Cardiomyopathy and myocarditis Flashcards
How do the ECGs differ between myocarditis and DCM?
Myocarditis: Typically small voltages
DCM: Large voltages reflective of ventricular hypertrophy
Location of LGE in myocarditis
Subepicardial, usually on the free wall
When are steroids indicated for the treatment of myocarditis?
Giant cell myocarditis
Associations:
Myocarditis + New england + CHB + Arthritis
Lyme myocarditis
Lyme myocarditis:
- Treatment
- When is IV preferred to oral?
Ceftriaxone x 3 days
IV preferred in 1st degree HB when PR > 300 ms or if higher degree of heart block
Associations:
Myocarditis + no improvement for weeks
Giant cell myocarditis
Giant cell myocarditis:
- Cause
- Diagnosis
- Treatment
- Prognosis
- Tx candidacy?
Cause: Autoimmune
Diagnosis: cath and biopsy
Treatment: Steroids
Prognosis: Poor
Tx candidacy: Can recur in transplanted heart
Associations:
Myocarditis + rub + pleuritic chest pain + abnormal ECG (diffuse ST segment changes)
Myopericarditis
Associations:
Myocarditis + LV aneurysm and clot + Latin america
Chagas disease
Chagas disease:
Causative agent
Diagnosis
Prognosis
Causative agent: Trypanosome cruzi
Diagnosis: ELISA test
Test: Benznidazole, Nifurtimox
Prognosis: Depends on HF symptomatology
How do you differentiate MIS-C from Kawaski disease?
MISC: More likely to be in hispanic or black individuals, more likely to have abdominal pain
MISC platelets are low, KD platelets are high
What nutritional deficiency is associated with DCM?
Thiamine deficiency
How does DCM appear on pathology?
Myocardial hypertrophy with interstitial fibrosis
Where the the LGE located in DCM?
Midwall (septum)
Medical treatment of DCM: Asymptomatic and symptomatic
Asymptomatic: ACEi +/- BB
Symptomatic: GDMT
Associations:
DCM + difficulty walking + elevated CK
Duchenne muscular dystrophy
Duchenne muscular dystrophy:
Etiology and inheritance
Dystrophin mutation (DMD)- located in the cell membrane
X-linked (hence why saw only males)
What medication has been found to slow disease progression in Duchennes MD?
Steroids
Associations:
DCM + muscle weakness + low ANC + elevated 3-methylglutaconic level
Barth’s syndrome
Barth’s syndrome:
Etiology
Inheritance
Etiology: Tefazzin defect (TAZ gene_
X-linked inheritance
Associations:
DCM + predominantly right sided disease
Arrythmogenic RV cardiomyopathy
Arrythmogenic RV cardiomyopathy:
Cause:
Pathology:
Inheritance
Treatment
Cause: Issue with Desmosomes
Pathology: Fibro-fatty deposits in RV and RVOT
Inheritance: Autosomal dominant
Treatment: GDMT, activity restriction
What kind of proteins are most commonly involved in HCM?
Sarcomere - MYPC1, MYH7
Describe the classic HCM cath tracing
Gradient as you pull from the LV apex to the base followed by a spike and dome aortic tracing
Describe the pathology findings of HCM
Muscle fiber disarray
Where is the LGE in HCM?
At the junctions between the RV and LV
Most common arrhythmia in HCM
A fib and other atrial arrythmias (but VT is most deadly)
What are the only factors found to reduce the risk of SCD in patients with HCM
ICD and exercise restriction
How do you differentiate HCM from an athletes heart?
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
What is the inheritance pattern of friederichs ataxia?
Autosomal recessive
Clinical findings in Pompe disease
Severe HCM, hypotonia, big tongue, big liver
Pompe disease
- Etiology
- Diagnosis
- Treatment
- Alternative diagnosis
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
Hurler disease:
- Findings
- Etiology
- Diagnosis
- Related syndromes
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
What mitral spectral doppler tracing will you find in restrictive CM?
Augmented E wave, increased E:A ratio
Differing exam findings in RCM vs. constrictive pericarditis
RCM: S3, loud S2 (2/2 increased PVR)
Constrictive pericarditis: loud “knock” - high pitched
Differing cath findings in RCM vs. constrictive pericarditis
RCM: LVEDP > RVEDP, increased PVR
Constrictive pericarditis: LVEDP = RVEDP, square root sign on the LV tracing
