Cardiac Miscellaneous Flashcards
(14 cards)
What should be done first in the setting of acute hemodynamic compromise at presentation?
What etiologies should be considered before acute systolic dysfunction from blunt cardiac trauma?
- FAST evaluation
- Common etiologies:
- hemorrhagic shock
- tension PTX
- cardiac tamponade
What is the sensitivity of genetic testing in familial dilated cardiomyopathy (FDC)?
If performed, who should it be performed in?
-
30-40% sensitivity
- heterogeneous genetic disease
- variable presentations
- reduced penetrance
- different modes of inheritance
- Most clearly affected person in the family
- mutations of 33 known genes –> account for only 40-50% of cases
Describe Familial Dilated Cardiomyopathy
- clinical diagnosis
- idiopathic dilated cardiomyopaty occurs in ► 2 closely related family members
- 80-90% autosomal dominant and rarely –>
- autosomoal recessive
- X-linked
What is the next step in management?
- 22 year old with syncope
- PMH and SH: none
- Tox screen: negative
- VS: stable
- EKG: NSR and QTc 492ms
- Echo: normal
- BB’s (first line)
-
Genetic screening
- important to identify a LQTS mutation to allow for risk stratification and therapeutic decisions and
- evaluate family members to identify silent carriers
- Lifestyle changes should also be implemented
What is the sensitivity of genetic testing in LQTS?
75% sensitivity
- typically autosomal dominant + variable penetrance
- subdivided into 15 types based on causative gene
- can help with:
- diagnosis
- screening at-risk family members
What is the genetic or clinical screening recommendation for HCM?
all first-degree family members of patients with HCM in those with unrecognized disease
What determines the interval for clinical screening for HCM in patient’s with a 1st degree family member with HCM?
Genotype of the patient
What is the sensitivity of genetic testing for Marfan’s syndrome?
95% sensitivity
- Autosomal dominant inheritance pattern
- mutations in the FBN1 gene
- not necessary for confirming diagnosis in index case
- can be helpful for other family members who need to be screened
What is the recommendation for genetic testing?
- HCM diagnosed clinically
- genetic testing of index patient is negative
no identifiable pathogenic mutation in index patient –>
no indication for futher genetic testing of other family members –>
repeat Echo testing in 5 years
What is a dreaded side effect of Thalidomide or Lenalidomide for treatment of multiple myeloma?
VTE - increased risk
What is a major physiologic change that occurs in pregnancy?
50% increase in CO
What hemodynamic change will occur in the 3rd trimester?
- 29 yer old with h/o tricuspid atresia and looking to become pregnant
- PMH: Fontan procedure years ago
LVEF will decrease
- pregnant women with structural heart disease may have a reduced cardiac reserve
What cardiac hemodynamic changes are seen in pregnancy?
- Increased:
- Cardiaco Output
- Stroke Volume
- LV mass
- E/E’ ratio
- Decreased:
- LVEF
- fractional shortening
What is the recommended monitorig in patients receiving androgen-deprivation therapy?
every 3-6 months
- first effects of androgen-depreciation therapy typically occur in this time frame
- BP, lipids and fasting glucose should be assessed
