Atrial consequences: High atrial pressures lead to atrial hypertrophy and dilatation, followed later by atrial fibrillation.
Causes and diagnostic assessment
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Most common UK cause: Amyloidosis is the most common cause in the UK.
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Other causes: Glycogen storage diseases, idiopathic perimyocyte fibrosis and a familial form can also cause restrictive cardiomyopathy.
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Assessment: Diagnosis can be difficult and requires Doppler echocardiography, computed tomography or magnetic resonance imaging, and endomyocardial biopsy.
Treatment and prognosis
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Treatment: Management is symptomatic, and cardiac transplantation may be indicated.