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Hypertrophic Cardiomyopathy:What’s new in the Guidelines and how does it impact clinical practice

Steve R. Ommen, MDDirector, Mayo Hypertrophic Cardiomyopathy ClinicProfessor of Medicine

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No conflicts of interest

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Guideline

• Advice

• Recommendation, but not mandatory

As opposed to “policy” which is meant to ensure compliance

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An expert is a person who has made all the mistakes which can be made in a very narrow field.

-paraphrased from Neils Bohr

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ACC/AHA & ESC Guideline Comparison

• What is the same?

• Families need to be screened

• Standard testing includes

• ECG, Holter, Transthoracic echo +/- MRI

• Medications first for obstruction

• SCD risk needs to be assessed in every patient

• VKA for atrial fibrillation

• Follow up with prudent testing

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ACC/AHA & ESC Guideline Comparison

What is the same?

Centers with experience / expertise should be involved:

specialized imaging

therapeutic procedures

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ACC/AHA & ESC Guideline Comparison

What is different in ESC?

• More specific details

• No preference regarding septal reduction therapy

• New SCD risk prediction tool

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Annualized rate of SCD

~1% per year

Heart 2008;94:1269–1275.

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How do we know which patients will

need a defibrillator?

05

1015202530

Cu

mu

lati

ve %

(3.7

+/-

2.8

years

)

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Nonsustained

Ventricular

Tachycardia

• Rest

• Exercise

Severity of LVH

Abnormal Exercise

BP

Response

Unexplained

Syncope

Family History of

Sudden Death

Fibrosis or scar

Role of Isolated

Myofilament

Mutations

Outflow

Obstruction

SCD In HCM

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Which HCM Pt needs ICD?Risk Factors Recommendation

0 Reassurance

1 Individualize

2+ Recommend ICD

Prior SCD Recommend ICD

Sustained VT Recommend ICD

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Young age

MLVWT approaching 30 mm

Remote or Unclear FHSCD

LVOTO and no planned intervention

Delayed enhancement

Septal Ablation

Known specific mutations

Syncope in past 5 years

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Receiver operating characteristic (ROC(t)) curves for the American College of Cardiology/European

Society of Cardiology (ACC/ESC) and ACC Foundation/American Heart Association (ACCF/AHA)

guidelines.

O'Mahony C et al. Heart 2013;99:534-541

Copyright © BMJ Publishing Group Ltd & British Cardiovascular Society. All rights reserved.

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49 yo male

HCM dx 1993 based on abnormal ECG

Active, asymptomatic

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49 yo male

Echo

Wall thickness = 21 mm

Non-obstructive

Exercise Test

121% FAC

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Recommended (4/28/2008):

24 Hour Holter Monitor

Continue current meds

No competitive sports

49 yo male

7/26/2008

Died,

Hiking

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Risk Stratification is Imperfect

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Risk Stratification is Imperfect

…if we expect to tell us yes or no for any individual patient

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Change the Mindset

Risk Stratification helps contextualize

risk

We help patients make decisions

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Help Patients Make Decisions

The decision for placement of an ICD in HCM patients should include prudent application of

individual clinical judgment, as well as thorough discussion of the strength of evidence, benefits,

and risks to allow active participation of the informed patient in decision-making

“…other clinical features that are of potential prognostic importance and when the likely benefit

is greater than the lifelong risk of complications and the impact of an ICD on lifestyle, socioeconmic

status and psychological health”

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Advocate, counsel, inform…..

If the patient is going to leave the office afraid to live because they might

die…then that’s the patient who should

strongly consider ICD

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