Emerging from the Darkness. Sudden Cardiac Death in Cardiac Amyloidosis

• Published in: Reviews in cardiovascular medicine Vol. 23; no. 10; p. 345
• Main Authors: Cammalleri, Valeria, De Luca, Valeria Maria, Antonelli, Giorgio, Annibali, Ombretta, Nusca, Annunziata, Mega, Simona, Carpenito, Myriam, Ricciardi, Danilo, Gurrieri, Fiorella, Avvisati, Giuseppe, Ussia, Gian Paolo, Grigioni, Francesco
• Format: Journal Article
• Language: English
• Published: Singapore IMR Press 01.10.2022
• Subjects: cardiac amyloidosis; implantable cardioverter defibrillator; monoclonal immunoglobulin light chains; Review; sudden cardiac death; transthyretin amyloidosis; ventricular arrhythmia; ventricular arrhythmia; cardiac amyloidosis; implantable cardioverter defibrillator; monoclonal immunoglobulin light chains; sudden cardiac death; transthyretin amyloidosis
• ISSN: 1530-6550; 2153-8174; 2153-8174; 1530-6550
• DOI: 10.31083/j.rcm2310345

Cardiac amyloidosis (CA) manifests as infiltrative cardiomyopathy with a hypertrophic pattern, usually presenting with heart failure with a preserved ejection fraction. In addition, degenerative valvular heart disease, particularly severe aortic stenosis, is commonly seen in patients with CA. However, amyloid fibril deposition might also infiltrate the conduction system and promote the development of electrical disorders, including ventricular tachyarrhythmias, atrio-ventricular block or acute electromechanical dissociation. These manifestations can increase the risk of sudden cardiac death. This review summarises the pathophysiological mechanisms and risk factors for sudden cardiac death in CA and focuses on the major current concerns regarding medical and device management in this challenging scenario.