Bristol-Myers Squibb

Home

Understanding HCM

Mavacamten MOA

Clinical Trials

Our Pipeline

Bristol Myers Squibb

Do You Recognize HCM?

Do You Recognize HCM?

Can you determine HCM from an echo? In this challenge, you will decide whether an image shows HCM or not. At the end you’ll be able to see how you stack up against your peers. Ready? All results are anonymous.

QUESTION 1:

Is it HCM?

A.

B.

Echo videos courtesy of Dr. Jay Mukherjee

QUESTION 1:

Is it HCM?

A.

Yes, HCM is present

B.

No

Videos courtesy of Dr. Jay Mukherjee

Clinical evaluation

These videos demonstrate moderate concentric left ventricular (LV) hypertrophy, and asymmetric septal wall hypertrophy; both are possible morphological expressions of HCM, with asymmetric septal wall hypertrophy being the most common form.30 Systolic anterior motion (SAM) is visualized with left ventricular outflow tract (LVOT) obstruction with at least moderate mitral regurgitation. Transesophageal echocardiogram also shows SAM of the mitral valve with moderate to severe mitral regurgitation

Clinical pearl

Abnormalities of the mitral valve apparatus (eg, hypertrophy of the papillary muscles) predispose the leaflets to be swept into the LVOT by drag forces created by a hyperdynamic ejection fraction (EF).31 This results in SAM of the mitral valve or chordate into the LVOT, which is a characteristic of obstructive HCM.31 This phenomenon results in turbulent flow, seen as a mosaic pattern by color flow Doppler.31 SAM also results in distortion of mitral leaflet coaptation, resulting in mitral regurgitation (MR).31 The maximal instantaneous gradient, reflecting the severity of LVOT obstruction, is determined by measuring the peak LVOT velocity.31 This is measured by continuous-wave Doppler.31 Please note that care should be taken to avoid contamination of the LVOT signal with the MR jet.31

LA=left atria; LV=left ventricle; RA=right atria; RV=right ventricle.

QUESTION 2:

Is it HCM?

A.

B.

Video courtesy of Dr. Mariko Harper

QUESTION 2:

Is it HCM?

A.

Yes, HCM is present

B.

No

Videos courtesy of Dr. Mariko Harper

Clinical evaluation

This image shows the same heart a decade ago. By comparison, we can see that the disease progression has become more apparent. Also, we see that it is a less common, though clinically significant, end-stage, profibrotic dilated phenotype of HCM

Clinical pearl

In very advanced cases, HCM can look like many other forms of systolic cardiomyopathy.32 A detailed history, including family history, is key to making the HCM diagnosis.33 Most cases of end-stage HCM with overt dysfunction can deteriorate rapidly and are diagnosed when there is regression of the more classic phenotypic LV thickening32

LVOT=left ventricular outflow tract; PLAX=parasternal long axis.

QUESTION 3:

Is it HCM?

A.

B.

Video courtesy of Dr. Mariko Harper

QUESTION 3:

Is it HCM?

A.

Yes, HCM is present

B.

No

Video courtesy of Dr. Mariko Harper

Clinical evaluation

This image shows the reverse curvature pattern of septal hypertrophy, which is seen in 30% to 40% of patients with HCM, without clear left ventricular outflow tract (LVOT) obstruction.34 The mitral valve apparatus appears relatively normal in this case, without classic anterior leaflet elongation, systolic anterior motion (SAM), or other frequently noted findings in HCM

Clinical pearl

LV thickening, even when involving the septum, does not necessarily mean LVOT obstruction is readily seen.25,35 In fact, based on current literature, one-third of patients with HCM will only demonstrate LVOT obstruction upon exertion, and approximately another one-third do not have any resting or dynamic LVOT obstruction at all (also known as nonobstructive HCM).25 There are also several patterns of LV thickening seen in HCM that are not always the classic sigmoid basal pattern, such as the reverse curvature, apical, or neutral subtype of HCM34

QUESTION 4:

Is it HCM?

A.

B.

Video courtesy of Dr. Mariko Harper

QUESTION 4:

Is it HCM?

A.

Yes, HCM is present

B.

No

Videos courtesy of Dr. Mariko Harper

Clinical evaluation

This is a case where the LV obstruction is more midcavity than in the left ventricular outflow tract (LVOT). There is chordal systolic anterior motion (SAM) noted on the parasternal long axis (PLAX) view image. The initial 4-chamber view suggested significant diastolic dysfunction with severe atrial enlargement and diminished longitudinal LV basal motion

Clinical pearl

The severe biatrial enlargement is a clue that this patient has significant diastolic dysfunction.36 Presence of atrial enlargement should raise suspicion for HCM, especially when there is unexplained LV thickening.22 Depending on the region of LV thickening and the mitral valve anatomy, LV obstruction can also occur more mid- to distal-cavity as seen in the 4-chamber with color flow and continuous wave Doppler image28

CW=color wave.

QUESTION 5:

Is it HCM?

A.

B.

[2020] Copyright material reproduced under a license from Bioscientifica LTD. All rights reserved

Pantazis A et al. Echo Res Pract. 2015;2(1):R45-R53 – Video 7

QUESTION 5:

Is it HCM?

A.

Yes

B.

No, this is cardiac amyloidosis (CA)

[2020] Copyright material reproduced under a license from Bioscientifica LTD. All rights reserved

Pantazis A et al. Echo Res Pract. 2015;2(1):R45-R53 – Video 7

Clinical evaluation

CA can present similarly to HCM in that its echocardiographic features include valvular abnormalities, diastolic dysfunction, and biatrial enlargement.22 The distinguishing diagnostic information for CA is the decreased longitudinal strain in the mid- and basal LV segments with relative preservation of the apical region.22 In this example, concentric LV hypertrophy is shown, with more prominence in the septum. Biatrial enlargement and loss of longitudinal systolic function are also observed35

Clinical pearl

Cardiac amyloidosis, among other possible HCM phenocopies, including Danon disease and Fabry disease, can all be identified with genetic or enzyme testing.36,37 All three are less likely to have significant left ventricular outflow tract obstruction and less likely to demonstrate mitral valve pathology (other than diffuse thickening), but are more likely to be more concentrically thickened than in HCM22,38

Do You Recognize HCM?

Enter your email address below to see how you scored and compared against your peers.

Do You Recognize HCM?

YOU SCORED:

5 of 5

CORRECTLY

83%

YOUR PEERS:

YOU SCORED HIGHER THAN:

72%

OF YOUR PEERS

RETURN TO HOME

CV-US-2100158

INTRO

1

2

3

4

5

FINISH

Bristol-Myers Squibb

© 2021 MyoKardia, Inc, a wholly owned subsidiary of Bristol-Myers Squibb™. All rights reserved. CV-US-2100443 08/21