Download Managing Hypertrophic Cardiomyopathy with Imaging

Managing Hypertrophic
Cardiomyopathy with Imaging
Gisela C. Mueller
University of Michigan Department of Radiology
Disclosures
¾
Gadolinium contrast material for cardiac MRI
Acronyms
Afib
Atrial fibrillation
PVC
Premature ventricular
contraction
CAD
Coronary Artery
Disease
RF
Radiofrequency
HCM
Hypertrophic
Cardiomyopathy
SAM
Systolic anterior motion
of mitral valve
LA
Left atrium
SAX
Short axis view
LV
Left ventricle
SCD
Sudden cardiac death
LVOT
Left ventricular outflow
tract
Vfib
Ventricular fibrillation
Non-sustained
ventricular tachycardia
Vtach
Ventricular tachycardia
NSVT
Agenda
I.
II.
III.
IV.
V.
VI.
VII.
VIII.
HCM: Management and Diagnostic Issues
Prevention of SCD
CMR: Late Gadolinium Enhancement
Dynamic LVOT and Mid-cavity Obstruction
Heart Failure and Atrial Fibrillation
Early Markers of HCM
Differential Diagnoses
References
I. Management
and Diagnostic
Issues
Br Heart J 1958 20: 1-8
¾
Autosomal dominant
¾
LV Hypertrophy: unexplained = Absence of
disease that would be capable of producing the
magnitude of hypertrophy evident in a given
patient
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Non – dilated ventricular chamber
Prognosis
Most common: normal life expectancy, and no
major complications
Complication
Prevention or Intervention
Vtach/Vfib => SCD (most
common ≤ 35 years of age)
• ICD for SCD prevention
Heart failure (diastolic and/or
systolic)
Atrial fibrillation (associated
with stroke)
• Heart failure drugs
• surgical myomectomy or
alcohol septal ablation if heart
failure 2nd to LVOT obstruction
• Heart transplant
• Anticoagulation, heart
rate/rhythm control
• RF ablation
• Surgical maze procedure
Gersh et al: Circulation. 2011;124:e783-831
Management Issues
Counseling (lifestyle, genetic testing)
¾ SCD Risk Assessment and Prevention
¾ Diagnosis and treatment of dynamic LVOT and
cavity obstruction
¾ Prevention, diagnosis, and treatment of heart
Failure and atrial fibrillation
¾ Family counseling and screening of family
members of HCM patients
¾ Monitoring of gene carriers for development of
the HCM phenotype
¾
HCM Management: Role of Imaging
SCD Risk Stratification
CMR, Echo
Diagnosis and monitoring of
heart failure
Echo, CMR
Evaluation of dynamic LVOT
and cavity obstruction
Echo, CMR
Pulmonary Vein Imaging for
treatment of Afib
CT, CMR
Family Screening
Echo, CMR
Monitoring of Gene Carriers for
Development of Hypertrophy
Echo, CMR
Diagnostic Issues
Other Conditions with LV
Hypertrophy
Diagnostic Methods
Athlete’s Heart
EKG, Echo, CMR, deconditioning
Systemic Hypertension
Blood pressure monitoring and
treatment, EKG, Echo, CMR
Aortic Valve Stenosis
Echo, CMR, CT,
Amyloid
EKG, Echo, CMR, pathology
Glycogen Storage Disease
EKG, Echo, CMR, pathology
Noncompaction
Echo, CMR
II. Prevention of
SCD
¾
Secondary prevention:
after the patient had
an arrest
¾
Primary prevention:
before the patient had
an arrest
Personal History: Vfib; sustained Vtach; aborted
SCD
Yes
No
ICD recommended
Risk Stratification for
primary SCD
prevention
ICD
reasonable
indeterminate
Assess potential risk
modifiers
ICD not
reasonable
Risk Stratification for Primary Prevention of
SCD
ICD is reasonable if one of the following applies:
¾
maximal myocardial thickness ≥ 3 cm
¾
Family history of HCM related SCD in one or more firstdegree relative
¾
Personal history of one or more recent unexplained syncopal
episodes
ICD can be useful, particularly if other risk factors or
risk modifiers present, if
¾
¾
NSVT = Ventricular tachycardia (3 or more beats at ≥ 120
bpm) with a duration of less than 30 seconds)
Systolic blood pressure rises < 20 mm Hg or decreases
during exercise
Gersh et al: Circulation. 2011;124:e783-831
Maximal Myocardial Thickness
¾
ECHO
¾
Limited visualization: large patients, apex, anterior wall
Most common site of maximal thickness: anterior
septum
parasternal long axis view
¾
Sometimes difficult delineation of RV border of septum
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¾
¾
CMR
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Entire myocardium visualized
¾
Most common sites of maximal thickness: intersection
of anterior septum and anterior wall; inferior septum
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Short axis view
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Good delineation of RV border of septum
Potential SCD Risk Modifiers
Are considered if conventional risk stratification is
indeterminate
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Delayed enhancement of myocardium on CMR
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Marked LVOT obstruction
¾
Double and compound mutations
CMR: Late
Gadolinium
Enhancement
Delayed Enhancement
¾
> 50% of patients with
HCM
¾
Thought to represent
replacement fibrosis
¾
Caveat: sparse pathologic
data, limited to end-stage
disease)
¾
More common in
hypertrophied areas of
myocardium and related
to extent of hypertrophy
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Inversely related to LVEF
¾
Associated with NSVT and
PVCs (Holter monitoring)
FWHM (dual threshold):
Core tissue: 1.10g ( 1%)
Grayzone: 74.62g (41%)
6 SD/ 4SD (dual threshold)
Core tissue: 39.47g (21%)
Grayzone: 21.12g (11%)