Echo 530

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  1. The MC cyanotic CHD is ___________________?
    Tetralogy of Fallot
  2. 4 defects that make up TOF?
    • 1. Lg overiding AO
    • 2. Perimembranous VSD
    • 3. PS or Pulmonary Atresia
    • 4. RVH
  3. 4 symptoms of TOF?

    • 1. Cyanosis
    • 2. Dyspnea
    • 3. Syncope
    • 4. Arrhythmias
  4. What is the deciding factor that determines the severity of TOF?
    • Degree of PS
    • Degree of RVOT obstruction
  5. What is Ebsteins?
    Congenital disorder: faulty dev't of RVIT; short septal leaflet
  6. 2D appearance of Ebsteins
    • Sail-like leaflet
    • RAE
    • RV small
    • LV squished
    • RVVO (paradoxical septal motion)
    • TV displacement >20mm
  7. What is the deciding factor that determines the severity of Ebsteins?
    If you see TV in PSLX @ same time you see MV.....bad
  8. What is Eisenmenger's Syndrome?
    Reversal of congenital shunt Rt--> Lt ; secondary to severe PHTN
  9. List the 3 types of pulmonary stenosis:
    • 1. Infundibular (RVOT)
    • 2. Valvular (MC)
    • 3. Supravalvular

    More common in females
  10. List the 4 types of Truncus Arteriosus
    • 1. Single PA arise from truncus
    • 2. LPA & RPA arise close together
    • 3. LPA & RPA arise independently (opposite side)
    • 4. Absent PA
  11. TOF is a ___________________ __________________ defect.
    bulbus cordis
  12. "Blue Baby" implies:
    deoxygenated blood to system d/t shunt and increased Rt sided pressures.
  13. RAP = ?
  14. On doppler, increased (with stenosis) pulmonary artery pressure = ________________ a dip
  15. What would you see in an adult with a TOF repair?
    • VSD patch (bright)
    • AO still enlarged (AI?)
    • RVOT some problems still?
    • PI
  16. With Ebstein's, what associated anomaly do you see 75% of the time?
    (like to see....keeps the baby alive)
  17. What is Wolf Parkinson White Syndrome?
    extra electrical connection between the atria and the ventricles is present at birth
  18. If in PSLX you see RVE, thick TV what would you think is wrong with this heart?
  19. Ebsteins Qp/Qs is about?
    What's a normal Qp/Qs?
    • 1 : 1.7
    • 1 : 1
  20. Can Eisenmenger's be repaired?
    What happens if medications do not work?
    • No
    • Transplant (heart and lung)
  21. Why is there a decreased incidence of Pulmonary Stenosis than in the past?
    decreased incidence of maternal rubella
  22. What is the MC form of pulmonary stenosis?
  23. Hemodynamic status of truncus hinges on whether the PA configuration allows adequate ________________ ______________ _______________.
    Pulmonary blood flow
  24. How do you differentiate TOF vs. truncus?
    • 1. placement of PA
    • 2. ID origin of PA off truncus then you know not TOF
  25. What does TAPVR stand for?
    Total anomalous pulmonary venous return
  26. Define: TAPVR
    • All pulmonary veins going into Rt side of heart
    • (need a shunt to live)
    • (MC into SVC)
  27. What are the 2 types of TAPVR?
    • 1. Obstructive
    • 2. Nonobstructive
  28. What is obstructive venous return?
    • think "abdomen"
    • veins go through abdomen via hepatics to diaphragm and to heart
  29. What is non-obstructive venous return?
    • think "cardiac"
    • SVC to behind heart
    • coronary sinus
    • or directly to RA
  30. Define: Partial Anomalous Pulmonary Venous Return
    Associated with sinus venosus ASD

    Any # pulmonary veins in any combination may drain into any of several venous structures
  31. Define: Cor Triatriatum
    MMB results from fusion of the common pulmonary veins and embryonic LA does not regress completely (upper and lower)

    • 3 pts:
    • mmb
    • PV drains to up
    • MV stenotic appearance
  32. Define: Double Outlet RV
    Both great arteries part/totally arise from RV
  33. Define: Tricuspid Atresia
    • No TV
    • RVIT failed to form
    • Dimple or mmb on floor of RA
  34. Define: Pulmonary Atresia
    • No PV
    • Cyanotic Heart Disease
    • Prox - Dist = RVOT can't be connected
  35. Define: MV Atresia
    • No MV
    • Very rare & potentially a lethal heart
  36. Define: Aortopulmonary window
    a shunt

    Connection btw AO & PA usually circular in shape and midway between PV and bifurcation of main pulmonary artery
  37. Because of the obstruction of mmb in cor triatriatum, the flow on doppler is _______________ with ________________ velocity which is detected proximal to MV
    • turbulent
    • high
  38. What is imperative for survival when one has a double outlet right ventricle?
  39. If you have tricuspid atresia, you do not want an ASD or VSD? T / F?
    • F
    • need for baby's survival
  40. What is the difference between PDA & aortico -pulmonary window?
  41. 2 common cardiac mechanisms causing cyanosis?
    • 1. Obstruction of pulmonary blood flow with proximal Rt -->Lt shunting
    • 2. Large volume venous mixtures
  42. What is associated with obstruction of pulmonary blood flow with prox rt to lt shunting? (4)
    • TOF
    • Eisenmengers
    • Ebsteins
    • Tricuspid Atresia
  43. What is associated to Cyanosis d/t large VOLUME of venous mixtures? (3)
    • 1. Persistant truncus arteriosus
    • 2. TAPVR
    • 3. Transposition of Great Vessels (AO arises off RV thus receives desat syst venous blood)
Card Set
Echo 530
Cyanotic Congenital Heart Disease
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