Cardiomyopathy and myocarditis

Question 1

How do the ECGs differ between myocarditis and DCM?

Answer 1

Myocarditis: Typically small voltages
DCM: Large voltages reflective of ventricular hypertrophy

Question 2

Location of LGE in myocarditis

Answer 2

Subepicardial, usually on the free wall

Question 3

When are steroids indicated for the treatment of myocarditis?

Answer 3

Giant cell myocarditis

Question 4

Associations:

Myocarditis + New england + CHB + Arthritis

Answer 4

Lyme myocarditis

Question 5

Lyme myocarditis:
- Treatment
- When is IV preferred to oral?

Answer 5

Ceftriaxone x 3 days

IV preferred in 1st degree HB when PR > 300 ms or if higher degree of heart block

Question 6

Associations:

Myocarditis + no improvement for weeks

Answer 6

Giant cell myocarditis

Question 7

Giant cell myocarditis:
- Cause
- Diagnosis
- Treatment
- Prognosis
- Tx candidacy?

Answer 7

Cause: Autoimmune
Diagnosis: cath and biopsy
Treatment: Steroids
Prognosis: Poor
Tx candidacy: Can recur in transplanted heart

Question 8

Associations:

Myocarditis + rub + pleuritic chest pain + abnormal ECG (diffuse ST segment changes)

Answer 8

Myopericarditis

Question 9

Associations:

Myocarditis + LV aneurysm and clot + Latin america

Answer 9

Chagas disease

Question 10

Chagas disease:
Causative agent
Diagnosis
Prognosis

Answer 10

Causative agent: Trypanosome cruzi
Diagnosis: ELISA test
Test: Benznidazole, Nifurtimox
Prognosis: Depends on HF symptomatology

Question 11

How do you differentiate MIS-C from Kawaski disease?

Answer 11

MISC: More likely to be in hispanic or black individuals, more likely to have abdominal pain

MISC platelets are low, KD platelets are high

Question 12

What nutritional deficiency is associated with DCM?

Answer 12

Thiamine deficiency

Question 13

How does DCM appear on pathology?

Answer 13

Myocardial hypertrophy with interstitial fibrosis

Question 14

Where the the LGE located in DCM?

Answer 14

Midwall (septum)

Question 15

Medical treatment of DCM: Asymptomatic and symptomatic

Answer 15

Asymptomatic: ACEi +/- BB
Symptomatic: GDMT

Question 16

Associations:

DCM + difficulty walking + elevated CK

Answer 16

Duchenne muscular dystrophy

Question 17

Duchenne muscular dystrophy:

Etiology and inheritance

Answer 17

Dystrophin mutation (DMD)- located in the cell membrane

X-linked (hence why saw only males)

Question 18

What medication has been found to slow disease progression in Duchennes MD?

Answer 18

Steroids

Question 19

Associations:

DCM + muscle weakness + low ANC + elevated 3-methylglutaconic level

Answer 19

Barth’s syndrome

Question 20

Barth’s syndrome:

Etiology
Inheritance

Answer 20

Etiology: Tefazzin defect (TAZ gene_
X-linked inheritance

Question 21

Associations:

DCM + predominantly right sided disease

Answer 21

Arrythmogenic RV cardiomyopathy

Question 22

Arrythmogenic RV cardiomyopathy:

Cause:
Pathology:
Inheritance
Treatment

Answer 22

Cause: Issue with Desmosomes
Pathology: Fibro-fatty deposits in RV and RVOT
Inheritance: Autosomal dominant
Treatment: GDMT, activity restriction

Question 23

What kind of proteins are most commonly involved in HCM?

Answer 23

Sarcomere - MYPC1, MYH7

Question 24

Describe the classic HCM cath tracing

Answer 24

Gradient as you pull from the LV apex to the base followed by a spike and dome aortic tracing

Question 25

Describe the pathology findings of HCM

Answer 25

Muscle fiber disarray

Question 26

Where is the LGE in HCM?

Answer 26

At the junctions between the RV and LV

Question 27

Most common arrhythmia in HCM

Answer 27

A fib and other atrial arrythmias (but VT is most deadly)

Question 28

What are the only factors found to reduce the risk of SCD in patients with HCM

Answer 28

ICD and exercise restriction

Question 29

How do you differentiate HCM from an athletes heart?

Answer 29

HCM: Will have increased mass to volume ration (athletes hear will have a normal ration)

Thickness decreases when an athlete deconditions

HCM may have LGE but athletes heart does not

Question 30

What is the inheritance pattern of friederichs ataxia?

Answer 30

Autosomal recessive

Question 31

Clinical findings in Pompe disease

Answer 31

Severe HCM, hypotonia, big tongue, big liver

Question 32

Pompe disease
- Etiology
- Diagnosis
- Treatment
- Alternative diagnosis

Answer 32

Etiology: Alpha glucosidase deficiency, lysosomal storage disease
Diagnosis: Low alpha glucosidase
Treatment: Enzyme replacement therapy
Alternative: If alpha glucosidase is normal, may be Dannon syndrome

Question 33

Hurler disease:
- Findings
- Etiology
- Diagnosis
- Related syndromes

Answer 33

Findings: Coarse facial features, developmental delay, hepatosplenomegaly and corneal clouding
- ETiology: Mucopolysaccharide 1. alpha-l-iduronidase deficiencyt. Cannot break down GAG.
- Diagnosis: urinary GAG levels
- Related: If more mild, no corneal clouding -> Hunters

Question 34

What mitral spectral doppler tracing will you find in restrictive CM?

Answer 34

Augmented E wave, increased E:A ratio

Question 35

Differing exam findings in RCM vs. constrictive pericarditis

Answer 35

RCM: S3, loud S2 (2/2 increased PVR)
Constrictive pericarditis: loud “knock” - high pitched

Question 36

Differing cath findings in RCM vs. constrictive pericarditis

Answer 36

RCM: LVEDP > RVEDP, increased PVR
Constrictive pericarditis: LVEDP = RVEDP, square root sign on the LV tracing