Heart Failure

Question 1

What is the indication for ivabridine in heart failure?

Answer 1

Ivabridine is indicated to reduce HF hospitalizations in patients with symptomatic HFrEF and HR >70 in sinus rhythm despite maximally tolerated BB

Question 2

Genes encoding what are involved in ARVC?

Answer 2

Desmosomes

Question 3

Classic ECG findings for ARVC

Answer 3

T wave inversions and epsilon waves in right precordial and inferior leads

Question 4

Indications for ICD in HCM (7 total)

Answer 4

1. LV wall thicknesss > 3cm
2. Extensive LGE
3. Prior unexplained syncope
4. SCD in one or more close relatives
5. HCM with EF < 50%
6. LV apical aneurysm
7. NSVT on monitoring

Question 5

Name some genes associated with HCM

Answer 5

-Myosin heavy chain beta 7 (MYH7)
-myosin binding protein c3 (MYBPC3)
-troponin I3 (TNNI3)
-troponin T2 (TTNT2)
-tropomyosin 1 (TPM1)
-myosin light chain 2 (MYL2) and 3 (MYL3)
- actin alpha cardiac muscle 1 (ACTC1)

Question 6

Galactosidase alpha (GLA) mutation is associated with which cardiomyopathy?

Answer 6

Fabry Disease

Question 7

Protein kinase AMP-activated non-catalytic subunit gamma 2 (PRKAG2) is associated with which cardiomyopathy?

Answer 7

Glycogen storage disease

Question 8

Fibrillin 1 mutation is associated with…?

Answer 8

Marfan’s

Question 9

SCN5A mutation is associated with…?

Answer 9

Long QT syndrome type 3 and ARVC

Question 10

name two ECG features of ATTR amyloid

Answer 10

low voltage; pseudo infarct pattern

Question 11

How should patients who have a first degree relative with dilated CM be screened?

Answer 11

TTE every 3 years; genetic testing of the proband only; routine genetic testing of relatives not recommended except select cases

Question 12

what are indications for EMBx in heart failure?

Answer 12

-New onset of unexplained CHF of <2 weeks’ duration associated with hemodynamic compromise;
-Unexplained new-onset HF between 2 weeks’ and 3 months’ duration that is associated with a dilated left ventricle and new bradyarrhythmia, new ventricular arrhythmias, or a failure to respond to standard care within 1-2 weeks of diagnosis; and
-HF that is rapidly progressing when there is a high suspicion that the cause can only be confirmed by histology.

Question 13

what is first line therapy for ARVC and arrythmias/appropriate shocks?

Answer 13

beta-blocker

Question 14

what two antiarrhythmics can be used for afib in heart failure?

Answer 14

amiodarone and dofetilide

Question 15

what four antiarrhythmics can be used for afib in patients with CAD

Answer 15

dofetilide, dronedarone, sotalol, and if those don’t work, amiodarone

Question 16

Next best step for diagnosing ARVC in a patient with suggestive features

Answer 16

Genetic testing (EMBx is much less sensitive/specific)

Question 17

what does mutation of lamin A/C cause?

Answer 17

dilated cardiomyopathy (autosomal dominant inheritance)

Question 18

how does Entresto affect BNP

Answer 18

BNP is degraded by neprilysin so Entreso increases BNP (though does not affect NT-proBNP)

Question 19

galactic-3 is a marker of…

Answer 19

cardiac fibrosis

Question 20

Notch-1 mutation is associated with…

Answer 20

bicuspid aortic valve and early aortic valve calcification

Question 21

Tbox-5 mutation (long arm of chromosome 12) is associated with…

Answer 21

Holt-Oram which is associated with limb deformities, ASD/VSD and HCM

Question 22

Collagen 3A1 (COL3A1) is associated with

Answer 22

the vascular form of Ehler’s Danlos