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
...Diagnosis...
...orithm to consider the presence of...
...hmogenic cardiomyopathyHaving trouble...
...re 3. Common pathway and genetic mutations...
...keletal protein complexes within the cardio...
...odified Task Force criteria for ARVC –...
...e 6. Representative 12-lead ECG obtained from AR...
...lassification of likelihood of pathog...
...Different methods for screening genesaHavi...
...Minimal set of genes to be prioritized in ACMHa...
...Who to Study...
...iduals and decedents with either a clinical or nec...
...genetic testing of the established...
...Cumulative prevalence of disease expression...
Treatment
...Treatment...
...Limitations of geneti...
...interpretation of a cardiac genetic t...
...enetic testing recommendations...
...Family...
...ed that a genetic counselor or appr...
...Age-rel...
...recommended that first-degree relati...
...ardiovascular evaluation should include...
...C...
...ess testing (arrhythmia provocation) may be co...
...Cascade ge...
...milies with a variant classified as pathog...
...gure 9. Summary of cascade family screening re...
...Risk stratification and...
...e decision to implant an ICD in an individu...
...individuals with ACM who have suffere...
...duals with ACM who have sustained VT not hem...
...n individuals with ACM and syncope...
...n individuals with ARVC with hemodynami...
...lantation is reasonable for individuals with ARV...
...implantation may be reasonable for individuals...
...duals with ACM with LVEF 35% or lower...
...s with ACM with LVEF 35% or lower and NYHA...
...viduals with ACM (other than ARVC) and h...
...ndividuals with phospholamban cardiom...
...ls with lamin A/C ACM and two or more...
...n individuals with FLNC ACM and an...
...duals with lamin A/C ACM and an indicati...
...jor criteria: aNSVT, inducibility to VT at...
...ntable cardioverter defibrillator recommen...
Prevention
...Preventio...
...Medical therapies...
...ividuals with ACM and symptomatic right...
...individuals with ACM and right ventricular...
...Antithrombotic t...
...with ACM, in the presence of atrial fibrillation,...
Antithrombotic therapy may be reas...
...Arrhythmia management...
Beta-blocker therapy is recommended in in...
...cker therapy is reasonable in individuals...
...darone (IIb, B-NR)573
...nd sotalol (IIb, C-LD)573...
...ide in combination with beta-blockers and in the a...
...ure 11. Recommendations for ventricu...
...e 12. Medical therapy recommendations fo...
...Role of cath...
...s with ACM and recurrent sustained monomorph...
...uals with ACM and recurrent symptom...
...tomatic individuals with ACM and a high burden...
...ith ACM and recurrent symptomatic susta...
...13. Catheter ablation recommendation...
...Prevention of Dise...
...Exercise a...
...linicians should counsel adolescent and adu...
...ividuals with ARVC should not participate in comp...
...e 14. Exercise recommendations for individual...
.... METS associated with common types of...
Other Disorders
...Other Disorde...
...sease mechanisms for ACMCardiomyoc...
...Am...
...oth symptomatic and asymptomatic individua...
...dividuals with cardiac amyloidosis...
...n individuals with cardiac amyloidosis, the use...
...individuals with cardiac amyloidosis and s...
...dividuals with AL-type cardiac amyloidosis with...
...ls with cardiac amyloidosis and sym...
...myloidosis arrhythmia treatment recomme...
...Left ventricula...
...band has a disease-causing gene varia...
In individuals with the clinical d...
...is recommended for individuals with LVNC and evid...
...implantation is reasonable for individuals...
...on is recommended in individuals with L...
...coagulation may be reasonable in individuals...
...with suspected LVNC, the diagnostic criteria by e...
...iduals with suspected LVNC and ventric...
...gnostic criteria for left ventricular non...
Figure 18. Diagnosis and risk stratification o...
Figure 19. LVNC treatment recommendati...