Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

BCC > Hypertrophic CM > Flashcards

Hypertrophic CM Flashcards

1
Q

What characterizes hypertrophic cardiomyopathy?

A

LV hypertrophy + absence of another cardiac, systemic, or metabolic disease producing the magnitude of hypertrophy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the prevelance of hypertrophic CM?

A

0.20%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

How is hypertrophic CM passed on?

A

Autosomal dominant with variable expression and age-releated penetrance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the pathophysiology of hypertrophic CM?

A

11 different sarcomeric mutations have been identified but only 30% of hypertrophic CM patients have a genetic etiology

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Who should get tested for hypertrophic CM?

A

Hypertrophic CM screening is recommended for 1st-degree and other close relatives; they should get an echo and a cardiac MRI (not genetic testing)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the histologic pathognomonic finding for hypertrophic CM?

A

Myocardial fiber disarray with interstitial and perivascular fibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the most common phenotypic variant for hypertrophic CM?

A

Asymmetric hypertrophy (septum and anterior wall, 70%) > isolated basal septal hypertrophy (15-20%) > midventricdular septal hypetrophy (8-10%) > apical hypertrophy (<2%)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Who do you see apical hypertrophy more often in?

A

East Asian patients (<2% of overall patients)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the most common location of hypertrophy in hypertrophic CM?

A

Basal anterior septum with continuity with the anterior free wall

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are patients with hypertrophic CM at risk for?

A

Sudden cardiac death, LVOT obstruction, diastolic dysfunction, heart failure with systolic dysfunction, ventricular arrhythmias, atrial fibrillation with increased risk of stroke

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What imaging findings are suggestive of increased risk of sudden cardiac death in patients with hypertrophic CM?

A
  1. Massive LVH (>/= 30mmHg)
  2. LV apical aneurysm
  3. systolic dysfunction (LVEF <50%)
  4. history of suspected cardiac syncope or family history of sudden cardiac death
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Which patients with hypertrophic CM are at a higher risk of lifelong adverse events?

A

Patients with pathogenic sarcomeric gene variants and those diagnosed earlier in life

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What % of patients have LVOT obstruction with hypertrophic CM?

A

75%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the peak LVOT gradient indicative of obstruction?

A

> /= 30mmHg (>/= 50mmHg is severe obstruction)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the most common cause of obstruction in hypertrophic CM?

A

Mitral-septal contact secondary to systolic anterior motion (SAM)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the principal mechanisms responsible for LVOT obstruction?

A
  1. Septal hypertrophy with narrowing of the LVOT; 2. Anatomic alterations in the MV apparatus (i.e. longer leaflets, anterior displacement of the pap muscles and MV apparatus)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What does systolic anterior motion of the MV lead to?

A

LVOT obstruction and MR (due to loss of leaflet coaptation)

18
Q

What do you see on CW through the AV with LVOT obstruction?

A

Dagger-shaped or Shark-toothed profiled

19
Q

What are other causes of LVOT obstruction outside of SAM?

A
  1. Anomalous papillary muscle (causes obstruction because of systolic apposition of the pap muscle and septum); 2. Apical hypertrophic CM (systolic apposition of the mid-LV walls leads to apical blood trapping with high apical chamber pressures leading to apical akinesis)
20
Q

Why does diastolic dysfunction occur with hypertrophic CM?

A

Secondary to myocardial hypertrophy + ischemia + fibrosis + delayed inactivation from abnormal intracellular Ca reuptake + altered systolic-diastolic coupling

21
Q

What does the MR jet look like with SAM?

A

The regurgitant jet is during mid-systole and posteriorly or laterally directed

22
Q

Why do you get myocardial ischemia with hypertrophic CM?

A

Mismatch between O2 supply and demand; there is hypertrophy and microvascular dysfunction with impaired coronary flow reserve + arteriolar intimal and medial hyperplasia + myocardial bridging

23
Q

What is myocardial bridging?

A

Overlying myocardial causing systolic compression of an epicardial coronary artery (can impair blood flow and cause ischemia)

24
Q

What types of autonomic dysfunction do you see with hypertrophic CM?

A

Impaired HR recovery + inappropriate vasodilation + abnormal BP response to exercise (failure to increase SBP by 20mmHg or a drop in SBP during exercise > 20mmHg is a poor prognostic factor)

25
Q

What is the rate of cardiac death due to hypertrophic CM in young athletes?

A

1/3 of non-trauma-related sudden cardiac death in young, asymptomatic athletes

26
Q

How is hypertrophic CM diagnosed?

A

Echo and/or cardiac MRI

27
Q

What increases your risk of LVOT obstruction?

A

Increased contractility (i.e. high catecholamine states) + decreased preload + decreased afterload + increased HR + absence of atrial kick

28
Q

What do you see on echo in hypertrophic CM?

A

Increased LV wall thickness + LVOT obstruction + quantitative peak LVOT gradients + MR

29
Q

What do you see on cardiac MRI in hypertrophic CM?

A
  1. Increased ventricular wall thickness (better than echo)
  2. Morphologic features in gene carriers with LVH (i.e. blood-filled myocardial crypts, elongated mitral leaflets, expanded extracellular space)
  3. Quantify LV mass
  4. Myocardial tissue scarring/fibrosis via late gadolinium enhancement
30
Q

What are common pharmacologic interventions to treat hypertrophic CM patients?

A

Beta blockers (first line), calcium channel blockers, disopyramide

31
Q

What procedures can help with hypertrophic CM patients?

A

Septal reduction therapy such as surgical septal myectomy or alcohol septal ablations + dual chamber pacing (via a short AV delay –> significantly reduces outflow tract gradients; not a first line treatment though)

32
Q

What treatment option for hypertrophic CM prolongs life expectancy?

A

ICD therapy (Class 1a in patients with hypertrophic CM with cardiac arrest or sustained VT and Class 2a in patients with one of the following: massive LV hypertrophy (>/= 30mm), history of suspected cardiac syncope, LV apical aneurysm, systolic dysfunction with LVEF < 50%, or family history of sudden cardiac death due to hypertrophic CM)

33
Q

When is an ICD placement considered a Class 2b indication for hypertrophic CM?

A

In adults with non-sustained VT or in patients with extensive late gadolinium enhancement on cardiac MRI

34
Q

Should you place an ICD in a child who has non-sustained VT due to hypertrophic CM?

A

Class 2a so it is reasonable to

35
Q

What are the indications for surgical myectomy for hypertrophic CM?

A

NYHA functional class III or IV AND severe resting or provoked LVOT obstruction (>/= 50 mmHg) despite maximal medical treatment

36
Q

What is the mortality rate of a septal myectomies at experienced centers?

A

<1%

37
Q

What are the complications of septal myectomies?

A

Complete heart block + LBBB + VSDs

38
Q

On TEE after septal myectomies, how do you tell the difference between a VSD and septal perforators?

A

Septal perforators will show color flow during diastole while VSDs will have color flow during systole

39
Q

When should a patient get an alcohol septal ablation for hypertrophic CM?

A

Severe symptoms despite optimal medical therapy + poor surgical candidates

40
Q

What is better, septal myectomies or alcohol septal ablations?

A

Septal myectomies are superior

41
Q

How is an alcohol septal ablation performed?

A

Prominent anteroseptal-perforating branch of the LAD supplying the hypertrophied portion of basal septum is injected with 1-3cc of ethanol

42
Q

What are complications of alcohol septal ablations?

A

Increased risk of AV block (mostly RBBB) + LAD dissection + reflux of alcohol back into the LAD resulting in massive anterior infarction

BCC (29 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions