THE 2023 ESC GUIDELINES
FOR THE MANAGEMENT OF CARDIOMYOPATHIES: THE 10 COMMANDMENTS
THE FIRST INTERNATIONAL CLINICAL PRACTICE GUIDELINE COVERING THE FULL RANGE OF CARDIOMYOPATHIES THROUGHOUT THE LIFE COURSE
THE PATIENT PATHWAY
Running as a thread throughout the guideline is the concept of the patient pathway that requires a
CARDIOMYOPATHY MINDSET
TO CONSIDER THE POSSIBILITY OF A CARDIOMYOPATHY AS THE UNDERLYING CAUSE OF COMMON CLINICAL PRESENTATIONS
HEART FAILURE
ARRHYTHMIA
INCIDENTAL FINDINGS
A MULTI-DISCIPLINARY APPROACH
EXPERT CARDIOMYOPATHY CENTRES is recommended [Class I, level of evidence (LOE) C]
A DIAGNOSTIC
WORKFLOW
that incorporates the findings of several CARDIAC AND EXTRACARDIAC traits to arrive at a specific AETIOLOGICAL DIAGNOSIS
WHICH IN MANY CASES WILL BE GENETIC is recommended (Class I, LOE C)
FAMILY HISTORY with close collaboration between EVALUATION AND MANAGEMENT
And the importance of a systematic, multi-parametric approach for its diagnosis and evaluation.
PHENOTYPIC DESCRIPTION OF CARDIOMYOPATHY
Subtypes based on the identification of..
KEY MORPHOLOGICAL FUNCTIONAL MYOCARDIAL TRIATS
including the presence of myocardial scar on cardiac magnetic resonance imaging (MRI), at clinical presentation.
THE RESULTING PHENOTYPE-BASED CLASSIFICATION CONSISTS OF
HYPERTROPHIC CARDIOMYOPATHY (HCM)
DILATED CARDIOMYOPATHY (DCM)
NON-DILATED LEFT VENTRICULAR CARDIOMYOPATHY (NDLVC)
ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY
RESTRICTIVE CARDIOMYOPATHY
Importantly, these should not be considered diagnoses in their own right; rather, a careful and consistent description of the phenotype is only a crucial first step in the diagnostic pathway.
CARDIAC MYOSIN INHIBITORS
(mavacamten) are recommended as second-line therapy for adults with symptomatic obstructive HCM (Class IIa, LOE A)
SUDDEN DEATH RISK STRATIFICATION
A central aspect of the management of all cardiomyopathies, individualized risk assessment using validated risk prediction scores/algorithms, is recommended (Class I/IIa, LOE B)
These should be used as aids to shared DECISION-MAKING
THIS ALLOWS AETIOLOGY-DRIVEN MANAGEMENT RECOMMENDATIONS
CARDIAC MRI
It has a central role in the diagnosis of CARDIOMYOPATHIES IN PATIENTS (Class I, LOE B) AND RELATIVES (Class IIa, LOE B) has important prognostic and therapeutic implications (Class IIa, LOE C)
GENERIC TESTING accompanied by GENETIC COUNSELLING is a key aspect of the clinical workup in patients with cardiomyopathy (Class I, LOE B)
For probands, it allows the confirmation of an aetiological diagnosis and,
For relatives of all ages, cascade genetic testing allows individuals who do not carry the familial variant to be discharged from clinical follow-up. in some cases, can have prognostic implications and guide therapy and reproductive advice and management.
GENOTYPE-BASED SUDDEN DEATH RISK PREDICTION
It is particularly important in DCM/NDLVC, where the identification of high-risk genotypes and accompanying clinical features informs implantable cardioverter-defibrillator (ICD) IMPLANTATION DECISION-MAKING (Class IIa, LOE B)
INDIVIDUALIZED EXERCISE PRESCRIPTION AND REGULAR LOW- MODERATE INTENSITY EXERCISE ARE RECOMMENDED IN ALL PATIENTS WITH CARDIOMYOPATHY
(Class I, LOE C); more nuanced recommendations for different cardiomyopathy phenotypes, including gene carriers, are also made. making (Class IIa, LOE B).