Cardiovascular dz differential diagnosis

[MudPhud20XX]

It came from one of the q from Kaplan.

A 32 year old ice skater suddenly dies on the ice during a performance in which he has lifted his wife over his head into the air many times. At autopsy, what would most likely be found in his heart?

Differential diagnosis? Or any idea? Many thanks in advance.

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[pd1112]

SCD DDx:
structural heart disease -> HCM
vascular disease -> aortic aneurysm, aortic dissection
SCD with apparently normal heart @ autopsy -> arrhythmia

 

[Radiolucent]

It came from one of the q from Kaplan.

A 32 year old ice skater suddenly dies on the ice during a performance in which he has lifted his wife over his head into the air many times. At autopsy, what would most likely be found in his heart?

Differential diagnosis? Or any idea? Many thanks in advance.

There are multiple causes for sudden death but Ill try to list those most HY for Step 1, others feel free to chime in.

The one cause all the questions Ive done seem to hit the hardest is hypertrophic cardiomyopathy, especially in athletes. Actually I would say that sudden death in an athlete = HCM.

A massive myocardial infarction is probably the overall most common cause of sudden death but less so in a young person. A massive stroke could also cause sudden death and have the same risk factors (+ afib).

Massive pulmonary embolism can also cause sudden death but the patient would likely have some concurrent risk factors (cancer, hypercoag, recent surgery etc).

In your q Id go for HCM.

 

[MudPhud20XX]

Good thinking guys... Yeah I was thinking HCM, so I was looking for a description of it such as "myofiber hypertrophy with disaaray."

Here are the answer choices:

A. Acute thrombosis of the LAD
B. Aortic outflow obstruction
C. Chronic ischemic heart dz
D. Infective myocarditis
E. Mitral valve prolapse

Now what do you guys think? Many thanks.

 

[Radiolucent]

Good thinking guys... Yeah I was thinking HCM, so I was looking for a description of it such as "myofiber hypertrophy with disaaray."

Here are the answer choices:

A. Acute thrombosis of the LAD
B. Aortic outflow obstruction
C. Chronic ischemic heart dz
D. Infective myocarditis
E. Mitral valve prolapse

Now what do you guys think? Many thanks.

B.
One subtype of HCM is obstructive HCM aka HOCM. Generally in HCM the interventricular septum is the part that hypertrophies the most. When the anterior mitral leaflet gets too close to the septum (systolic anterior motion) --> aortic outflow obstruction.

Check this image out if youre having trouble visualising it:
http://www.scancrit.com/wp-content/uploads/2013/04/hypertrophy2.gif

 

[seminoma]

Agree with HCM. It might be helpful to think of HCM as a sub-aortic valve aortic stenosis.

 

[sloop]

Hypertrophic cardiomyopathy is my #1, followed by arrhythmia. This patient is somewhat older than the "classic" patient (usually a teenage athlete) for hypertrophic cardiomyopathy, but the presentation of sudden death in an otherwise presumably healthy person in his 4th decade of life has few better explanations.

Arrhythmia is always a possibility in sudden death, but we don't really have great reason to suspect arrhythmia. I might think differently if he had a history of syncope or something that might suggest Wolff-Parkinson-White Syndrome or if he was taking digoxin.

Other possibilities all would typically require some other symptoms or a PMH with risk factors:

Aortic dissection/aneurism (no marfanoid habitus described, no chronic HTN)
Ventricular rupture would typically have previous MI
etc.

 

[sloop]

Good thinking guys... Yeah I was thinking HCM, so I was looking for a description of it such as "myofiber hypertrophy with disaaray."

Here are the answer choices:

A. Acute thrombosis of the LAD
B. Aortic outflow obstruction
C. Chronic ischemic heart dz
D. Infective myocarditis
E. Mitral valve prolapse

Now what do you guys think? Many thanks.

If those are the answer choices, it's definitely B. Hypertrophic cardiomyopathy causes the anterior leaflet of the mitral valve to obstruct the aortic outflow tract.

Agree with HCM. It might be helpful to think of HCM as a sub-aortic valve aortic stenosis.

What do you mean by this? The obstruction in HCM is due to the mitral valve, not a stenotic outflow tract. HCM causes dynamic obstruction of the outflow tract, not static obstruction. That's the reason the prototypical "young athletes" are typically asymptomatic until they suddenly die on the field one day.

 

[pd1112]

.

 

[pd1112]

What do you mean by this? The obstruction in HCM is due to the mitral valve, not a stenotic outflow tract. HCM causes dynamic obstruction of the outflow tract, not static obstruction. That's the reason the prototypical "young athletes" are typically asymptomatic until they suddenly die on the field one day.

LVOT/aortic stenosis can be valvular, subvalvular, or supravalvular. HCM and subaortic stenosis are the two categories of subvalvular LVOT. The name HCM doesn't tell you anything about the physiology, but if you remember to think of it as a subvalvular stenosis, it's easier to remember its effect.
Was that what you were going for
@seminoma ?

 

[seminoma]

If those are the answer choices, it's definitely B. Hypertrophic cardiomyopathy causes the anterior leaflet of the mitral valve to obstruct the aortic outflow tract.



What do you mean by this? The obstruction in HCM is due to the mitral valve, not a stenotic outflow tract. HCM causes dynamic obstruction of the outflow tract, not static obstruction. That's the reason the prototypical "young athletes" are typically asymptomatic until they suddenly die on the field one day.

It's generally hypertrophy of the IV septum, including the area immediately below the valve orifice. I was just relaying the same message that Pathoma does ("HCM = functional aortic stenosis").

 

[Jabbed]

If those are the answer choices, it's definitely B. Hypertrophic cardiomyopathy causes the anterior leaflet of the mitral valve to obstruct the aortic outflow tract.



What do you mean by this? The obstruction in HCM is due to the mitral valve, not a stenotic outflow tract. HCM causes dynamic obstruction of the outflow tract, not static obstruction. That's the reason the prototypical "young athletes" are typically asymptomatic until they suddenly die on the field one day.

The mitral valve can obstruct the LVOT (HCM with SAM), but it is the asymmetric septal hypertrophy that is the root cause of the obstruction.

 

[MudPhud20XX]

Alright, so the way pathoma explains HCM is: Lt ventricular hypertrophy leads to diastolic dysfunction (filling problem), so is answer choice B really explaining HCM? Please enlighten me.

 

[seminoma]

Alright, so the way pathoma explains HCM is: Lt ventricular hypertrophy leads to diastolic dysfunction (filling problem), so is answer choice B really explaining HCM? Please enlighten me.

It is a diastolic dysfunction. A proportion of patients with HCM have HOCM (obstructive) due to LVOT. That proportion of patients are the ones that die of SCD. The others are just diastolic heart failure patients. At least that's how I've understood it.

 

[MudPhud20XX]

Dang, my bad. Sattar does say interventricular septum involvement right beneath the aortic valve leading to "functional aortic stenosis." Though I don't really understand why enlargement of the Lt. ventricular leads to preferential involvement of interventricular septum close to the aortic valve.

 

[nds86]

I think there was a ques specifically for HOCM in Uworld and it is Px in FA 2015. If there is an obstruction then it is usually below the AV valve. As Goljan explains, blood exits LV, and the anterior leaflet of the MV is drawn against hypertrophied IVS.

 

[seminoma]

Dang, my bad. Sattar does say interventricular septum involvement right beneath the aortic valve leading to "functional aortic stenosis." Though I don't really understand why enlargement of the Lt. ventricular leads to preferential involvement of interventricular septum close to the aortic valve.

LV enlargement doesn't lead to preferential involvement of anything. The disease process involve IV septum preferentially, for whatever reason, and that same process throughout the LV leads to LV enlargement.

 

[MudPhud20XX]

ok ok got it. Thanks man.

 

[Transposony]

Here is some interesting discussion on the topic: http://forums.studentdoctor.net/threads/murmurs-at-left-sternal-border.1129706/

 

[Radiolucent]

I would like to add one more thing to the discussion:

As have been mentioned arrythmias due to channelopathies (hereditary QT-syndromes) and abnormal conduction systems (WPW) can cause SCD. An important cause of arrythmia-caused SCD especially in young athletes that can be relevant for the boards is Brugada syndrome. It was one of the new additions to FA this year so therefore its likely to appear on the real thing.

If you see a RBBB with ST-elevation on lead v1-v2 (the ST-segment normally is depressed on v1-v2 in RBBB) = Brugada syndrome.

Brugada: http://2.bp.blogspot.com/-Ui34BK2Fx...DMf_fPQ/s1600/Fig-1-Brugada-(8-7.44-2012).jpg

RBBB: http://3.bp.blogspot.com/_A8JQOadS5...ion+Review+-+3+-+BBB%2C+ST-T+wave+changes.jpg