Evaluation, Risk Stratification, and Management of Arrhythmogenic Cardiomyopathy
Key Points
Key Points
- Arrhythmogenic cardiomyopathy (ACM) is defined as an arrhythmogenic heart muscle disorder not explained by ischemic, hypertensive, or valvular heart disease.
- ACM incorporates a broad spectrum of genetic, systemic, infectious, and inflammatory disorders. This designation includes, but is not limited to, arrhythmogenic right ventricular cardiomyopathy (ARVC), arrhythmogenic left ventricular cardiomyopathy (ALVC), cardiac amyloidosis, sarcoidosis, Chagas disease and left ventricular noncompaction.
- The etiology may be part of a systemic disorder (e.g. sarcoidosis, amyloidosis), an apparently isolated cardiac abnormality (e.g., myocarditis), an infection (e.g., Chagas disease) or may be genetic (e.g., desmosomal ARVC or ALVC, lamin A/C, filamin C, phospholamban) with particular phenotypic (cardiac, cutaneous, immunologic) features (Figure 1 and Figure 2).
- A distinguishing feature of ACM is the clinical presentation with documented and/or symptomatic arrhythmia (e.g., atrial fibrillation, conduction disease, ARVC and/or ALVC (Figure 1).
- Clinical evaluation for diagnosis and management of ACM should assess genetic and nongenetic causes with assessment of electrocardiographic and structural abnormalities and arrhythmic risk.
Diagnosis
...gnosis...
...1. Algorithm to consider the presence...
...Arrhythmogenic cardiomyopathyHaving trouble viewi...
...3. Common pathway and genetic mutati...
...ytoskeletal protein complexes withi...
...ied Task Force criteria for ARVC –...
...6. Representative 12-lead ECG obtained f...
...ification of likelihood of pathogenicity of a...
...ble 2. Different methods for screening gen...
...inimal set of genes to be prioritized in AC...
...o to Study...
...and decedents with either a clinical or necrops...
...ing of the established ACM-susceptibi...
...lative prevalence of disease expression...
Treatment
...reatme...
...ions of genetic testing...
...pretation of a cardiac genetic test by a...
...e 8. Genetic testing recommendations...
...ly history...
...mended that a genetic counselor or appropriatel...
Age-related penetrance of disease in at-risk...
...mended that first-degree relatives undergo clinica...
...ovascular evaluation should include...
...ascade cardiac investigati...
...testing (arrhythmia provocation) may be...
...ade genetic testing...
...amilies with a variant classified as pathogenic...
...Summary of cascade family screening recomm...
...k stratification and ICD decis...
...n to implant an ICD in an individual with ACM sho...
...with ACM who have suffered a cardiac arrest with...
...duals with ACM who have sustained VT not hemod...
...n individuals with ACM and syncope sus...
...uals with ARVC with hemodynamically toler...
ICD implantation is reasonable for individuals...
...ation may be reasonable for individua...
...als with ACM with LVEF 35% or lower and NYHA clas...
...ls with ACM with LVEF 35% or lower and...
...n individuals with ACM (other than...
...n individuals with phospholamban cardiomyo...
...dividuals with lamin A/C ACM and two or more o...
...duals with FLNC ACM and an LVEF...
...n individuals with lamin A/C ACM and an indic...
...jor criteria: aNSVT, inducibility to VT at EPS...
...10. Implantable cardioverter defibrillator...
Prevention
...evention...
...l therapies for right ventricular fai...
...uals with ACM and symptomatic righ...
...n symptomatic individuals with ACM...
...thrombotic therapy in ACM...
...or individuals with ACM, in the presence of...
...herapy may be reasonable in individuals wi...
...rrhythmia management
...a-blocker therapy is recommended in ind...
...rapy is reasonable in individuals with ACM who do...
...arone (IIb, B-NR)573...
...sotalol (IIb, C-LD)573...
...in combination with beta-blockers a...
...commendations for ventricular dysfunction an...
...gure 12. Medical therapy recommendations fo...
...of catheter abla...
...iduals with ACM and recurrent sustained monomorph...
...iduals with ACM and recurrent symp...
...c individuals with ACM and a high b...
...ividuals with ACM and recurrent symp...
Figure 13. Catheter ablation recommendations for...
...ention of Disease Progressi...
...cise and other...
...ans should counsel adolescent and adult individua...
...viduals with ARVC should not particip...
.... Exercise recommendations for individu...
...gure 15. METS associated with common type...
Other Disorders
...r Disorder...
...sease mechanisms for ACMCardiomyocyte showin...
...myloidosis...
...symptomatic and asymptomatic individuals with car...
...ls with cardiac amyloidosis who hav...
...with cardiac amyloidosis, the use of digo...
...viduals with cardiac amyloidosis and symptom...
...s with AL-type cardiac amyloidosis with non...
...individuals with cardiac amyloidosis and s...
...re 17. Amyloidosis arrhythmia treatment recomme...
...lar noncompaction treatment...
...oband has a disease-causing gene variant, i...
...with the clinical diagnosis of pathologic LV...
...n is recommended for individuals with LVNC a...
...implantation is reasonable for individuals with...
...tion is recommended in individuals with LV...
Anticoagulation may be reasonable in individu...
...with suspected LVNC, the diagnosti...
...with suspected LVNC and ventricular...
...4. Diagnostic criteria for left ventr...
...agnosis and risk stratification of...
...NC treatment recommendations...