Hypertrophic Cardiomyopathy

A Comprehensive Assessment

Kyle W. Klarich, MD
Professor of Medicine
Mayo Clinic, College of Medicine, Rochester, MN
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DISCLOSURE

Relevant Financial Relationship(s)

None

Off Label Usage

List: Manufacturer/Provider & Product/Device
OR None

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 Objectives
What do Clinicians Need from Echocardiography
in Hypertrophic Cardiomyopathy?

1. Make or confirm the diagnosis of HCM

2. Cause of symptoms to target relief
3. Risk stratification for sudden cardiac death
4. Family Screening

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Hypertrophic Cardiomyopathy
What is HCM?
Increased Wall
Increased Wall
Thickness >15
Thickness >15 mm
mm Prevalence in
Asymmetric >> symmetric
Asymmetric symmetric
1/500 people

Risk of SCD
In the absence of 1% annually
identifiable etiology
DDx: Hypertension, Infiltrative CM, Fabry’s disease, Glycogen
Storage diseases, Mitochondrial abnormalities
Marron BJ and McKenna WJ: JACC, 2003
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 Diagnosis of HCM
• What is needed?
• Left ventricular hypertrophy
• Any pattern
• What is not needed?
• Obstruction – LVOT
• Systolic anterior motion
• Asymmetric septal hypertrophy
• Gene positivity
• What must be excluded?
• Hypertension, AS, sub-valvular AS
• Infiltrative disease & storage
diseases
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Pathophysiology of HCM

Obstruction Diastolic Sequelae

SAM*/MR ~100%
~70%

Chest pain
Ischemia
Pressure

Fatigue Dyspnea
MR Dyspnea Fatigue
 LAp  C.O.
 C.O.
Dizziness Dizziness

*SAM = Systolic Anterior Motion ©2018 MFMER | slide-6

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 24 yo Female Presents with Dyspnea
• Never participated in athletics
• Dyspnea while walking with friends
• BMI 29
So What next?
• Medications – oral contraceptive
ECHO,
• No family history ofCMR or CT Scan
heart disease
• Systolic ejection murmur on exam
• ECG LVH with strain; CXR borderline cardiac
enlargement

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Echo Evaluation
Making and Going Beyond the Diagnosis
Echo is first test and 2D exam is where we start
1. Show extent and distribution of LVH
2. Show mitral valve and apparatus
3. Show SAM
4. Show MR jet
5. CW of LV outflow
6. CW of MR jet

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 24 yo female with dyspnea
Always focus on 2D echo initially

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Extent of LVH is Important

A major risk factor for sudden cardiac death

if > 30 mm in any segment
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 24 yo female
Look for Systolic Anterior Motion (SAM) and Timing

LVO

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24 yo female
Look for Systolic Anterior Motion (SAM) and Timing

SAM Septal Contact – Timing into systole informs severity

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 24 yo female
Look for Systolic Anterior Motion (SAM) and Timing

RVOT

Aorta

LA

Premature closure of the aortic valve ©2018 MFMER | slide-13

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Hypertrophic Cardiomyopathy
Echo Defines: anatomic variation

Predicts:
Physiologic consequences
Genetic screening yield

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 Hypertrophic Cardiomyopathy
Echo Defines: Morphologic Heterogeneity Extent & Distribution of LVH

Reverse Curve Morphologic Variant

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Hypertrophic Cardiomyopathy
Echo Defines: Morphologic Heterogeneity Extent & Distribution of LVH

Neutral Septal Morphologic Variant

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 Hypertrophic Cardiomyopathy
Echo Defines: Morphologic Heterogeneity & Distribution of LVH

Sigmoid Septal Morphologic Variant

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Hypertrophic Cardiomyopathy
Echo Defines: Morphologic Heterogeneity Extent & Distribution of LVH

Apical Morphologic Variant

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 Progression of Apical Pouch to Aneurysm
Apical HCM with outpouching, Apical HCM
Apical HCM no aneurysm with aneurysm

LV

LA

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Hypertrophic Cardiomyopathy
Echo Defines: Morphologic Heterogeneity
Apical Morphologic Variant

Apical Pouch Apical Aneurysm

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 Hypertrophic Cardiomyopathy
Echo Defines: Morphologic Heterogeneity Extent & Distribution of LVH

Mid-Ventricular Obstruction

Interruption of Paradoxical
midsystolic flow Jet Flow

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Hypertrophic Cardiomyopathy
Echo Defines: Mitral Valve Anatomic Variation and Abnormalities

• Abnormal papillary muscle insertion

• Hypertrophied papillary muscles
• Multiple papillary muscles
• Abnormal Mitral Valve
• Elongated and redundant

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 Hypertrophic Cardiomyopathy
Echo Defines: MV anatomic variation and abnormalities

Hypertrophied papillary muscles Apically displaced insertion

Subcostal/transgastric oriented views

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Abnormal Mitral Valvular Anatomy

Elongated AML; PML Closes Above the Mitral Annular Plane

Rest Valsalva

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 Echo Evaluation
Making and Going Beyond the Diagnosis
1. Show extent & distribution of LVH
2. Show mitral valve & apparatus
3. Show Sam
4. Show MR jet
5. CW of LV outflow
6. CW of MR jet

Posteriorly directed

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24 yo female with dyspnea

2D and color flow Doppler

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 Unusual Jet of MR
Imaging With 2D, Color & TEE

Unusual MR jet → TEE (class IIa)

SAM related mitral regurgitation is posteriorly directed

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Mitral Regurgitation in HCM

Not Always SAM (Systolic Anterior Motion)
Prolapse ± Infective
Flail leaflets endocarditis

Primary Mitral Valve Lesions

Associated with MR

Abnormal
Anterior leaflet
chordal/papillary
thickening/sclerosis
Muscle support
Important to help select the septal reduction method
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 Echo Evaluation
Making and Going Beyond the Diagnosis
1. Show extent and distribution of LVH
2. Show mitral valve and apparatus
3. Show Sam
4. Show MR jet Next step look at
5. CW of LV outflow the Doppler exam
6. CW of MR jet

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Echo Evaluation
Making and Going Beyond the Diagnosis
Doppler Evaluation
• Resting gradient
• Provoked gradient
• Valsalva Patients have
• Exercise symptoms with
• Pharmacological exertion

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 Dynamic LV Outflow Obstruction
Rest Valsalva Amyl

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HCM Morphology and LVOT Obstruction

Mayo Clinic HCM Database – 2,856 Patients
Mid-cavity
Resting gradient obstruction
<30 mm Hg 2%
Provoked gradient
>30 mm Hg
27%
Non-obstructive
Resting gradient 23%
>30 mm Hg
41%

Apical HCM
7%

Ommen et al: Mayo Clinic, 2006

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 Pursuit in Assessing Obstruction in HCM
If suggestive symptoms

Amyl
Rest No Valsalva No nitrite No Stress No Cath
gradient gradient gradient gradient gradient
>50 mmHg? >50 >50 >50 mmHg >50 mmHg?
mmHg? mmHg?

Yes Yes Yes Yes Yes

Obstructive
physiology Courtesy of J. Geske, MD
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LVOT Doppler MR Doppler

Velocity reflects Velocity reflects
gradient between LV gradient between LV
and Aorta and LA

Highest Velocity Will Always be the MR Jet

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 Estimated LVOT Gradient
Estimated
LVOT Gradient =
LV Pressure – systolic BP
BP~100 LVO vel. 5.5 m/s MR - 7.2 m/s
~15
Estimated
LVOT Gradient = LVOT Velocity
222mmHg – 100mmHg 5.5m/s=121mmHg
222

LV Pressure = MRvel2 + est LA

4(7.2)2 = 207 + 15 = 222mmHg

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Differentiating the MR from the LVOT Doppler

MR LVOT

MR duration is longer than the LVOT velocity

MR is higher velocity than the LVOT velocity
MR shape, although touted may not be as helpful, as may be late
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 24 yo female
CW Doppler of LVOT and MR signals

LVOT velocity = 5.4 m/s MR velocity = 7 m/s

LV Pressure is the highest pressure in heart MR is
the highest velocity signal in the heart
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24 yo female with dyspnea

The next step in the management:

Medical Therapy

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 Dynamic LV Outflow Obstruction
Physiology – Conditions that increase LVO obstruction
More Vigorous
Contraction

Decreased Peripheral
Resistance

Decreased Volume
Eject → Obstruct → Leak

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Strain In HCM

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 Longitudinal Strain
Risk Stratification in HCM and Risk Stratification in HCM

Strain abnormalities correlate with myocardial

fibrosis by DGE on MRI and increase wall
thickness

The presence of strain of ≥-10 in 3/18 segments

correlates with VT as an independent predictor
Sensitivity = 81%
Specificity = 97%

Popovic: JASE, 2008

Di Slavo: JASE, 2010 ©2018 MFMER | slide-41

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Pattern – Strain Reduced in Area of Hypertrophy

Apical HCM with STRAIN

Apical regions significantly reduced strain

Average global longitudinal strain -14

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 How Does Echo Help Determine Septal Reduction Therapy?

Guide Surgical or Interventional Procedures

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Symptomatic HCM Despite Medical Therapy

Septal Reduction Therapy

Obstruction

Anatomy

Abnormal mitral
valve and/or Mid-cavity LVOTO
massive LVH obstruction only
Myectomy
Myectomy Myectomy or Septal
Ablation

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 Septal Myectomy

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Myectomy

Courtesy of Roger Click

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 Post Myectomy Echo

Septal Perforators

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Post Myectomy Echo

Long Axis Midesophageal TEE – post myectomy

Post Myectomy VSD

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 Alcohol Septal Ablation

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Septal
Ablation
Success rate
Structural Anatomy
Anatomy Coronaries

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 Septal perfusion: just right

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Septal perfusion: too much

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 Imaging Informs Screening
Genetic Screening and or Imaging Based
Screening

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Left Ventricular Morphology in HCM

Echo/Imaging predicts the utility of genetic screening
Sigmoid Reverse Neutral Apical
Septum Septum Septum Variant

181 (47%) 132 (32%) 32 (8%) 37 (10%)

Gene + (8%) Gene + (79%) Gene + (41%) Gene + (32%)

Binder et al: Mayo Clin Proc 81:459, 2006

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 Family Screening
Genetic Imaging
Negagive
Screening Based

• <10 years old

• Optional unless malignant family history,
competitive athletes, symptoms, or suspicion
• 10-21 years old
• Every 12 months
• >21 years old
• At onset of symptoms or at least every 5 years
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HCM Echocardiography Key Points:

Prevalence 1:500
Overall prognosis: Good
Echo & Imaging Make Diagnosis
Medication First to treat symptoms
Septal reduction For failed medical Rx
SCD rate Individualized ICD
Family screening 1st degree relatives

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 Questions

&
Discussion
klarich.kyle@mayo.edu

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