Amyloidosis is a systemic disorder characterized by extracellular deposition of insoluble fibrils. The most common forms are amyloid light chain and amyloid transthyretin (ATTR) amyloidoses. Cardiac involvement may be found in both these forms, and is an important cause of morbidity and mortality. The clinical presentation of cardiac amyloidosis (CA) may be represented by congestive heart failure (HF), possibly progressing to end-stage HF, as well as atrial fibrillation with possible thromboembolic events, and also conduction disturbances related to amyloid infiltration of conduction fibres. Beyond therapies targeting the blood dyscrasia or the ATTR amyloidogenic cascade, a careful choice of drug therapies, need for device implantation, and possibly treatments for advanced HF is then warranted. In the present review, we try to provide a useful guide to clinicians treating patients with CA by enucleating 10 main questions and answering them based on the evidence available as well as expert opinion and our clinical experience.
Management of complications of cardiac amyloidosis: 10 questions and answers
Aimo A.;Vergaro G.;Giannoni A.;Passino C.;Emdin M.
2020-01-01
Abstract
Amyloidosis is a systemic disorder characterized by extracellular deposition of insoluble fibrils. The most common forms are amyloid light chain and amyloid transthyretin (ATTR) amyloidoses. Cardiac involvement may be found in both these forms, and is an important cause of morbidity and mortality. The clinical presentation of cardiac amyloidosis (CA) may be represented by congestive heart failure (HF), possibly progressing to end-stage HF, as well as atrial fibrillation with possible thromboembolic events, and also conduction disturbances related to amyloid infiltration of conduction fibres. Beyond therapies targeting the blood dyscrasia or the ATTR amyloidogenic cascade, a careful choice of drug therapies, need for device implantation, and possibly treatments for advanced HF is then warranted. In the present review, we try to provide a useful guide to clinicians treating patients with CA by enucleating 10 main questions and answering them based on the evidence available as well as expert opinion and our clinical experience.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.