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The MC cyanotic CHD is ___________________?
Tetralogy of Fallot
4 defects that make up TOF?
- 1. Lg overiding AO
- 2. Perimembranous VSD
- 3. PS or Pulmonary Atresia
- 4. RVH
4 symptoms of TOF?
- 1. Cyanosis
- 2. Dyspnea
- 3. Syncope
- 4. Arrhythmias
What is the deciding factor that determines the severity of TOF?
- Degree of PS
- Degree of RVOT obstruction
What is Ebsteins?
Congenital disorder: faulty dev't of RVIT; short septal leaflet
2D appearance of Ebsteins
- Sail-like leaflet
- RV small
- LV squished
- RVVO (paradoxical septal motion)
- TV displacement >20mm
What is the deciding factor that determines the severity of Ebsteins?
If you see TV in PSLX @ same time you see MV.....bad
What is Eisenmenger's Syndrome?
Reversal of congenital shunt Rt--> Lt ; secondary to severe PHTN
List the 3 types of pulmonary stenosis:
- 1. Infundibular (RVOT)
- 2. Valvular (MC)
- 3. Supravalvular
More common in females
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
TOF is a ___________________ __________________ defect.
"Blue Baby" implies:
deoxygenated blood to system d/t shunt and increased Rt sided pressures.
On doppler, increased (with stenosis) pulmonary artery pressure = ________________ a dip
What would you see in an adult with a TOF repair?
- VSD patch (bright)
- AO still enlarged (AI?)
- RVOT some problems still?
With Ebstein's, what associated anomaly do you see 75% of the time?
(like to see....keeps the baby alive)
What is Wolf Parkinson White Syndrome?
extra electrical connection between the atria and the ventricles is present at birth
If in PSLX you see RVE, thick TV what would you think is wrong with this heart?
Ebsteins Qp/Qs is about?
What's a normal Qp/Qs?
Can Eisenmenger's be repaired?
What happens if medications do not work?
- Transplant (heart and lung)
Why is there a decreased incidence of Pulmonary Stenosis than in the past?
decreased incidence of maternal rubella
What is the MC form of pulmonary stenosis?
Hemodynamic status of truncus hinges on whether the PA configuration allows adequate ________________ ______________ _______________.
Pulmonary blood flow
How do you differentiate TOF vs. truncus?
- 1. placement of PA
- 2. ID origin of PA off truncus then you know not TOF
What does TAPVR stand for?
Total anomalous pulmonary venous return
- All pulmonary veins going into Rt side of heart
- (need a shunt to live)
- (MC into SVC)
What are the 2 types of TAPVR?
- 1. Obstructive
- 2. Nonobstructive
What is obstructive venous return?
- think "abdomen"
- veins go through abdomen via hepatics to diaphragm and to heart
What is non-obstructive venous return?
- think "cardiac"
- SVC to behind heart
- coronary sinus
- or directly to RA
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
Define: Cor Triatriatum
MMB results from fusion of the common pulmonary veins and embryonic LA does not regress completely (upper and lower)
- 3 pts:
- PV drains to up
- MV stenotic appearance
Define: Double Outlet RV
Both great arteries part/totally arise from RV
Define: Tricuspid Atresia
- No TV
- RVIT failed to form
- Dimple or mmb on floor of RA
Define: Pulmonary Atresia
- No PV
- Cyanotic Heart Disease
- Prox - Dist = RVOT can't be connected
Define: MV Atresia
- No MV
- Very rare & potentially a lethal heart
Define: Aortopulmonary window
Connection btw AO & PA usually circular in shape and midway between PV and bifurcation of main pulmonary artery
Because of the obstruction of mmb in cor triatriatum, the flow on doppler is _______________ with ________________ velocity which is detected proximal to MV
What is imperative for survival when one has a double outlet right ventricle?
If you have tricuspid atresia, you do not want an ASD or VSD? T / F?
- need for baby's survival
What is the difference between PDA & aortico -pulmonary window?
2 common cardiac mechanisms causing cyanosis?
- 1. Obstruction of pulmonary blood flow with proximal Rt -->Lt shunting
- 2. Large volume venous mixtures
What is associated with obstruction of pulmonary blood flow with prox rt to lt shunting? (4)
- Tricuspid Atresia
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)