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Congenital Heart Defects
Advanced Principles 3 Final Exam Congenital Heart Defects from Santina Marras
113
Medical
Graduate
11/12/2014

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Term
When is the fetal heart developed?
Definition
Between the 6th & 8th week
Term
How is the fetus affected if heart development is altered?
Definition
They are protected since their lungs are not the source of gas exchange.
Term
How does the fetus receive O2 and nutrients?
Definition
From the umbilical vein which derives from the placenta.
Term
What is the first major organ of the fetus perfused?
Definition
The liver
Term
Describe fetal blood flow.
Definition
-Half of the blood bypasses the liver via Ductus Venosus which connects the IVC to SVC.
-IVC (oxygenated blood) is mixed with SVC deoxygenated blood)
-From Vena cava to Right Atrium
-From Right Atrium through foramen ovale to L Atrium
-From L Atrium to L Ventricle and Aorta
-2/3 of blood from Aorta to coronaries and carotids
-1/3 of blood from Aorta into descending aorta
-Blood from the pulmonary artery joins the descending aortic blood via the Ductus Arteriosus
-From descending aorta to umbilical artery
Term
What keeps the Ductus Arteriosus patent?
Definition
-High Pulmonary Vascular Resistance.
-Because the fetal lungs are collapsed, PVR is high which causes blood from the pulm artery to bypass the lungs and push through the PDA into the aorta.
Term
Ductus Venosus
Definition
-Connects IVC to SVC
-Functional @ birth
-Anatomical Closure @ 5-7days
Term
Foramen Ovale
Definition
-Connects RA to LA
-Functional @ birth
-Anatomical Closure @ 3 mos - 1 year
Term
Ductus Arteriosus
Definition
-Connects PA to Descending Aorta
-Functional @ 10-15 hours post birth
-Anatomical Closure @ 4-6 weeks
Term
Circulatory Changes from fetus to newborn
Definition
After birth:
-Dramatic fall in PVR
-Increase in pulmonary BF with inflation & oxygenation of lungs )occurs @ first breath)
These changes lead to:
-Closure of Ductus Venosus
-Constriction of PDA
-Reversal of pressure gradients in RA & LA = closure of FO
Term
What is transitional circulation?
Definition
A temporary state after birth when PVR drops but high PAP and resistance (less than utero) exist.

A PDA allows for L -> R shunting and patient survival.

A labile state where failure to maintain low PVR can lead to R-> L shunting and a reversion back to fetal circulation
Term
How is transitional circulation maintained?
Definition
By trying to maintain some fetal circulatory pathways by:
-Maintaining a PDA w/ PGE1
-Allow normal heart circulation to occur slowly over a few weeks.
-Keep PVR low?
Term
What factors aid transition from fetal to mature circulation?
Definition
-Normal O2 tension
-Physical expansion of the lungs
-Normal pH
-Nitric Oxide
-Prostacycline (PGI2) - a lipid molecule known as eicosanoils: inhibits platelet activation and is a pulm vasodilator
Term
What factor favor reversing back to fetal circulation?
Definition
-Low O2 tension
-Acidotic pH
-Lung Collapse
-Inflammatory mediators: leukotrienes, thromboxane A2, platelet activating factor)
-Sepsis
Term
Preload
Definition
The force that distends the ventricular muscle to its preconstriction length
Term
Afterload
Definition
The impedance that is faced by contracting heart.
Term
Contractility
Definition
Cross-bridge formation within the myocyte
Term
Differences between myocardial function in infants vs adults
Definition
THE NEONATE:
-Is less compliant to volume shifts
-Less tolerant to increased afterload
-Less responsive to increased preload
-CO is greatly affected by HR
-Resting tension is greater
-Operates at the top of the Frank-Starling curve
Term
How is cardiac output maintained in a newborn?
Definition
They have a fixed stroke volume, so it is maintained with a faster HR
Term
Is a newborn's lung compliance lower or higher than an adults?
Definition
They have lower lung compliance.
This leads to higher RR and lower BP
Term
Is a newborn's chest compliance lower or higher than an adults?
Definition
They have a higher chest wall compliance leading to a lower FRC
Term
How does an infants body surface area compare to their weight?
Definition
They have a higher ratio of body surface area to body weight.
Term
What is different about an infants total body water content?
Definition
It is higher
Term
Infant heart pressure (in mmHg)
Definition
RA 5
LA 10
RV 25
LV 100
PA 25/10
Ao 100/50
Term
What factors increase Pulmonary Vascular Resistance (PVR)?
Definition
-Hypoxia
-Hypercarbia
-Acidosis
-Hyperinflation (high PEEP or PIP)
-Sympathetic stimulation
-High Hct
-Atelectasis (low FRC)
-Surgical Retraction
-Anesthetics (+/- Ketamine, N20)
Term
What Factors decrease Pulmonary Vascular Resistance (PVR)?
Definition
-Oxygen (potent pulm vasodilator)
-Hypocarbia
-Normal FRC
-Low Hct
-Nitric Oxide
-Blocking sympathetic response (ie narcotics)
-Viagra (caution, may cause a 6 hour long....)
Term
How does CHF differ in adults and children?
Definition
Adults:
-Heart ischemia leads to LV dysfunction
-LV dysfunction leads to low EF
-Low EF leads to increased LAP

Child:
-Large ventricular septal defect
-Increased blood flow to the lungs and R heart
-Increased pressure on R&L side = LV failure
Term
What are the S/S of CHF in newborns?
Definition
-Low CO
-Increased respiratory work
-Increased metabolic work
Term
How does low CO present in the CHF newborn?
Definition
-Tachycardia
-Poor extremity perfusion
-Gallop rhythm
-Cardiomegaly
-Hepatomegaly
Term
How does increased respiratory work present in the CHF newborn?
Definition
-Tachypnea
-Grunting
-Nasal Flaring
-Chest wall retraction
Term
How does increased metabolic work present in the CHF newborn?
Definition
-Failure to thrive
-Poor weight gain
(all their energy is spent on breathing instead of growing)
Term
How is CHF treated in newborns?
Definition
-Low dose inotropes (milrinone)
-Avoid tachycardia to optimize filling time
-Vasodilators to increase flow
-Diuretics
Term
What are the categories of CHF in newborns?
Definition
-Obstructive lesions

-Regurgitant lesions

-Shunt Lesions
Term
Obstructive Lesions
Definition
Aortic Stenosis
Mitral Stenosis
-similar to adults
Term
Regurgitant Lesions
Definition
Mitral Regurg
Aortic Regurg
-Similar to adults
-Blood volume overload leads to ventricular dilation and failure
Term
Shunt Lesions
Definition
Atrial Septal Defect
Ventricular Septal Defect
Patent Ductus Arteriosus
-Direct communication and mixing of systemic and pulm blood
Term
List the Acyanotic Heart Lesions
Definition
Atrial Septal Defect
Ventricular Septal Defect
Patent Ductus Arteriosus

Dont need to know:
AV septal defect
Pulm valve stenosis
Aortic valve stenosis
Coartation of aorta
Term
List the Cyanotic Heart Lesions
Definition
Tetralogy of Fallot (TOF)
Tranposition of great vessels (TGA)
Hypoplastic Left Heart Syndrome (HLHS)

Dont need to know:
Pulm atresia
Total anomalous pulm venous return
Truncus arteriosus
Tricuspid vavle abnormalities
Term
Describe Acyanotic Shunt Lesions
Definition
-When fetal openings stay open past fetal stage
-PDA, ASD, VSD are the most common
-Blood shunts from L -> R
-Decreased PBF

Amount of shunting depends on:
1. Size of the lesion
2. Relative resistances of the systemic and pulm circulations
3. The ability to manipulate PVR to control amount and direction of shunt
Term
Patent Ductus Arteriosus (PDA) definition
Definition
A connection between the main PA and Aorta
Term
PDA clinical findings
Definition
-Continuous murmur
-Widened pulse pressure
-Increased pulm blood flow
(Diastole will increase once PDA is fixed from aortic runoff)
-Resp failure
-Cong heart failure
Term
Why do does the PDA stay open?
Definition
-Premies are unable to metabolize PGE1 (immature livers)
-Premies have decreased arterial O2 tension
(PDA closes when exposed to increased O2 tension)
-Shunts as much as 80% of CO to lungs
(can lead to Necrotizing EnteroColitis (NEC) & Resp failure)
Term
Medical Management of PDA*
Definition
-Indomethacin: an NSAID that inhibits COX to reduce prostaglandin sythesis
(Contraindicated for IVH & Renal failure)
-Fluid restriction
-Diuretics
Term
Surgical Management of PDA*
Definition
(If Indomethacin contraindicated)
-Left thoracotomy
-Device closure in older children and adults
Term
How/Why would you maintain an open PDA?*
Definition
Why
-Sometimes a PDA is needed to sustain life
(HLHS, PA, TGA)
-The PDA mixes blood

How
-Prostaglandin infusion @ 0.05mCg/kg/min
Term
Atrial Septal Defect (ASD)
Definition
-A communication between the L & R atria
-Account for 12% of CHD in peds
-Closed via median sternotomy on bypass or in cath lab if hole is small enough

Three Types (said we didn't need to know):
-Secundum
-Venosus
-Primum
Term
Ventricular Septal Defect
Definition
-An abnormal communication between the L & R ventricle
-Most common CHD (20% of all CHDs)
-Closed w/ Sternotomy on bypass

Four types (said we don't need to know):
-Supracristal
-Infracristal
-Canal Type
-Muscular
Term
Recap of L -> R (Acyanotic) shunts
Definition
-Develop a hyperdynamic left ventricle w/o benefit of increased systmic circulation
-Develop increased lung water which leads to L&R heart failure, Meisenger syndrome
-Increased LAP from LV continuously recirculated to the lungs
-Dyspnea and pulm edema (inc. RR & HR)
-Wheezing, diaphoresis, cardiomegaly, failure to thrive
Treatment:
1. Low dose inotropes (milrinone)
2. Avoid tachycardia
3. Vasodilators to inc. flow
4. Diuretics
Term
Anesthetic Management of L -> R (Acyanotic) shunts
Definition
-Avoid myocardial depressants (ie Propofol)
-High narcs preferred
-Avoid all air bubbles (Bi-directional shunting can occur)
-Maintain PVR to avoid inc. L -> R shunting
-Avoid hyperventilatio (slow and easy, keep CO2 normal to high)
-Avoid 100% O2 (Induce w/ room air)
-Isoflurane is VA of choice
Term
What is faster for patients with L -> R (Acyanotic) shunts, inhalation induction of IV induction?*
Definition
In theory, inhalation should be faster because of incr. blood flow to the lungs.
Term
Describe Cyanotic Lesions
Definition
-Typically R -> L shunting
-Decreased PBF (thus, cyanosis)
-Increased systemic blood flow
Term
Anesthetic Management of Cyanotic Lesions
Definition
-Manipulate systemic perfusion: pulm perfusion by keeping FiO2 low
-Maintain CO and myocardial function
-Avoid myocardial depressants
-Maintain PGE1 until repair
-IV induction is faster
Term
Tetralogy of Fallot
Definition
-R -> L shunt lesion
-Most common cyanotic heart disease

4 Anatomical Abnormalities*:
1. Large unrestricted VSD (flow goes back & forth)
2. Right Ventricular Outflow Tract (RVOT) obstruction
3. Overriding of Aorta above RVOT (aorta is open to both ventricles)
4. RV hypertrophy
Term
Variants of TOF
Definition
-TOF w/ pulm atresia and VSD (total RVOT obstruction)
-TOF w/ pulm stenosis (classic)
-TOF w/ absent pulm valve syndrome (most severe)
Term
What are some common coexisting cardiac diseases in patients w/ TOF?
Definition
-Left SVC
-AV Septal defect
-PDA
-ASD
-Interrupted IVC
Term
What are some associated Extracardiac Malformations in patients with TOF?
Definition
-VACTERL
-Di George Syndrome
-Charge Syndrome
-Chromosome 22q 11 deletion
Term
What is VACTERL?
Definition
Abnormalities of:
-vertebral
-vascular
-cardiac
-tracheoesophageal
-renal
-limb abnormalities
Term
What is Charge Syndrome?
Definition
-Coloboma (a hole in one of the structures of the eye)
-Heart anomaly
-choanal Atresia
-Retardation
-Genital anomalies
-Ear anomalies
Term
Describe a Blue TET
Definition
-Extreme cyanosis
-Right ventricular outflow tract obstruction (RVOTO)
-Profound R->L shunting through VSD
-Pulm stenosis
-Infundibular or Suprafundibular stenosis
Term
Describe a Pink TET
Definition
-No cyanosis
-Minimal RVOTO
-Net L->R shunt
Term
Describe the pathology of TOF?
Definition
-The severity of the symptoms are determined by the severity of the RVOTO
-Non-restrictive VSD effectively equalizes pressure in both ventricles
-A restrictive VSD may cause suprasystemic RV pressures
-RV hypertrophy D/T high after load and diastolic dysfunction
Term
What is a TET spell?
Definition
-Infundibular spasm
-Acute drop in PBF
-Shunting of desaturated blood into systemic circ
Term
What causes a TET spell?
Definition
Spontaneously from:
-crying
-defecation
-agitation
-injury
-fright
Precipitated by:
-Acute fall in SVR (maybe from anesthesia?)
Term
What can worsen the cyanosis during a TET spell?
Definition
-Anemia
-Acidosis
-Infection
-Stress
-Posture
Term
What is the treatment for a TET spell?
Definition
-100% O2
-B blockers (reduce infundibular spasms)
-Sedation/deepening anesthesia
-Morphine (to dilate PA)
-Fluids (to increase preload)
-Phenylephrine 0.1mCg/kg (to increase SVR:PVR ratio)
-Abdominal compression (to increase SVR)
-Squatting legs
-Correct acidosis
-Surgical repair
Term
What do we want to avoid during anesthesia of TOF patients?
Definition
-Tachycardia
-Increased contractility
-Dehydration
(These 3 increase RVOTO and decrease filling time)
-Hypovolemia
-Hypotension
(These 2 decrease preload and SVR)
Term
What is Transposition of the Great Arteries (TGA)?
Definition
-Failure of the great arteries to rotate
-Aorta and coronies arise from RV
-PA arises from LV
-Pulm and Sys circ are separate and parallel
-Venous blood is re-circulated to the RV
-Aorta is not oxygenated
Survival: from mixing of blood via VSD, ASD, or PDA
Term
What are some treatments for Transposition of the Great Arteries (TGA)?
Definition
Balloon Atrial Septostomy:
-Creates a larger communication between atria
-Increases saturations to 80-90%
Arterial switch:
-Reimplantation of coronary arteries
-Return to normal physiology
-Residual effects of: coronary artery ischemia, aortic stenosis, or aortic regurg
Mustard Procedure:
-Make a more patent ventricular shunt
-Makes the RV systemic
-RV eventually wears out
Term
What is Eisenmenger's Syndrome?
Definition
-Irreversible pulm vasc disease from an unrepaired cardiac shunt (often VSD)
-PVR is higher than SVR because of pulm vascular obstruction.
-The shunt changes direction from L->R to R->L
-Leads to pulm HTN and Pulm Obstructive Disease
-A fixed PVR is unresponsive to pharm manipulation
Survival: 80% 10 years after diagnosis
:42% 15 years
:30% of all pregnancies end in maternal death
Term
What are some S/S of Eisenmenger?
Definition
-Clubbing
-Cyanosis
-Fixed pulm HTN
-R->L shunt
-Systemic HoTN (give vasopressin)
-Infective Endocarditis (need ABX)
Term
How do you treat a patient w/ Eisenmenger?
Definition
-21-30% FiO2
-GA to control ventilation and oxygenation
-Heparin (to prevent emboli)
-De-Air lines (can lead to paradoxical air emboli)
-Minimize cardiac & vascular depression
Term
What is Hypoplastic Left Heart Syndrome (HLHS)?
Definition
-AKA single ventricle syndrome
-Underdevelopment or absence of the LV & Asc. Aorta
-Stenosis or absence (atresia) of the aortic valve and mitral valve with little or no flow via AV
-Bloodflow is dependent on a PDA (maintained by PGE1) which provides blood to lower body, brain, coronaries in a retrograde manner
-Asc aorta is hypoplastic, atretic, or coarcted.
-Usually an ASD I present
Term
What happens to the chambers and valves during HLHS?
Definition
-Dilated Right Atrium
-Dilated RV
-Dilated main PA
-Dilated Tricuspid orifice (entire CO passes through here)
-PFO (allows pulm venous return)
Term
What happens to the lungs in HLHS?
Definition
Lungs are normal
Hypoxemia depends upon balance of SVR:PVR
Term
Describe systemic venous flow in a patient with HLHS.
Definition
SVC & IVC -> RA -> RV-> PA-> PDA->AO ->Body ->SVC&IVC
(LV is bypassed)
Term
Describe pulmonary venous flow in a patient with HLHS.
Definition
Pulm blood -> LA -> PFO -> RA -> RV -> PA -> Lungs -> pulm blood
Term
What are the options for a patient with HLHS?
Definition
-Cardiac transplant
-Three stage Norwood procedure
-Death (not the preferred option)
Term
Describe stage 1 of the Norwood procedure.
Definition
-The main PA and small AO are reconstructed to make a new larger AO
-This new AO arises from the RV to provide systemic blood flow
-Atrial septectomy (hole in the atrial septum) is made to allow BF from LA to RA to bypass the hypoplastic left heart
-The pulm valve is used to construct the new aorta
-New source of PBF is provided by creating a systemic PA shunt called a BT Shunt (Blalock Taussig)
Term
What is the goal of stage 1 Norwood?
Definition
-Create PBF w/ BT shunt
Achieved through:
-New aorta
-BT shunt
-Good common atrium
Term
What is the anesthetic plan for a stage 1 Norwood procedure?
Definition
-Keep pulm:systemic flow @ 1:1 ratio
-Maintain SPO2 70-80s
-Avoid pulm vasodilation (high PO2 or low PCO2)
Term
Describe the BT shunt.
Definition
-Connects the aorta to the Right PA
Term
Describe Norwood stage 2.
Definition
-Done at 2-6mos age
-Creation of a Bi-directional cavo-pulmonary connection (AKA Glenn)
-BT shunt is taken down
-SVC is connected directly to the right PA
Stabilizes circulation by:
-Decreases load on RV
-Diverts SVC directly to lungs
Term
What is the goal of Norwood stage 2?
Definition
To partially separate Red & Blue blood
Term
Describe Norwood stage 3.
Definition
-Blood from the IVC is redirected to the PA via a tube made of gortex
-Blood enters the lungs without being pumped
-Results in a RV that is exclusively for systemic circulation
-BF to the lungs depends on the transpulmonary pressure from VC into PA
-The flow is augmented by normal breathing and negative thoracic pressure
Term
What is the goal of a stage 3 Norwood?
Definition
Completely separate Red & Blue blood
Term
What are some possible SEs of a norwood and how are they treated?
Definition
High R sided pressures
-Fenestration are placed in the gortex tube to allow the heart to gradually adjust to the new BF
-S/S: pleural effusions, ascites, LE edema

Low CO
-Augmented by small R -> L shunt
-Patients can desat to 85-90%
Term
What is the anesthetic management plan for a patient who has had a Norwood procedure?
Definition
-Avoid excessive PPV (reduces the transpulmonary pressure gradient resulting in lower CO)
-Low PEEP to promote venous return
-Avoid hypovolemia
-Might need ICU postop
Term
What are some reports you might want when doing a surgery on a patient with a repaired CHD?
Definition
-Recent physical
-Current S&S
-Previous surgical record
-Cath procedures and reports
-Recent ECHO
-EKG
Term
Why are TOF and single ventricle patients prone to arrhythmias?
Definition
Because their repairs involved manipulation along the conduction pathway.
Term
What are common medications a patient with a repaired CHD might be on?
Definition
-ACE
-BB (especially TOF)
-Amiodarone (watch for arrhythmias)
-Diuretics (CHF patients)
-ASA
-Anti-platelet regimes
Term
Why might a patient with a repaired CHD be on Coumadin?
Definition
-Might have a mechanical valve
-Avoid thrombus is created shunts
Term
If a patient with a repaired CHD has a PM or AICD, what do you want to know?
Definition
-Underlying rhthym
-Device settings
Term
What is the mainstay of diagnostic testing for a patient with a repaired CHD?
Definition
ECHO
Term
Does a patient with a repaired CHD always need a cardiology consult prior to anesthesia?
Definition
Not if their cardiac function is corrected and compensated and they have been consistently followed by their cardiologist.

Must have a cardiology consult & ECHO within 3-6 mos:
-Complex lesion
-Not well compensated
-Cyanotic or single ventricle patient
-If they have changes significantly since their last visit to the cardiologist
Term
Where should surgeries be done on a patient with a repaired CHD?
Definition
-Non-cardiac procedures for well compensated patients can be done at a community hospital
-Poorly compensated or single ventricle patients should be done in a specialty center
-Complex pts should be able to be admitted if needed
Term
What are some common preop orders for a patient with a repaired CHD?
Definition
-Continue cardiac meds
-Standard NPO
-Patients with cyanotic lesions, are shunt dependent, or have an outflow tract obstruction should not be NPO for a long time (clears up to 2 hours prior)
-ASA stopped 7-10 days prior to major surgery
Term
What are some considerations for a patient with a repaired CHD presenting for a dental procedure?
Definition
Need excellent dental hygiene to prevent infective endocarditis
Term
What are some considerations for a patient with a repaired CHD presenting for a laparoscopic procedure?
Definition
Caution with high insufflation pressures
-Increases intra-abdominal pressures
-hypercarbia and decr venous return result
-Not tolerated in single ventricle or fontans pats
Term
What are some considerations for a patient with a repaired CHD presenting for a scoliosis procedure?
Definition
-Hypovolemia and HoTN not well tolerated
-Careful blood and volume replacement (esp fontan pts)
-SC monitoring
Term
What are some considerations for a patient with a repaired CHD presenting for a craniofacial procedure?
Definition
Cleft lip. Palate, or craniosynostosis repair:
-Prevent air embolus
-monitor and treat blood loss carefully
Term
What should the anesthic plan be for a patient with a repaired CHD presenting for MRI or diagnostic testing?
Definition
Sedation or GA are acceptable depending on hemodynamic and resp tolerance
Term
What are some considerations for a patient with a repaired CHD presenting for a elective procedure?
Definition
If they have a single ventricle, the procedure should be delayed until after the cavopulmonary connection is completed.
Term
What are some examples of patients that should have surgery at a Tertiary Care Children’s Hospital?
Definition
-Single Ventricle
-Unrepaired cyanotic CHD
-Repaired CHD w/ poor ventricular function
Term
For a patient with CHD, what are the considerations of using dexmedetomidate?
Definition
-Works well to smooth things out after anesthesia
-Can cause bradycardia and HoTN
Term
For a patient with CHD, what are the considerations of using opioids?
Definition
-Good option
-Used in caudals and nerve blocks
Term
For a patient with CHD, what are the considerations of using neuraxial blocks?
Definition
Lumbar/Thoracic spinals and epidurals should be avoided.
Term
For a patient with CHD, what are the considerations of using propofol?
Definition
Ok to use, but avoid venous dilation.
Term
For a patient with CHD, what are the considerations of using ketamine?
Definition
Good choice since it preserves myocardial function.
Term
For a patient with CHD, what are the considerations of using etomidate?
Definition
Excellent choice.
Little effect on hemodynamics.
Term
What are the indications for Infective Endocarditis Prophylaxis?
Definition
1) Cardiac anomaly
2) Prior to certain surgical procedures
Term
What are the cardiac implications for prophylaxis of Endocarditis?
Definition
A) prothetic valve or material
B) previous IE
C) unrepaired CHD including shunts and conduits, completely repaired CHD with prosthetic material or device first 6 mos after procedure, repaired CHD with residual defects at or near site of prosthetic patch or device
D) Cardiac transplants
Term
What are the surgical indications for prophylaxis of Endocarditis?
Definition
-Dental procedures
-Respiratory tract
-Procedures on Infected skin or musculoskeletal tissue
Term
What medications are given for prophylaxis of Infective Endocarditis?
Definition
Ampicillin (50mg/kg Im/IV x1 max 2g/dose)

Clindamycin, Cefzolin, Ceftriaxone can also be used
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