Hypertrophic Cardiomyopathy HCM – Latest Management Certificate Course/Hong... · 2019. 9. 9. ·...
Transcript of Hypertrophic Cardiomyopathy HCM – Latest Management Certificate Course/Hong... · 2019. 9. 9. ·...
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Hypertrophic Cardiomyopathy“ HCM ”– Latest Management
Cardiology Structured Training Course
Dr. Kam Tim, Chan
HK College of Cardiology / HKPHCA Ltd
July, 2019
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Conflicts of Interest
• I have NOTHING to disclose concerning this presentation
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Outlines• Epidemiology• Diagnosis of HCM• Differentiating from Athlete’s Heart• Overview of Treatment Options - Medical vs Surgical • Role of Genetic Testing • Sudden Cardiac Death
- Assessing Risk- Assess ICD use
Recent Guidelines :
HCM – European Heart J, 2014;35, 2733-2779
JACC 2015;66;2362-2371JACC Heart Failure; 11,Apr,2018
Sudden Death - EHJ 2015;36;2793-2867
Circulation 2015;133;1006-1026Heart Rhythm, 15;10,oct,18
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EPIDEMIOLOGY
Sudden Death in young athelets
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-Autosomal DominantVariable Penetrance and expressability
-Caused by mutation in1 of 11 cardiac sarcomere genes oradjacent Z disc genes
-1500 individual mutations known
-90% missense
Mutant genes - %Beta-myosin Heavy chain -30-40% Myosin binding protein C-30-40%Troponin T - 10%
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HCM - Diagnosis
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• LV wall thickness > 15mm by Echocardiogram; CMR or
CT in Absence of Secondary causes
• LVH typically manifests as Asymmetrical Septal Hypertrophy
•Other patterns : Apical; Concentric; Lateral wall or RT
ventricular can occur
• FIRST degree relatives of PATIENTS with unequivocal
diseases; Unexplained wall thickness >/= 13 mm ->
Sufficient for diagnosis
HCM – Diagnosis
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If LVOTO > 50 mmHg – Cause hemodynamic consequences
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NO
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STRESS ECHO
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IVS
AO
LV
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ECHOCARDIOGRAM in HCM
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Cardiac -MRI in HCM • Advantages :
- Superior spatial resolution
- Accurate volumetric assessment of ALL chambers ; LV mass calculation
- NO Limited echo windows – in Lung diseases; chest wall AbN
- Better define apical aneurysm, thrombus etc
- LGE for fibrosis
Drawbacks : • Costs
• Inaccessible
• CRF- C/I for gadolinium
• Prolonged breath holding
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•Differential Diagnosis - DDX
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Congenital Subaortic Stenosis – Fibromuscular membrane
Peak grad -108mmHgMean grad - 63mmHg
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Left Ventricular NON-Compaction Syndrome
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HCM vs Ventricular Septal Bulge ( Sigmoid Septum )
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HOCM – Sudden Death During Competitive Sports
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VS
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Treatment
• Life Styles Measures
• Symptomatic treatment
( MEDICAL vs. SURGICAL )
•Prevention of Sudden Cardiac Death
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General LIFE Styles Measures
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Pre-assessment Checklist forINVASIVE Septal Reduction therapies
EHJ 2014;35:2733-2779
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• Predication / Prevention of Sudden Cardiac
Death
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ESC 2014
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Types of VT :
2017 ACC/ AHA/HRS Guidelines
Monomorphic VT
Polymorphic VT
Bidirectional VT
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2017ACC/AHA/HRS Guidelines
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2017 ACC/ AHA/ HRS Guidelines
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2017 ACC/ AHA/ HRS Guidelines
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Prevention of SCD in HCM Patients
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2017 ACC / AHS / HRS Guidelines
ESC
Vs
ACC/AHA
Guidelines
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Heart Failure Treatment in HCM
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Clinical Course in HCM associated with HF Symptoms and Functional Impairment
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• Role of GENETIC Testing
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SUMMARY
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SUMMARY
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• Thank you very much
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•Questions and Answers
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