What are the current guidelines for the management and referral of patients with restrictive cardiomyopathy?

Management and Referral of Patients with Restrictive Cardiomyopathy

Restrictive cardiomyopathy is a form of heart failure characterised by impaired ventricular relaxation and diastolic dysfunction, often with preserved ejection fraction. The management and referral pathways align with those for chronic heart failure with preserved ejection fraction.

Referral: Patients suspected of having restrictive cardiomyopathy should be referred for specialist assessment, including transthoracic echocardiography performed by experienced operators to assess ventricular function and exclude other causes such as valve disease or intracardiac shunts. If transthoracic echocardiography images are suboptimal, alternative imaging modalities such as cardiac MRI should be considered. Specialist referral is essential for diagnosis confirmation, assessment of severity, and management planning.

Management: Management should be multidisciplinary, involving a specialist heart failure team including a cardiologist with subspecialty training, specialist heart failure nurse, and healthcare professionals skilled in heart failure prescribing. Treatment focuses on symptom control and optimisation of heart failure therapy, considering the diastolic dysfunction characteristic of restrictive cardiomyopathy. Beta-blockers may be considered if heart failure symptoms are present, especially if there is concomitant valve disease.

Patients should be regularly reviewed in primary care with effective communication between primary and specialist teams, with recall at least every 6 months. Palliative care needs should be assessed if symptoms worsen despite optimal treatment, with involvement of palliative care specialists as appropriate.

Overall, the approach emphasises early specialist referral for diagnosis and management, multidisciplinary care, and ongoing review to optimise outcomes.

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