HF

Question 1

what is the recommended screening interval for HCM if genetic testing is negative or clinical family screen is negative.

Answer 1

Every 3 years until 30 years of age except yearly during puberty
After 30 yrs&raquo_space; if symptoms develop

Question 2

if mutation for HCM is present what is the recommended screening interval

Answer 2

Q 3 yrs until 30, except yearly during puberty; every 5 years thereafter

Question 3

Dilated Cardiomyopathy screening interval if negative genetic testing

Answer 3

Every 3 - 5 yrs beginning in childhood.

Question 4

screening interval if +ve genetic testing for dilated cardiomyopathy

Answer 4

yearly in childhood; every 1 - 3 yrs in adults.

Question 5

screening interval for ARVD if genetic testing is negative

Answer 5

Q 3 - 5 years after age 10

if mutation present screen yearly after age 10 to 50

Question 6

Screening recommendation for LVNC

Answer 6

Every 3 years beginning in childhood

if mutation present every yr during childhood, 1 - 3 yrs in adults.

Question 7

screening interval for mutation negative pt for restrictive cardiomyopathy

Answer 7

Every 3 - 5 yrs beginning in adulthood.

if mutation is present screen yearly in childhood; every 1 -3 years in adults.

Question 8

Oxygen consumption cut off during cardiopulmonary stress testing for cardiac transplant consideration

Answer 8

14ml/kg/min in HF patient intolerant to Beta blocker therapy.

12ml/kg/min for patient on beta blocker therapy.

or < 50% age predicted = worse prognosis.

Question 9

Major criteria for dx of ARVD

Answer 9

• dx in a firs degree relative by autopsy or task force criteria.
• detection of genetic mutation

Echo;

• regional RV dyskinesis, akinesis or aneurysm + one of the following
  
  • PLAX RVOT > 32mm
  • PSAX RVOT > 36mm
  • Fractional area change of 33%

Question 10

Loefflers endocarditis

Answer 10

syndrome of hypereosinophillia associated with aggressive thromboembolism, mural thrombus formation, thromboembolism and systemic arteritis

tx with corticosteroids and cytotoxic agents during the acute inflammatory phase.

Question 11

characterized by fibrotic obliteration of the apex of one or both ventricles with associated mural thrombus formation. fibrosis may extend to AV valves affecting their structure.

Answer 11

Endomyocardial fibrosis.

common in equatorial Africa
accounting for 25% of CHF and death

Question 12

This disease may present as restrictive or dilated cardiomyopathy

Answer 12

Sarcoidosis.

characterized by infiltrating non caseating granulomas

tx with steroids and immunosuppressive agents &raquo_space; may help to suppress ventricular arrhythmias and improve myocardial function.

Question 13

patchy involvement of the myocardium in this disease results in high rate of false negative myocardial biopsies

Answer 13

Sarcoidosis.

Question 14

characterized by mutation in cardiac sacomeres?

Answer 14

Hypertrophic cardiomyopathy.

results in various phenotypic expressions.
Most common location: basal anteroseptum continuing to anterolateral wall.

Question 15

why are pts with HCM at increased risk of ischemia

Answer 15

The intramural coronary arterioles are structurally abnormal, with decreased luminal cross-sectional area and impaired vasodilatory capacity resulting in blunted myocardial blood flow during stess (ie. small vessel ischemia).

over time recurrent bouts of small vessel ischemia results in myocyte cell death and ultimately repair in form of replacement fibrosis.

Question 16

90% of mutations causing HCM are described as _______ ? with what kind of transmission?

Answer 16

Missense mutations. most commonly affect are the beta myosin heavy chain and myosin binding protein C (each 30 - 40% of cases) follow Troponin T (15 - 20%)

Autosomal dominant.

Question 17

in the absence of genetic testing what is the recommended screening guideline for HCM

Answer 17

All relatives of pt with HCM must undergo echo screening starting at the onset of puberty Q 1 to 1.5 yrs through end of adolescence (age 12 - 18)

Question 18

what is the recommended screening for patient with HCM + mutation but phenotypically negative by adulthood

Answer 18

continue screening q 5 - 7 years as some mutation present late.

Question 19

Features of athlete heart useful in differentiating from HCM

Answer 19

concentric hypertrophy
LV cavity > 55mm
VO2 > 110%

no late gadolinium enhancement
no LA enlargement
normal LV filling
improves w/ deconditioning

Question 20

indication Myomectomy/alcohol septal ablation

Answer 20

Drug refractory progressive HF symptoms class III/IV gradient >/= 30mmHg at rest or with provocation

Question 21

Indication for ICD in HCM pt

Answer 21

1. Family Hx of SCD
2. Septal diameter > 30mm
3. NSVT
4. Unexplained Syncope
5. Attenuated or hypotensive BP response to exercise

Question 22

CMR can be used in assessment of SCD risk in patients with HCM. what are we looking at?

Answer 22

> 15% of Late gadolinium enhancement is considered elevated risk of SCD.

Question 23

Vast majority of HCM are managed with single chamber devices. which patients are Dual chamber devices reserved for______?

Answer 23

Those with SVT or forced AV pacing is indicated to treat symptoms related to LV outflow tract obstruction.

Question 24

first line therapy for symptomatic HCM

Answer 24

Beta blockers or verapamil

Key: would avoid ccb in patients with marked resting obstruction, advanced heart failure.

Alternative > Disopyramide (parasympathetic side effects may occur limiting its use)

Question 25

Risk of PPM with alcohol septal ablation (ASA)

Answer 25

10%

Question 26

Risk of repeat procedure with ASA

Answer 26

5%

Question 27

Genetic cardiomyopathies

Answer 27

• HCM
• ARVC/D
• LVNC
• Glycogen storage&raquo_space; Danon / PRKAG2
• Conduction defects
• Mitochondrial myopathies
• Ion channel disorders&raquo_space; LQTS / Brugada / SQTS / Asian SUNDS

Question 28

Differentials for acquired primary cardiomyopathies

Answer 28

Inflammatory 
Stress provoked 
peripartum 
tachycardia induced 
Infants of insulin dependent diabetic mothers

Question 29

Mechanism in which cocaine causes cardiomyopathy

Answer 29

multifactorial&raquo_space;

• cathecolamine surge
  
  • Hypertension&raquo_space; increased after load and wall stress
• alteration in G protein signal similar to patients w/ pheo
• Coc assoc. procoagulant effects and coronary vasospasm may provoke ischemic myocardial injury or infarction even in absence of CAD.

Question 30

cancer drugs associated with Cardiomyopathy

Answer 30

Anthracyclines - Doxorubicin, Daunorubicin

Mechanism&raquo_space; enhanced oxidative stress

Question 31

Risk factors for cardiomyopathy 2/2 anthracyclines

Answer 31

• cumulative dose of >550mg/m^2
• extremes of age
• Pts receiving adjuvant radiation or concomitant tx with non anthracycline agents such as taxanes (paclitaxel and Docetaxel) or trastuzumab HER-2

Question 32

X linked cardiomyopathies

Answer 32

mutation in dystrophin gene
- associated with Duchennes muscular dystrophies and Becker muscular dystrophies

Barth Syndrome

• mutation in gene tafazzin which encodes acytltransferase –> besides barth syndrome can also lead to LVNC, DCM, endocardial fibroelastosis
• cardioskeletal myopathy assoc. w/ mitochondrial dysfunction and cyclic neutropenia

Question 33

what is the pathophysiology of permpartum cardiomyopathy

Answer 33

Oxidative stress and impaired angiogenic signaling
- increasing circulating levels of a toxic, proapoptotic fragment of prolactin cleaved by cathepsin D may contribute to enhanced oxidative stress

In mouse models treatment with bromocriptine a dopamine D2 receptor agonist which supresses prolactin production prevents the onset of PPCM