Dear colleagues,

Since the 20^th century, standard ECG and cardiac MRI have allowed for the differentiation of arrhythmogenic cardiomyopathy into right-dominant, biventricular, and left-dominant forms, leading to varying treatment strategies. Implantable cardioverter-defibrillator (ICD) implantation is no longer the sole standard for preventing sudden cardiac death; rather, treating the underlying cause of ventricular tachycardia (VT) is considered a fundamental preventative step. Early research, including small studies and retrospective analyses, showed that sotalol could be effective in preventing VT/ventricular fibrillation induction in some patients and suppressing sustained VT. Subsequent research showed that amiodarone is effective in controlling VT when used alone or in combination with beta-blocking agents. Some studies have proposed that flecainide might have a direct blocking effect on ryanodine receptor type 2 calcium release channels, reducing spontaneous calcium release events. In patients with arrhythmogenic right or biventricular cardiomyopathy, heart failure therapy seems to reduce the risk of ICD intervention. Angiotensin-blocking agents like angiotensin-converting enzyme (ACE) inhibitors or angiotensin II type 1 (AT1) blockers can have a positive impact in smaller studies. To date, angiotensin receptor-neprilysin inhibitor (ARNI) has not been tested for its maximum effect on ST2 levels and reducing fibrosis. Sodium-glucose transport protein 2 (SGLT2) inhibitors reduce inflammation and fibrosis, thus leading to beneficial effects on the right and left ventricles. In arrhythmogenic left ventricular cardiomyopathy, standard therapy with the “great four” medications is warranted in reducing ICD intervention.

Genetic diagnostics is essential to exclude lamin A/C, filamin C, desmin, and phospholamban mutations as markers of preventive ICD implantation. Published studies on gene therapy, including those with significant side effects and utilizing AAV vectors, suggest that AAV-supplemented gene therapies may become the therapy of choice in the coming years. Uhl anomaly, as a possible severe manifestation of arrhythmogenic right ventricular cardiomyopathy, requires similar management strategies.

Dear readers, I would like to invite you to be possible authors in the field of management in arrhythmogenic cardiomyopathy.

Dr. Stefan Peters
Guest Editor