Conotruncal Defects/ Single Ventricle Defects Flashcards
TOF, Trucus arteriosus, DORV, Double inlet LV, TOF, Trucus arteriosus
What is DORV
Where both of the great vessels arise completely or mostly from the RV
Clinical signs of DORV
-Cyanotic
-CHF
-failure to thrive
Severity of DORV is determined by the
location and size of the VSD and relation to great arteries and any obstructions.
DORV is classified into subtypes based off the location of the VSD.
What are those subtypes?
- Subaortic VSD: VSD is below AV, LV flow crosses VSD and mostly goes out AO
- Subpulmonary VSD: VSD sits below PV (D-TGA) most of the LV flow goes out the PA
- Doubly committed VSD: Common to both AO and PA, LV blood maybe committed to either
- non-committed/ remote VSD: VSD is situated away from both PV & AV
DORV may also be classified based off the presence of PS
Most Common:
1. subaortic with PS
2. subaortic w/o PS
3. Subpulmonic without PS
What distinguishes DORV from TOF?
Lack of mitral and aortic fibrous continuity.
where the anterior leaflet of the mitral valve does not properly insert into the aortic root
DORV often resembles
*Tetralogy of Fallot (PS)
*Transposition of the great arteries
Great arteries maybe;
normally related (rarely)
transposed (D or L type)
side by side (typical presentation, AO to the right of PA)
Things to look for and rule out on echo with DORV patients
Large VSD and location
MV and AV abnormalities are common (fibrous continuity)
LV could be hypoplastic
RV maybe hypertrophic
Coronary arteries orientation can be affected
carefully differentiate DORV from TET (PS) and TGA
determine degree AV override
What congenital anomalies and shunts are commonly associated with DORV
Large VSD
CoA
Pulmonary atresia
interrupted aortic arch
TAPVR
Heterotaxy syndromes (asplenia/polysplenia)
DORV surgical repair goal is to
Predominantly close the VSD and attach the AO to the LV.
this can vary drastically depending on type of DORV and PS.
DORV with subaortic VSD repair involves
VSD repair
A tunnel in the RV is formed to incorporate the AO as part of LV
Easiest to correct
DORV with subpulmonary VSD without PS repair involves
Typically, great arteries are side-by-side with the AO to the right of the PA.
when the **VSD is closed **the arteries are transposed (AO coming off the RV and PA coming off the LV)
Then an **arterial switch **aka “Jatene Procedure” corrects the transposition
Jatene Procedure aka arterial switch
An operation used in complete TGA that involves removal of the aorta and PA from their roots and then repositioning them over the other ones root. Allowing AO to come off the LV and PA to come off the RV.
(so the old AV is now the neo-pulmonary and the old PV is the neo-aorta)
lastly the coronaries are reattached
DORV with subaortic VSD and PS repair involves
the interventricular patch repairs the VSD and connects the LV to the AO
stenosis is corrected then by patch augmentation (widening) of RVOT or PV homograft or Rastelli procedure may be used to bypass PV via a conduit in order to establish connection between the RV to the PA bifurcation
Complex DORV patients require what type of repair?
Think Hypoplastic LV
Single Ventricle repair
-Bidirectional Glenn
-Fontan at age 3-4years old
Classic findings of Tetralogy of Fallot
- Large perimembranous malalignment VSD
- RVOT obstruction (PS)
- Overriding AO, dilated root
- Right ventricular hypertrophy
These findings are a result of the RVOT infundibulum being malaligned due to the IVS failure to attach to the annulus during embryology
What can cause a TET baby to be acyanotic verse a cyanotic baby?
the degree of PS and direction of VSD shunt
want to keep PDA open via prostaglandin
look for subvalvular stenosis and hypoplastic pulmonary arteries
What other congenital cardiac defects is TET often seen with?
- Right AO arch
- Anomalous coronary arteries (look for LAD???)
- PAPVR
- Persistent LSVC
- Pulmonary atresia with VSD
On chest X-ray Tetralogy of Fallot appears as
a Boot-shaped heart
Tetraology of Fallot EKG shows
right axis deviation and RVH
What are “Tet spells”
sudden drop of O2 saturation due to increase RV outflow obstruction. Associated with TET.
may be relieved by squatting or bending the knees forward
What is the most common cyanotic congenital heart defect?
Tetralogy of Fallot
Tetralogy of Fallot repair consist of?
At what age?
In severe cases a palliative procedure may be required to increase flow prior to single stage procedure.
- Blalock-Taussing shunt
Usually about 6 months the complete repair involves:
* VSD closure with synthetic patch or autograft
* ASD closed if present
* PDA closed if remained opened
* RVOT reconstruction to relieve PS, pulmonary valvotomy, RV-PA conduit
What is the difference between a classic BT-shunt, modified BT-shunt, Waterston, and Potts shunt?
-What type of shunt is shown below?
-What type of incision on the chest?
Classic BT-shunt: right subclavian artery to RPA (end-to-side anastomosis), rarely performed
Modified BT-shunt: Gore-Tex conduit between Rt subclavian artery and RPA (side-to-side)
Waterston shunt: AAO and RPA (direct side to side anastomosis)
**Potts shunt: ** DAO and LPA (direct side to side anastomosis)
Central shunt: PTFE tube graft from the ascending aorta to the main PA
Purpose is to increase Pulmonary blood flow in cyanotic CHDs
performed through median sternotomy or lateral thoracotomy
What cyanotic congenital defects typically require a palliative shunt such as, a BT-Shunt?
- TET
- HLHS
- Tricuspid Atresia
- Pulmonary Atresia
Classic findings of truncus arteriosus?
Common arterial trunk overriding a large VSD
-3 types
Frequently secundum ASD present
Usually NO PDA!!! PDA absent 50% of the time
PA branches may be hypoplastic
LAE/LVE due to volume overload»»CHF
Cyanosis is common
aortic and pulmonic valves are fused into a large semilunar valve with multiple leaflets called truncal valve
What are the 4 types of truncus arteriosus?
Type I 60% : Main PA comes off the AAO “common arterial trunk”
Type II 30% : No main PA, Branches arise off posterior portion of common arterial trunk, right arch is common
Type III 10% : No main PA, branches come off the sides of the common arterial trunk
Type IV: Neither PA arise off the common trunk, usually considered Pulmonary atresia with VSD or TET
With truncus arteriosus majority of the mixing of blood occurs at what level?
ventricles across the VSD
Truncus arteriosus valve can have as many as ___ leaflets?
as many as 5 or (rarely) 6 leaflets
Most commonly has 3 cusps
What other congenital cardiac defects is truncus arteriosus often seen with?
Right AO arch
interrupted AO arch
CoA
What syndrome is common with Truncus Arteriosus?
Di-George Syndrome/ Chromosome 22q11
truncal arteriosus, IAA, VSD, Cleft Pallet
Truncus Arteriosus affects Males or Females more?
Males
Truncus arteriosus surgical repair consist of?
- VSD closure
- separation of branch pulmonary arteries from common trunk
- RV to PA conduit “Rastelli Procedure”
-additionally aortic arch may need repaired if there is an IAA
trunk and truncal valve become the neo-aorta
What does a double inlet left ventricle consist of?
both AV valves are related to one large single ventricle.
- small non-dominate ventricle may be present
- AV valves may be dysplastic or atretic
think Large VSD to no IVS only one chamber present
What other congenital cardiac defects is DILV often seen with?
double inlet left ventricle
- Transposition of the great vessels
- Pulmonary stenosis
- Coarctation of the aorta
Surgical treatment for DILV consist of?
Double inlet left ventricle
palliative shunt if PS present
Norwood procedure
Fontan procedure
What is the most common type of VSD in DORV patients
Sub aortic VSD
What cardiac defect is associated with leftward juxtaposition of the right atrial appendage
Double– Outlet right ventricle
With a double outlet right ventricle, what type of VSD and other associations occur with taussig-bing
Sub-pulmonary VSD, side-by-side great arteries, unobstructed pulmonary outflow tract, and possible sub-aortic obstruction
A DORV patient can have an interrupted aortic arch when the conal septum is deviated towards
When the conal septum is deviated towards the subaortic outflow tract
Which type of VSD are you most likely to encounter a straddling mitral valve in a DORV patient
DORV with sub-pulmonary VSD
What is a straddling mitral valve?
SMV is a abnormal mitral valve Cordae attaching to both the right and left ventricle
In theology of low, the VSD malalignment is positioned anterior or posterior
Anterior, causing PS
In TOF the pulmonic valve is most commonly seen to have how many leaflets?
Bicuspid pulmonic valve
Leftward juxtaposition of the right atrial appendage can be seen in what congenital anomalies?
DORV
DTGA
TV atresia
HRHS
