Peripheral edema
- Ascites
- Hepatosplenomegaly
- Wheezing
- Rales
- Rhonchi
- Signs of systemic disease
- S3 heart sound
- Mitral and tricuspid regurgitation (systolic murmur)
- Kussmaul sign
- Similarities to hypertrophic cardiomyopathy (HCM)
- Reduced ventricular filling
- Thickened walls
- Similarities to dilated cardiomyopathy (DCM)
- Pronounced heart failure symptoms – dyspnea, wheeze, crackles
- Signs of right heart failure – peripheral edema, ascites, hepatosplenomegaly
- S3 heart sound present
- Unique features of restrictive cardiomyopathy (RCM)
- Decreased amplitude on EKG
- Characteristic echo findings
- Investigation
- Echocardiography
- Rapid diastolic filling
- EKG:
- Decreased QRS amplitude
- LBBB
- Chest X-ray to evaluate for pulmonary oedema
- Cardiac MRI to distinguish constrictive pericarditis from restrictive cardiomyopathy
- Echocardiography
- Treatment
- Palliative
- Furosemide to reduce fluid overload
- Beta blockers and/or calcium channel blockers
- Treat the underlying cause
