Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy

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Raja Selvaraj MD DNB FCE (Toronto)
Professor of Cardiology
JIPMER

Guidelines

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  • 34 year old male
  • Chest pain - abnormal ECG, evaluation suggestive of HCM, referred
  • DOE class II NYHA

Recommended initial evaluation

  • Clinical examination (1, B-NR)
  • 12 lead ECG (1, B-NR)
  • Echocardiogram (1, B-NR)
  • 24 to 48 hour ambulatory ECG monitoring (1, B-NR)

Family history

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Echocardiogram

  • Left ventricular hypertrophy
  • IVS 18 mm - thickest part of LV
  • Outflow gradient - 24 mm Hg
  • LVEF 65%
  • No apical aneurysm

Echocardiogram - Questions

  • Provocative maneuvers?
  • Exercise
  • Echo contrast ?
  • TEE ?

Provocative maneuvers / Exercise

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CMR

  • When is it needed ?
  • What information does it provide ?
  • CT is an alternative if CMR not available (2b, C-LD)

CMR

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Heart Rhythm assessment

  • 12 lead ECG (1, B-NR)- Left ventricular hypertrophy
  • 24 hr Ambulatory ECG (1, B-NR) - No arrhythmias
  • Event recorders in case of symptoms to document rhythm correlation (1, B-NR)

Screening for AF

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Cath / Angiography ?

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  • Completed clinical evaluation
  • Echo / ECG / Holter
  • CMR / AECG
  • Genetic testing ?

Genetic testing

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SCD evaluation - Essential

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SCD - Optional evaluation

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SCD - Risk factors

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5 year SCD risk assessment

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Constantinos O'Mahony et al, for the Hypertrophic Cardiomyopathy Outcomes Investigators, A novel clinical risk prediction model for sudden cardiac death in hypertrophic cardiomyopathy (HCM Risk-SCD), European Heart Journal, Volume 35, Issue 30, 7 August 2014, Pages 2010–2020

Review of our patients risk - Does he need an ICD ?

  • No previous cardiac arrest / sustained VT
  • Sudden death in close relative < 50 yrs
  • LVH not > 30 mm
  • No arrhythmic syncope recently
  • No LV apical aneurysm
  • Normal LVEF

Shared decision making

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Decision

  • Young age, dependent family
  • Family history of sudden death
  • Decided to undergo ICD implant
  • CMR could have helped if undecided

Which ICD ?

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Pharmacologic management - Obstructive HCM

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Septal reduction therapy

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Pharmacologic management - Non obstructive HCM

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Lifestyle - Sports and activity

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Outcome of evaluation and prescription

  • Non-obstructive HCM, NYHA II
  • SCD risk (one risk factor) - Single chamber ICD
  • Drugs - beta blocker
  • Screening of family members
  • Periodic evaluation schedule

Follow up schedule

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Family screening

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Family screening

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Genotype positive, phenotype negative

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Six months later - Acute breathlessness

  • AF ecg
  • Paroxysmal AF
  • CHADS-VASC 0 - OAC or not ?

Anticoagulation for AF

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Top take home messages

  • Imaging plays a crucial role in care, Echo remains most important, MRI useful in many patients
  • Assessment of individual patient's risk continues to evolve
  • Septal reduction therapies continue to improve in efficacy and safety
  • In patients with AF, anticoagulant therapy needed irrespective of CHADS-VASC score
  • EF < 50 indicates significant dysfunction and higher risk
  • Moderate exercise is beneficial