5th July 2026

Management

Medical management 

• Broadly speaking, patients with symptomatic heart failure should be managed with loop diuretics for symptomatic relief and guideline-directed medical therapies (beta-blockers, ACEi/ARB/ARNi, MRA, SGLT2i). 
• However, caution should be exercised when using beta-blockers and ACEi/ARB/ARNi in patients with cardiac amyloidosis, as these drugs are poorly tolerated due to hypotension and autonomic dysfunction. Withdrawal of these drugs may help to improve symptoms. 
• All RCM patients with atrial fibrillation, in particular those with cardiac amyloidosis, should be started on oral anticoagulation irrespective of CHA2DS2-VA score due to a high risk of thromboembolism. 
• Disease-modifying treatment for specific RCM phenotypes are listed in Table 4. Patients with bradyarrhythmias may benefit from pacemaker implantation as per guidance from the European Society of Cardiology (ESC). 

Table 4: Selected RCM phenotypes and their respective disease-modifying therapies.

Surgical management

• Heart Transplantation
  • Heart transplantation may be considered in end-stage RCM patients with refractory heart failure and poor prognosis despite optimal medical therapy. 
  • Pulmonary vascular resistance study can be used to guide the timing of transplantation.
• ICDs
  • In select cases of RCM associated with severe ventricular arrhythmias or high risk of sudden cardiac death, ICDs may be considered for primary or secondary prevention.
  • Indicated in patients with sarcoidosis when a ventricular arrhythmia is detected