CHF/Cardiomyopathy (19%)

Question 1

What is the first line medical treatment for lupus myocardiits?

Answer 1

Systemic glucocorticoids

many would also add cyclophosphamide to steroid therapy

Question 2

What medication to add to HFfEF patient with EF<35% on maximally tolerated b-blocker and other GDMT and has resting HR >70bpm?

Answer 2

Ivabradine

funny current in SA note inhibitor

Question 3

Which medical condition from physical finding?

prominent v wave with a sharp y descent.

Answer 3

Tricuspid regurgitation.

The pressure is transmitted back to the atrium, causing a giant c-V wave (called the Lancisi sign)

Question 4

HFrEF, class II or III, tolerating ACE or ARB, what medication is recommended( (Class I)?

Answer 4

valsartan/sacubitril
Reduces morbidity and mortality.
PARADIGM-HF trial.
Stop ACEi 36hrs prior to starting valsartan/sacubitril
ARNI=angiotensin receptor-neprilysin inhibitor)

Question 5

What medical condition from physical finding?

elevated systemic venous pressure with sharp y descent Kusmaul sign and pericardial knock?

Answer 5

Constrictive pericarditis
the rapid y suggests rapid ventricular filling (so, constrictive pericarditis or restrictive cardiomyopathy), but 50% of patients with constrictive pericarditis have a pericardial knock (not seen in rcm).

Question 6

Which trial?

ICD use for primary prevention is more effective in NYHA Class II than in patients with NYHA Class III.

Answer 6

SCD-HeFT

Question 7

S/P orthotopic heart transplant distantly (over 1 year), presenting with CHF symptoms, LBBB, and low EF with regional wall motion abnormality. What is most likely diagnosis?

Answer 7

allograft vasculopathy.

rejection is generally earlier ~6-12 months

Question 8

CHF, darker complexion despite not going outside much, DCM, normal coronaries, hepatosplenomegaly, diabetes diagnosed within past several months.
What unifying diagnosis?

Answer 8

Hemachromatosis

Hemachromatosis is Autosomal Recessive

Question 9

Which trial?

Either continuous or bolus furosemide can be used to good effect in acute decompensated CHF.

Answer 9

DOSE study.

Question 10

What is the drug of choice for beta-blocker toxicity?

Answer 10

Glucagon

stimulates cAMP through nonadrenergic pathways

Question 11

What is the genetic characterization for HCM?

Answer 11

Autosomal Dominant (AD)
Leads to altered or reduced amount of sarcomere protein.

Question 12

What is the gene associated with autosomal dominant ARVC?

Answer 12

plakophilin-2

plakophilin-2, desmoglein-2, desmocolin-2, TMEM43

Question 13

What is the gene associated with autosomal recessive ARVC?

Answer 13

Plakoglobulin and Desmoplakin

name for AR ARVC is Naxos Disease

Question 14

What is the receptor associated with Catecholaminergic VT (CPVT)?

Answer 14

Ryanodine Receptor

Causes Calcium Overload, thus polymorphic VT

Question 15

What is the most common genetic mutation for HCM?

Answer 15

MYBPC3 gene.
Mutation in the cardiac myosin binding protein C gene.
MY Binding Protein C

Question 16

What are the 3 criteria for positive response to vasodilator therapy in pulmonary hypertension (Challenge when PASP>50, TPG >15, or PVR >3 Woods Units while maintaining SBP >85)?

Answer 16

1. Fall in Mean PAP of at least 10mmHg
2. Mean PAP goes below 40mmHg
3. No change or an increase in Cardiac Output

ALL THREE MUST BE PRESENT TO QUALIFY.

Question 17

Which cardiomyopathy?

Accumulation of globotriaosylceramide.

Answer 17

Fabry’s Disease