Exam 3 CHD 4-5 Flashcards
Corrective procedure for Transposition of the Great Arteries (Vessels) (TGA)?
Arterial Switch
What are the 2 forms of TGA?
D-Transposition (Dextrotransposition)
L-Transposition (Levotransposition)
Which type of TGA is most common?
D-Transposition
Which type is where misdirected folding of embryonic heart tube- folding to the left side instead of right?
L-Transposition
Which type is where the Truncus Arteriosus fails to divide properly?
D-Transposition
What is the common origin of the aorta and pulmonary artery?
Truncus Arteriosus
Describe what happens with Dextrotransposition of the Great Arteries
The Truncus Arteriosus (common origin of the aorta and PA) fails to divide properly and creates 2 parallel circulations.
What 3 defects creates an exception to the parallel circulation that normally occurs with D-TGA?
PDA, ASD, VSD (Additional communications)
Trace the blood flow from the RA-Aorta with L-TGA
RA-MV-LV-PA-LA-TV-RV-Aorta
Which TGA is associated with switching the position of the RV and LV with NO AFFECT on the Great Vessels?
L-TGA
Describe what happens with Levotransposition of the Great Arteries
Misdirected folding of the embryonic heart tube occurs, it folds to the left side instead of the right. The RV and LV are switched and there is no affect on the great vessels.
TGA normally occurs with other anomalies, or in isolation?
Isolation
TGAs accounts for ____% of all CHDs.
6%
Sx of L-TGA at birth?
Asymptomatic at birth
Sx of R-TGA at birth w/o shunting lesions?
Profound Cyanosis
Sx of R-TGA at birth w/ shunting lesions?
Initially asymptomatic, progresses to tachypnea, tachycardia, heart failure, feeding problems, respiratory distress w/o cyanosis. LV volume overload and L to R shunting.
Auscultation, ECG and CXR associated with R-TGA
No murmur to Loud murmur (depends on shunt lesion)
RAD and RVH on ECG
Egg-shaped heart with narrow Stalk on CXR
Med/Surg Tx of D-TGA in neonate w/o sufficient shunting.
Prostaglandin Infusion- for patency of DA or stent placement
Balloon Septostomy- to create or increase ASD
O2
Tx of HF
Decrease PAP
Arterial Switch Operation- transecting PA and Aorta and reanastomosing to RV and LV
Anesthetic MGMT of TGA
Induction technique?
Inhalation or IV Induction
Ketamine 1-2mg/kg incrementally
Fentanyl 2-15mcg/kg incrementally
Rocuronium 1mg/kg
Anesthetic MGMT of TGA
Invasive lines?
Yes Arterial and CVP
Note- VSD closure needs bicaval cannulation, so use femoral venous line, not jugular
Anesthetic MGMT of TGA
Myocardial Ischemia concerns?
MI can occur after cross clamping is removed due to poor coronary anastomosis or air emboli in coronary artery- Increase CPP to flush out air. If no improvement, may need to go back on CPB to reassess anastomosis.
Anesthetic MGMT of TGA
____ to assess function of repair and presence of air
Echo
Anesthetic MGMT of TGA
Anticipate _______ HTN
Pulmonary HTN- can cause compression of Coronary Arteries and MI
Anesthetic MGMT of TGA
Inotropes?
More than likely
Use Dopamine, Epinephrine, and Milrinone
Anesthetic MGMT of TGA
LV will be _________, so be careful with ______
LV will be NONCOMPLIANT, be careful with FLUIDS- give slowly and in small amounts
Anesthetic MGMT of TGA
Coagulation is possible?
Yeap- often need antifibrinolytics
Anesthetic MGMT of TGA
Postoperatively, have high risk of:
dysrhythmias and conduction defects
Anesthetic MGMT of TGA
Maintenance- Keep PVR ____
Keep PVR DOWN
Deep GA will blunt reactive Pulm. HTN
Anesthetic MGMT of TGA
Avoid ____HR and ___ CO with limited myocardial reserve.
Avoid Decreased HR and Decreased CO
Control BP
Single vessel from the heart gives rise to both aorta and pulmonary artery.
What is Truncus Arteriosus?
Truncus Arteriosus is associated with ______Syndrome
DiGeorge
List the Clinical Features and Concerns of DiGeorge Syndrome (Table 15-4)
Absent of small thymus
T-Cell Abnormality w/ associated immunodeficiency
Hypoparathyroidism w/ associated hypocalcemia
Dysmorphic features, particularly a small mouth
Increased surgical morbidity and mortality
Irradiated blood products needed to prevent graft-vs-host disease
Mortality is high with Truncus Arteriosus, and surgery is performed _____
early in life
3 factors influencing mortality with TA.
Presence of:
Truncal Valve Stenosis
Coronary Abnormalities
Low Birth Weight
Describe the surgery for TA repair
Closure of VSD, disconnect pulmonary arteries, place graft between RV and PA to provide pulmonary blood flow.
Type 1 TA
Main PA and Aorta arises from BASE of truncus
Type 2 TA
R and L pulmonary arteries arise SEPARATELY from truncus, close to each other
Type 3 TA
Pulmonary arteries arise on OPPOSITE sides of truncus
Type 4 TA
PA branches are ABSENT, pulmonary blood flow from aortopulmonary collaterals.
All forms of TA have mixing of _____ and ____ blood, with significant ____ to _____ shunting and ______overcirculation.
All forms of TA have mixing of oxygenated and deoxygenated blood, with significate L to R shunting and PULMONARY overcirculation
TA leads to (4 things)
Cyanosis
Failure to Thrive
CHF
Pulmonary HTN
Induction concerns with TA
Intubation may be _____ due to DiGeorge facial anomalies.
Do not _____ or ________ if not intubated
Intubations may be DIFFICULT
Do not PREOXYGENATE or HYPERVENTILATE if not intubated.
Anesthetic MGMT of TA
IV induction agents:
Ketamine 1-2mg/kg
Fentanyl 2-4mcg/kg
Roc 1mg/kg
Anesthetic MGMT of TA
Avoid _____ventilation and maintain O2 sat _____%
Avoid Hyperventilation
O2- 75-85%
Anesthetic MGMT of TA
Keep DBP > ____ to perfuse coronary arteries
20mmHg
Anesthetic MGMT of TA
Maintenance drugs
Fentanyl 20-50mcg/kg
Roc
Midazolam 0.1-0.2 mg/kg
+/- Volatile agent
Anesthetic MGMT of TA
FiO2 -and avoid _________
Fio2 0.21
Avoid Hyperventilation
Anesthetic MGMT of TA
Will probably require postop ____
ventilation
Why do we avoid hyperventilation with TA?
Hyperventilation causes decreased PVR, leading to Increased Shunt and CHF
What art the two types of Anomalous Pulmonary Venous Connections
Total and Partial
Describe the difference between TAPVC and PAPVC.
TAPVC- All pulmonary veins insert into anomalous site
PAPVC- One or more pulmonary veins drain into either venous or right side of heart instead of the LA.
What are the 3 types of TAPVC
Supracardiac
Cardiac
Infracardiac
Describe Supracardiac TAPVC
Supracardiac- Pulmonary veins connect to SVC through an ASCENDING VERTICAL VEIN
Describe Cardiac TAPVC
Cardiac- Pulmonary veins connect to RA through CORONARY SINUS
Describe Infracardiac TAPVC
Infracardiac- Pulmonary veins connect to IVC through COMMON VEIN.
S/Sx of TAPVC
CHF, Cyanosis, Respiratory Distress, and Tachypnea
TAPVC
ECG
CXR
ECHO
TAPVC
ECG- RA and RV Enlargement
CXR- Cardiomegaly and Pulmonary Edema
ECHO- Will Identify Shunting Lesion, Cardiac Size and Ventricular Function
Tx for TAPVC
Surgical Correction
Closing Shunting Lesions with a Patch
Anesthesia MGMT of TAPVC
Induction Roc 1mg/kg, Fent 1-3mcg/kg IV
Volatile agents are rarely tolerated in obstructed TAPVC
Maintenance- Fent 20-50mcg in divided doses. Reduction of PVR will worsen pulmonary edema
Postop vent support. May need inotropes.
Anatomic Features (4) of Hypoplastic Left Heart Syndrome HLHS
Hypoplastic LV
Mitral Stenosis or Atresia
Aortic Stenosis or Atresia
Hypoplastic Aortic Arch
At birth, neonates with HLHS will present with (3)
Heart Failure
Shock
Cardiovascular Collapse
ECG and CXR findings with HLHS
ECG- RAD, RVH
CXR- Cardiomegaly, prominent pulmonary vascular markings.
Surgical Tx of HLHS
Convert to single-ventricle circulation where RV becomes single systemic ventricle and pulmonary blood flow moves passively from SVC and IVC (aka Fontan Circulation)
Anesthetic management of HLHS
Balance PVR with SVR Infuse Prostaglandin Use normal to high PaCO2 and Low FIO2 High dose Opioid technique Venous Access through Femoral Vein Hypothermia may be needed Coagulation is possible, antifibrinolytics are used (TXA)
Ventilation for HLHS
Maintain O2 Sat 75-85%
Avoid Hyperventilation- Increases pulmonary blood flow,
Sat >85% leads to hypoperfusion
Maintenance drugs for HLHS
Fent 20-40mcg/kg
Rock 1mg/kg
Volatiles on CPB
Midazolam 0.1-0.2mg/kg
HLHS Stage 2 correction occurs at _____ and Stage 3 correction at ______
6 months old
2-6 years old
Long term outcome of HLHS
RV will fail and Pt will need transplant
Describe Tricuspid Atresia (TA)
Absence or permanent closure of tricuspid valve
Describe the different types of TA
I - Most common, normal relationship of great vessels to ventricles
II - D transposition of the Great Vessels
III- L transposition of the Great Vessels
3 features of TA
RV is hypoplastic
ASD is present
Pulmonary Blood Flow is restricted d/t pulmonary stenosis or atresia
TA- Will have ____to ____ shunting, with various degrees of cyanosis
R to L shunting
Initial palliative procedure for TA
Blalock-Taussig Systemic to PA shunt
Definitive procedure for TA
Bidirectional Glenn Shunt or Modified Fontan
Sx of TA
50% of patients are symptomatic by _____ of life
24 hours
Sx of TA
If Pt has decreased pulmonary blood flow:
R-to-L shunting and Cyanosis
Tachypnea
Prominent A Waves
Failure to Thrive
Sx of TA
If Pt has increased pulmonary blood flow:
minimal cyanosis
but will still have
tachypnea, tachycardia, hepatomegaly, prominent A waves, feeding difficulties and CHF
TA
Auscultation
ECG
ECHO
TA
Auscultation- Holosystolic murmur of a VSD or continuous murmur of PDA
ECG- LAD, LVH, RA enlargment
ECHO- absent of closed tricuspid valve, enlarged chambers (except RV), RVOT obstruction, PAP, flow moving across VSD.
Development of Severe Pulmonary HTN due to L-to-R shunt, where the increased PVR eventually causes shunt reversal to R-to-L shunt
What is Eisenmenger Syndrome
Sx of Eisenmenger Syndrome
Increased Hypoxia with Decreased Exercise Tolerance
Enlarged RA and RV with Arrhythmias
Hyperviscosity due to Hypoxia
Eisenmenger Syndrome
ECG
CXR
ECG - RVH
CXR- Prominent pulmonary vessels
Anesthetic MGMT of Eisenmenger Syndrome
Similar to management of other forms of pulm. HTN
Avoid Insufflation- Worsens R-to-L shunt
PVR is fixed and does not respond to SVR changes
Keep SVR at preop levels
Overall CHD anesthetic implications
Is air bubbles good?
Nope
Overall CHD anesthetic implications
Qp:Qs > 1.5:1 limit _____ blood flow to prevent ___failure due to volume overload
pulmonary
RV
L to R Shunts
Little affect on onset of IV and inhalational agents as long as ________ is maintained
CO
L to R shunts
Pts with elevated pulmonary blood flow- Maintain or slightly increase ______
PAP
L to R shunts
Minimize agents that ___SVR or ____PVR
Increase SVR or Decrease PVR
R to L shunts
These favor R-to-L shunting
__PVR, ___RVOTO (infundibula spasm), ___SVR
Increased PVR
Increased RVOTO
Decreased SVR
all favor R to L shunting
With RVOTO, changes in _____ does not alter Qp:Qs ratio, while changes in _____ does.
PVR does not alter
SVR does
Anaphylactic/neurogenic shock Anemia Cirrhosis Vasodilators Anesthetic Agents Histamine Alpha Blockers Ganglionic Blockers All Do What?
Decrease SVR
Increase or Decrease PVR Syndromes of low CO Hypovolemia Cardiogenic Shock Hypothermia Vasoconstrictors All Do What?
Increase SVR
Increase or Decrease PVR 100% O2 Hypocarbia Normothermia Low mean AW pressures or SV
Decrease
Increase or Decrease PVR Hypothermia A1 agonists Sympathetic Stimulation Increased SVR
Decrease
Increase or Decrease PVR Acidosis Hypoxemia Hypervarbia High mean AW pressure Catecholamine release
Increase
Increase or Decrease PVR Avoidance of catecholamine release Low mean AW pressures or SV Hypothermia Increased SVR Hypocarbia
Decrease
Meds like neo, ketamine, N2O Hypothermia B2 agonist All A1 agonists Deep GA NA Decreased SVR
Increase
and yes, this table lists hypothermia and A1 agonist as both increasing and decreasing PVR. What a fuck!
Who can go fuck right off?
You guessed it!!!
