HF Flashcards
what is the recommended screening interval for HCM if genetic testing is negative or clinical family screen is negative.
Every 3 years until 30 years of age except yearly during puberty
After 30 yrs»_space; if symptoms develop
if mutation for HCM is present what is the recommended screening interval
Q 3 yrs until 30, except yearly during puberty; every 5 years thereafter
Dilated Cardiomyopathy screening interval if negative genetic testing
Every 3 - 5 yrs beginning in childhood.
screening interval if +ve genetic testing for dilated cardiomyopathy
yearly in childhood; every 1 - 3 yrs in adults.
screening interval for ARVD if genetic testing is negative
Q 3 - 5 years after age 10
if mutation present screen yearly after age 10 to 50
Screening recommendation for LVNC
Every 3 years beginning in childhood
if mutation present every yr during childhood, 1 - 3 yrs in adults.
screening interval for mutation negative pt for restrictive cardiomyopathy
Every 3 - 5 yrs beginning in adulthood.
if mutation is present screen yearly in childhood; every 1 -3 years in adults.
Oxygen consumption cut off during cardiopulmonary stress testing for cardiac transplant consideration
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.
Major criteria for dx of ARVD
- 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%
Loefflers endocarditis
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.
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.
Endomyocardial fibrosis.
common in equatorial Africa
accounting for 25% of CHF and death
This disease may present as restrictive or dilated cardiomyopathy
Sarcoidosis.
characterized by infiltrating non caseating granulomas
tx with steroids and immunosuppressive agents »_space; may help to suppress ventricular arrhythmias and improve myocardial function.
patchy involvement of the myocardium in this disease results in high rate of false negative myocardial biopsies
Sarcoidosis.
characterized by mutation in cardiac sacomeres?
Hypertrophic cardiomyopathy.
results in various phenotypic expressions.
Most common location: basal anteroseptum continuing to anterolateral wall.
why are pts with HCM at increased risk of ischemia
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.
90% of mutations causing HCM are described as _______ ? with what kind of transmission?
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.
in the absence of genetic testing what is the recommended screening guideline for HCM
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)
what is the recommended screening for patient with HCM + mutation but phenotypically negative by adulthood
continue screening q 5 - 7 years as some mutation present late.
Features of athlete heart useful in differentiating from HCM
concentric hypertrophy
LV cavity > 55mm
VO2 > 110%
no late gadolinium enhancement
no LA enlargement
normal LV filling
improves w/ deconditioning
indication Myomectomy/alcohol septal ablation
Drug refractory progressive HF symptoms class III/IV gradient >/= 30mmHg at rest or with provocation
Indication for ICD in HCM pt
- Family Hx of SCD
- Septal diameter > 30mm
- NSVT
- Unexplained Syncope
- Attenuated or hypotensive BP response to exercise
CMR can be used in assessment of SCD risk in patients with HCM. what are we looking at?
> 15% of Late gadolinium enhancement is considered elevated risk of SCD.
Vast majority of HCM are managed with single chamber devices. which patients are Dual chamber devices reserved for______?
Those with SVT or forced AV pacing is indicated to treat symptoms related to LV outflow tract obstruction.
first line therapy for symptomatic HCM
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)
Risk of PPM with alcohol septal ablation (ASA)
10%
Risk of repeat procedure with ASA
5%
Genetic cardiomyopathies
- HCM
- ARVC/D
- LVNC
- Glycogen storage»_space; Danon / PRKAG2
- Conduction defects
- Mitochondrial myopathies
- Ion channel disorders»_space; LQTS / Brugada / SQTS / Asian SUNDS
Differentials for acquired primary cardiomyopathies
Inflammatory Stress provoked peripartum tachycardia induced Infants of insulin dependent diabetic mothers
Mechanism in which cocaine causes cardiomyopathy
multifactorial»_space;
- cathecolamine surge
- Hypertension»_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.
cancer drugs associated with Cardiomyopathy
Anthracyclines - Doxorubicin, Daunorubicin
Mechanism»_space; enhanced oxidative stress
Risk factors for cardiomyopathy 2/2 anthracyclines
- 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
X linked cardiomyopathies
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
what is the pathophysiology of permpartum cardiomyopathy
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
