<s> [INST] <<SYS>> You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, say that you don't know. Please don't share false information. <</SYS>>What aret the TOP 10 TAKE-HOME MESSAGES - 2020 AHA/ACC .GUIDELINE FOR THE DIAGNOSIS AND TREATMENT
OF PATIENTS WITH HYPERTROPHIC CARDIOMYOPATHY? [/INST] 1. Shared decision-making, a dialogue between patients
and their care team that includes full disclosure of all
testing and treatment options, discussion of the risks
and benefits of those options and, importantly,
engagement of the patient to express their own goals,
is particularly relevant in the management of conditions such as hypertrophic cardiomyopathy (HCM).
2. Although the primary cardiology team can initiate
evaluation, treatment, and longitudinal care, referral
to multidisciplinary HCM centers with graduated
levels of expertise can be important to optimizing care
for patients with HCM. Challenging treatment decisions—where reasonable alternatives exist, where
the strength of recommendation is weak (e.g., any
Class 2b decision) or is particularly nuanced, and for
invasive procedures that are specific to patients with
HCM—represent crucial opportunities to refer patients
to these HCM centers.
3. Counseling patients with HCM regarding the potential
for genetic transmission of HCM is one of the cornerstones of care. Screening first-degree family members
of patients with HCM, using either genetic testing or
an imaging/electrocardiographic surveillance protocol, can begin at any age and can be influenced by
specifics of the patient/family history and family
preference. As screening recommendations for family
members hinge on the pathogenicity of any detected
variants, the reported pathogenicity should be
reconfirmed every 2 to 3 years.
4. Optimal care for patients with HCM requires cardiac
imaging to confirm the diagnosis, characterize the
pathophysiology for the individual, and identify risk
markers that may inform decisions regarding interventions for left ventricular outflow tract obstruction and sudden cardiac death (SCD) prevention.
Echocardiography continues to be the foundational
imaging modality for patients with HCM. Cardiovascular magnetic resonance imaging will also be helpful
in many patients, especially those in whom there is
diagnostic uncertainty, poor echocardiographic imaging windows, or where uncertainty persists
regarding decisions around implantable cardioverterdefibrillator (ICD) placement.
5. Assessment of an individual patient’s risk for SCD
continues to evolve as new markers emerge (e.g.,
apical aneurysm, decreased left ventricular systolic
function, and extensive gadolinium enhancement). In
addition to a full accounting of an individual’s risk
markers, communication with patients regarding not
just the presence of risk markers but also the magnitude of their individualized risk is key. This enables
the informed patient to fully participate in the
decision-making regarding ICD placement, which incorporates their own level of risk tolerance and
treatment goals.
6. The risk factors for SCD in children with HCM carry
different weights than those observed in adult patients; they vary with age and must account for
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different body sizes. Coupled with the complexity of
placing ICDs in young patients with anticipated
growth and a higher risk of device complications, the
threshold for ICD implantation in children often differs from adults. These differences are best addressed
at primary or comprehensive HCM centers with
expertise in children with HCM.
7. Septal reduction therapies (surgical septal myectomy
and alcohol septal ablation), when performed by
experienced HCM teams at dedicated centers,
continue to improve in safety and efficacy such that
earlier intervention may be possible in select patients
with drug-refractory or severe outflow tract obstruction causing signs of cardiac decompensation. Given
the data on the significantly improved outcomes at
comprehensive HCM centers, these decisions represent an optimal referral opportunity.
8. Patients with HCM and persistent or paroxysmal atrial
fibrillation have a sufficiently increased risk of stroke
such that oral anticoagulation with direct oral anticoagulants (or alternatively warfarin) should be
considered the default treatment option independent
of the CHA2DS2VASc score. As rapid atrial fibrillation
is often poorly tolerated in patients with HCM,
maintenance of sinus rhythm and rate control are key
pursuits in successful treatment.
9. Heart failure symptoms in patients with HCM, in the
absence of left ventricular outflow tract obstruction,
should be treated similarly to other patients with
heart failure symptoms, including consideration of
advanced treatment options (e.g., cardiac resynchronization therapy, left ventricular assist device, transplantation). In patients with HCM, an ejection
fraction <50% connotes significantly impaired systolic function and identifies individuals with poor
prognosis and who are at increased risk for SCD.
10. Increasingly, data affirm that the beneficial effects of
exercise on general health can be extended to patients
with HCM. Healthy recreational exercise (moderate
intensity) has not been associated with increased risk
of ventricular arrhythmia events in recent studies.
Whether an individual patient with HCM wishes to
pursue more rigorous exercise/training is dependent
on a comprehensive shared discussion between that
patient and their expert HCM care team regarding the
potential risks of that level of training/participation
but with the understanding that exercise-related risk
cannot be individualized for a given patient. </s>