ESC guidelines are the first to "include all cardiomyopathy subtypes, and the first time that specific recommendations are made for cardiomyopathies other than hypertrophic cardiomyopathy.
Cardiomyopathy can present at any age. It can have multiple complex etiologies, including genetic predisposition, heart muscle injury due to disease, or a mix of participating factors. The ESC task force employed several strategies in taking a comprehensive approach to the condition.
From my point of view, the key innovations include a diagnostic workup that starts with a detailed phenotypic description, including the new phenotype of nondilated left ventricular cardiomyopathy, that then triggers a multiparametric, systematic evaluation.
Guidelines have been organized around the patient pathway, meaning that focus should be placed on recognizing the presenting phenotype as a critical first step in discerning the underlying etiology and its treatments.
The new guidelines include specific recommendations about genetic testing of children. They also emphasize the value of cardiovascular magnetic resonance (CMR) imaging in the "diagnosis, screening, monitoring, and prognostication" for patients of all ages, according to Kaski.
CMR is recommended at the initial evaluation for every patient with cardiomyopathy.
It should be "considered" during follow-up and for many other applications, including the evaluation of "genotype-positive but phenotype-negative relatives.
Etiologic prediction models have been incorporated into the guidelines, including genotyping for dilated cardiomyopathies and nondilated left ventricular cardiomyopathy.
Each of the recommendations in the guidelines is provided with an evidence-based classification. In order, these are class I (recommended), class IIa (should be considered), class IIb (may be considered), and class III (not recommended).
Guidelines draw attention to the relationship of cardiomyopathy to common cardiovascular conditions, such as heart failure, arrhythmia, and chest pain. Kaski pointed out that these are the types of problems commonly encountered by general cardiologists and well as primary care physicians.
In 2014, the ESC published guidelines specific to HCM. The new broader guidelines do not overlook this subtype. According to Kaski, there have several innovations in HCM since the previous guidelines, such as when to consider cardiac myosin inhibitors for symptomatic left ventricular outflow tract obstruction.
The ESC guidelines place an emphasis on a "coordinated, systematic, and individualized" care pathway based on a multidisciplinary approach, according to Abelo. Although the composition of the interdisciplinary team depends on the individual case, the guidelines recognize a key role for general cardiologists in managing the majority of patients. Suggestions of when to refer challenging cases to expert centers are outlined.
The guidelines include almost 90 pages of recommendations. The task force isolated 32 key messages from 13 sections ranging from descriptions of how the patient pathway is defined to what types of physical activity should be considered for different forms of cardiomyopathy. There is also a section devoted to important gaps in evidence and areas in which there is the most need for further studies.
The guidelines end with a comprehensive list of "what to do" and "what not to do" in the diagnosis and care of cardiomyopathy. These include most of the class I recommendations and summarize some important class III cautions.
"Most of the recommendations in the guideline are new," the authors write in the introduction. Although they acknowledged that they did not attempt to provide detailed recommendations for every cardiomyopathy phenotype, they have endeavored to cover general evaluation and management issues supported by relevant evidence.
https://www.medscape.com/viewarticle/996083
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