Iacopo Olivotto,MDReferral Center for Cardiomyopathies
Careggi University Hospital
Florence, Italy
Referral Center for Cardiomyopathies University Hospital of Careggi - Florence
Referral Center for Cardiomyopathies University Hospital of Careggi - Florence
DYNAMIC LV OUTFLOW OBSTRUCTION IN HCM
1958 1969
Referral Center for Cardiomyopathies University Hospital of Careggi - Florence
Am J Cardiol, in press
Centro Riferimento Cardiomiopatie
Cardiologia AOU Careggi - FirenzeReferral Center for Myocardial Diseases
University Hospital of Careggi - Florence
DYNAMIC LV OUTFLOW OBSTRUCTION IN HCM
Referral Center for Cardiomyopathies University Hospital of Careggi - Florence
Provokable Obstruction During Physiologic Exercise
Courtesy of Dr. Stefano Nistri
Circulation, 2006;114:2232
Referral Center for Cardiomyopathies University Hospital of Careggi - Florence
LVOT Obstruction in HCM: a Pathophysiologic Conspiracy
↑ MitralLeaflet Size
↓ LV Outflow Tract Size
↓ LV CavitySize
Referral Center for Myocardial Diseases University Hospital of Careggi - Florence
Hyperdynamic LV
Effects of LVH on Flow Direction
↓ SubaorticCurtain Excursion
Abnormal Papillary MusclesChordal Slack
Centro Riferimento Cardiomiopatie
Cardiologia AOU Careggi - FirenzeReferral Center for Myocardial Diseases
University Hospital of Careggi - Florence
DYNAMIC LV OUTFLOW OBSTRUCTION IN HCM
Centro Riferimento Cardiomiopatie AOU Careggi - Firenze
DYNAMIC LV OUTFLOW OBSTRUCTION IN HCM
ASSOCIATED MITRAL VALVE DISEASE
Referral Center for Myocardial Diseases
University Hospital of Careggi - Florence
Centro Riferimento Cardiomiopatie
Cardiologia AOU Careggi - FirenzeReferral Center for Myocardial Diseases
University Hospital of Careggi - Florence
Referral Center for Cardiomyopathies University Hospital of Careggi - Florence
Circulation, 1991
Referral Center for Cardiomyopathies University Hospital of Careggi - Florence
Circulation, 1993
Referral Center for Myocardial Diseases University Hospital of Careggi - Florence
Mitral Valve Abnormalities Identified by Cardiovascular Magnetic Resonance Represent
a Primary Phenotypic Expression of Hypertrophic Cardiomyopathy
Martin S. Maron, MD, Iacopo Olivotto, MD, Caitlin Harrigan, BA, Evan Appelbaum, MD, C. Michael Gibson, MD,
John R. Lesser, MD, Tammy S. Haas, RN, James E. Udelson, MD, Warren J. Manning, MD and Barry J. Maron, MD
Circulation, 2011
An
terio
r M
itra
l L
ea
fle
t
Length
(m
m)
≤ 20 21-30 31-40 41-50 51-59 ≥6
0
15
20
25
30
35
HCM
Controls
p=0.34
p=0.63
** * * * *
Figure 2
30
Age (years)
Po
ste
rio
r M
itra
l
Le
afle
t L
en
gth
(m
m)
p=0.98
p=0.49
* †* * * * HCM
Controls
≤20 21-30 31-40 41-50 51-59 ≥6015
20
25
Maximum LV Wall Thickness (mm)
Figure 3L
ea
fle
t L
en
gth
(m
m)
5
30
10
20
Anterior (p=0.55)
Posterior (p=0.40)
* ** * † * * * * *
Epicardium-Derived Cells
Normal HCM
Smooth Muscle Cells
Adventitial FibroblastsCoronary Vessels Microvascular Remodeling
Mesenchimal Cells
AV Endocardial CushionMitral Valve Apparatus MV Abnormalities
Interstitial
FibroblastsFibrous Heart Skeleton Fibrosis / Disarray
Purkinje Fibers Arrhythmogenesis ?Instructive Cells
Centro di Riferimento per le Cardiomiopatie AOU Careggi - Firenze
PERI-OPERATIVE EVALUATION
Referral Center for Myocardial Diseases
University Hospital of Careggi - Florence
PERI-OPERATIVE EVALUATION
Referral Center for Myocardial Diseases
University Hospital of Careggi - Florence
Referral Center for Cardiomyopathies University Hospital of Careggi - Florence
The mitral valve is intrinsically abnormal in HCM.
Increased mitral leaftlet dimensions are a main determinant of
dynamic LV outflow obstruction, in the context of a
“pathophysiological conspiracy”.
The etiology of mitral abnormalities is unknown; a developmental
origin may be hypothesized.
Understanding mitral valve pathophysiology in HCM is crucial for
clinical decision making in symptomatic patients with obstruction.
CONCLUSIONS
