Infiltrative and Restrictive Cardiomyopathy Cardiomyopathy •Least common of the cardiomyopathies...
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Transcript of Infiltrative and Restrictive Cardiomyopathy Cardiomyopathy •Least common of the cardiomyopathies...
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Gerard P. Aurigemma MD, FASE, FAHA, FACC
University of Massachusetts Medical School
Infiltrative and
Restrictive
Cardiomyopathy:
Recognition by Echo
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2 Garcia JACC 2016
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City of Worcester, MA
Mobile Strain Screening
Program
4
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Restrictive
Cardiomyopathy in
Clinical Practice 2017
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80 year old man with
DOE and new onset AF
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74 year old man s/p TAVR
Still has HF
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88 year old initial
visit at TAVR clinic
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58 year old with dizziness and presyncopal symptoms
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19 year old with chest pain and dyspnea
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Restrictive Cardiomyopathy
• Least common of the cardiomyopathies
• The cardiac chambers cannot stretch
normally = stiff/noncompliant
• Filling at normal pressure is limited
• Normal LV and RV chamber size
• Atrial enlargement – reflects increased
ventricular filling pressures/atrial pressure
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13 Garcia JACC 2016
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Pathophysiology
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90% x small EDV = small SV
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Rise in PCWP Flat stroke volume response
to exercise
Kitzman Circulation 1991
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Case 1
• 80 year old male with
mild AS, COPD not on O2
who presented with with
DOE x 1 month.
• new Afib that was rate
controlled.
• Sudden dyspnea after
CXR
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ECG
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The ‘Transducer of Truth’
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TTE
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23
4 years ago Now
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RHC
• RA 6 mmHg
• RV 26/9 mmHg
• PA 27/11/16 mmHg
• PCWP 14 mmHg
• Fick CO 3.82, CI 2.16
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Whole Body Scan
• Degenerative change
in the right shoulder,
otherwise normal
scan.
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Light Chain (AL)
• Fibrills composed of light
chain Ig.
• Produced by clonal
population of plasma cells.
• Treatment with
chemotherapy.
Transthyretin related
(ATTR)
• Familial (ATTRm)
• Wild/Senile type (ATTRwt)
• Mutation of the TTR
protein
• ATTRm all ages
• ATTRwt mostly old age
Amyloidosis
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74 year old man s/p TAVR
Still has HF
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88 year old initial
visit at TAVR clinic
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LVEF: GLS ( 6:1)
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4 Questions • Can you tell types apart?
• Why the gradient?
• Is wall thickness all due to
amyloid deposition?
• Do they benefit from
TAVR?
40
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• GLS correlates with
WT
• TTRwt had worst
GLS but best
outcome
• Light chain toxicity
in AL might be
important
mechanism
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LVEF: GLS ( 6:1)
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47 Garcia JACC 2016
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Case 2 • 43 year old man
• Excessive EtOH usage
• Abdominal discomfort
• Now DOE
• + troponin QuickTime™ and aMicrosoft Video 1 decompressorare needed to see this picture.
48
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EKG
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Case The Attorney
58 year old with dizziness and presyncopal symptoms left leg pain, numbness and swelling in both lower extremities.
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Fabry’s Disease
• Fabry Disease
• Anderson-Fabry
Disease
• α-galactosidase A
deficiency
• Leukocyte levels
• Angiokeratosis
• X-linked recessive
• Affects predominantly
males
• Affects homozygous
females
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Fabry Disease Cardiac
Manifestations • Left ventricular hypertrophy
• Hypertrophic cardiomyopathy, generally
symmetric.
• Conduction defects: Short PR interval, Qt
prolongation often with RBBB,
tachyarrythmias, SCD.
• Aortic root dilatation
• Aortic and mitral valve insufficiency
• Ischemic disease, either due to endothelial
dysfunction, microvasculature dysfunction or
secondary to severe LV hypertrophy.
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19 year old with chest pain and dyspnea