Cardiac amyloidosis is caused by amyloid fibrils that deposit in the myocardial interstitium, causing restrictive cardiomyopathy and eventually death. The electromechanical, inflammatory, and autonomic changes due to amyloid deposition result in arrhythmias. Atrial fibrillation is by far the most common arrhythmia. The rate control strategy is generally poorly tolerated due to restrictive filling physiology and heart rate dependance, favoring adoption of the rhythm control strategy. Anticoagulation for stroke prophylaxis is warranted, irrespective of CHA₂DS₂-VASc score in patients with a favorable bleeding profile; data on left appendage closure devices are still insufficient. Ventricular arrhythmias are also not uncommon, and the role of implantable cardioverter-defibrillator in cardiac amyloidosis is controversial. There is no evidence of improvement in outcomes when used for primary prevention in these patients. Bradyarrhythmia is most commonly associated with sudden cardiac death in cardiac amyloidosis. Pacemaker implantation can help provide symptomatic relief but does not confer mortality benefit.

Cardiac amyloidosis is caused by amyloid fibrils that deposit in the myocardial interstitium, causing restrictive cardiomyopathy and eventually death. The electromechanical, inflammatory, and autonomic changes due to amyloid deposition result in arrhythmias. Atrial fibrillation is by far the most common arrhythmia. Rate control strategy is generally poorly tolerated due to restrictive filling physiology and heart rate dependance, favoring adoption of rhythm control strategy. Anticoagulation for stroke prophylaxis is warranted irrespective of CHA2DS2Vasc score in patients with favorable bleeding profile; data on left appendage closure devices is still insufficient. Ventricular arrhythmias are also not uncommon and the role of implantable cardioverter-defibrillator in cardiac amyloidosis is controversial. There is no evidence of improvement in outcomes when used for primary prevention in these patients. Bradyarrhythmia is most commonly associated with sudden cardiac death in cardiac amyloidosis. Pacemaker implantation can help provide symptomatic relief but does not confer mortality benefit.

The prevalence of cardiac amyloidosis (CA), especially as a cause of heart failure, has significantly increased in recent years. Early detection and accurate assessment of the disease burden are crucial for initiating timely treatment and ensuring precise prognosis. CA primarily results from the infiltration of the myocardium by either immunoglobulin light chain fibrils (AL) or transthyretin fibrils (ATTR), leading to restrictive cardiomyopathy and eventual death if untreated. Over the past decade, advancements in diagnostic imaging and heightened clinical awareness have revealed a substantial presence of CA, particularly ATTR, among the elderly. These diagnostic improvements encompass echocardiography, cardiac computerized tomography scans, magnetic resonance imaging, and radionuclide scintigraphy with bone-avid tracers. Concurrently, significant progress has been made in therapeutic options, with new disease-modifying treatments now available that can dramatically alter the disease trajectory and improve survival rates when administered early. However, despite these advancements, there remains an urgent need for the early and accurate detection of CA to ensure that patients can fully benefit from these emerging therapies.

The prevalence of cardiac amyloidosis (CA), especially as a cause of heart failure, has significantly increased in recent years. Early detection and accurate assessment of the disease burden are crucial for initiating timely treatment and ensuring precise prognosis. CA primarily results from the infiltration of the myocardium by either immunoglobulin light chain fibrils (AL) or transthyretin fibrils (ATTR), leading to restrictive cardiomyopathy and eventual death if untreated. Over the past decade, advancements in diagnostic imaging and heightened clinical awareness have revealed a substantial presence of CA, particularly ATTR, among the elderly. These diagnostic improvements encompass echocardiography, cardiac computerized tomography scan, magnetic resonance imaging, and radionuclide scintigraphy with bone-avid tracers. Concurrently, significant progress has been made in therapeutic options, with new disease-modifying treatments now available that can dramatically alter the disease trajectory and improve survival rates when administered early. However, despite these advancements, there remains an urgent need for the early and accurate detection of CA to ensure that patients can fully benefit from these emerging therapies.