Pediatric Anesthesia Week 3 Flash Cards
What is the most common cyanotic congenital heart disease? How common? What is its classification?
- Tetrology of Fallot
- Accounts for 6- 11% of congenital heart disease
- “Simple” Right- to- Left Shunt
What are 2 major characteristics of Tetrology of Fallot? What are the other characteristics?
- VSD
- RV-Outlet Tract Obstruction (infundibular obstruction/ spasm)
- Overriding Aorta
- RV Hypertrophy
What is an “Overriding Aorta”?
In TOF, the aorta is displaced to the right so that it appears to arise from both ventricles and straddles the VSD
What are “tet spells”?
In TOF, hypercyanotic spells occur when there is an increase in RIGHT-to-LEFT shunting (increase in PVR)
What relationship determines the degree of RIGHT-to-LEFT Shunting, ergo the degree of hypoxemia in a patient with TOF?
The relationship between RV-Outlet Tract Obstruction (RVOTO) AND Systemic Vascular Resistance (SVR)
What causes “tet spells”? What triggers “tet spells”?
Unclear, but they occur during:
- Crying -Feeding
- Anesthesia/surgical stimulation -Metabolic acidosis
- Increased PaCO2 - Circulating catacholamines
How do you treat TOF- Hypercyanotic “tet spells”?
- 100% O2
- Hyperventilation (decrease in eTCO2 will decrease PVR)
- Increase PRELOAD (IVF)- give fluid deficit early or give bolus of 10 ml/kg of crystalloid
- Sedation
- Vasoconstrict with Neosynephrie (to INCREASE SVR to reverse R-L shunt)
- Use a BETA-BLOCKER to relax infundibular spasm and reduce HR
When is surgical repair of TOF considered? What is done?
- Early
- Complete repair which involves closure of the VSD and relief of RV outlet tract obstruction (RVOTO)
What is the name of the surgical procedure to repair a TOF?
Modified “Blalock-Taussig (BT) Shunt” to improve systemic-to-pulmonary shunt and improve pulmonary blood flow
The Modified “Blalock-Taussig (BT) Shunt” shunts from the ______ ________ to the _______ _______, creating a _______ blood flow.
- Subclavian Artery
- Pulmonary Artery
- Passive
What are some anesthetic considerations for a TOF repair?
- PRESEDATE to prevent crying on induction increasing risk for “tet spells”
- A-Line is placed on the arm OPPOSITE to the side of the anastomosis ( Subclavian artery is going to be clamped).
- SNUG ETT with NO AIR LEAK
- Prepare for BLOOD TRANSFUSION potential
What is predominantly dependent on the size of PT shunts?
Postoperative pulmonary blood supply
How does post-operative blood supply effect the size of the BT shent?
- If the BT shunt is too small = Low saturation
- If the BT shunt is too large = Infant may develop heart failure/ pulmonary edema
- TEE intraoperatively will assess RV function
Pulmonary blood flow is also dependent on ? How?
- Systemic blood pressure
- The greater the blood pressure, the more blood flow toward the lungs, thus increase in saturation
*Postoperative ventilation may be required
Does blood shunt right-to-left or left-to-right through the VSD in Tetrology of Fallow (Cyanotic Heart Disease)?
Blood shunt RIGHT-to-LEFT, permitting unoxygenated blood to mix with oxygenated blood, resulting in CYANOSIS
What are goals of anesthetic management for the patient who has Tetrology of Fallot?
- Maintain intravascular volume and SVR
- AVOID increase in PVR
What pharmacologic agent decreases a right-to-left shunt?
Any drug that increases SVR, like Phenylnephrine, increases SVR and decreases right-to-left shunt
** An infant has Tetrology of Fallot (cyanotic heart disease). Which of the following arterial blood gas parameters will NOT typically be changed: PaO2, pH, PaCo2?
pH and PaCo2 typically maintain in the normal limits
PaO2 is usually markedly decrease (< 50 mmHg)
During the case, SpO2 decreases, apparently because of increased shunting. The patient has Tetrology of Fallot. What agents might be selected to decrease shunt and increase SpO2?
- Volume must be maintained with IV FLUID administration as hypovolemia increases the magnitude of the right-to-left shunt
- An alpha agonist, such as PHENYLNEPHRINE must be available
What change in SVR and PVR increase shunt in a patient with Tetrology of Fallot patient?
- Shunt increases when SVR decreases or PVR increases
- Volatile anesthetics, drugs that release histamine,
What drugs increase shunt in the patient with TOF by altering SVR or PVR?
Drugs that decrease SVR such as:
- Volatile anesthetics
- Histamine releasing drugs
- Ganglionic blockers
- Alpha blockers
- Vasodilators, like Nitroprusside, that decrease SVR
Drugs that increase PVR such as:
- Nitrous Oxide (N2O), detreimental to children with right-to-left shunts
List 3 conditions that increase right-to-left shunt (Tetrology of Fallot)?
- Acidosis
- Hypercarbia
- Hypotension
List 4 congenital heart defects involved with Tetrology of Fallot (cyanotic heart disease)?
- VSD
- Right Ventricular Outflow Tract (RVOT) Obstruction i.e. Pulmonary Stenosis
- RV Hypertrophy
- Overriding Aorta- Dextroposition (to the right) of the aorta with overriding of the VSD
What is a Complex Shunt? What are the 5 types?
Mixing of pulmonary and systemic blood flow with cyanosis
- TGA- Transposition of Great Arteries
- Truncus Arteriosus
- DORV- Double-Outlet Right Ventricle
- HLHS- Hypoplastic Left Heart Syndrome
- TAPVC- Total Anomalous Pulmonary Venous Connection
What does TGA stand for? How is it defined?
- Transposition of the Great Vessels (Arteries)
- The Aorta arises from the RIGHT VENTRICLE, and the Pulmonary Artery arises from the LEFT VENTRICLE. The coronary arteries are shown arising from the aorta
- These children are cyanotic
What is the only way children with TGA can receive oxygenated blood?
Through their Patent/ Persistant Foramen Ovale (PFO) and Patent Ductus Arteriosus (PDA)
What kind of circulation is TGA considered? Does mixing occur? How often?
- Parallel Circulation: Two circulations run parallel rather than in series
- Yes, through the PDA and/or VSD
- Present in 25%
What is done to ensure ductal patency is maintained in a patient with TGA?
- An infusion of PROSTAGLANDIN E1
- Urgently performed BALLOON ATRIAL SEPTOSTOMY
- An Atrial Septal Defect is created to buy time
What procedure is performed to correct Transposition of Great Arteries? What vessels are disconnected?
- An “Arterial Switch” is performed
- The Aorta, Pulmonary Artery, and Coronary Arteries
During an “Arterial Switch”, the Pulmonary Artery is moved ________ to the Aorta. The Aorta is connected to the _____ ventricle. The Pulmonary Artery is connected to the _______ ventricle. The ________ ________ are connected to the _________ _____.
- Anterior
- Left
- Right
- Coronary Arteries
- Neo-Aortic Root
** What is considered the most crucial part of a successful outcome of an “Arterial Switch” procedure?
The successful anastomosis of the Coronary Arteries to the Neo-Aortic Root
When should an “Arterial Switch” be performed for a patient with TGA? What results if left untreated? If treated, what is the outlook on life?
- Often required early at 2 to 3 weeks of life
- If untreated, patient will usually die within 1 year due to HYPOXIA and HEART FAILURE.
- If successful, child can expect to live a normal life
What risks do these patients have post-Cardiopulmonary Bypass?
- Inherently poor Left Ventricle
- Poor Myocardial Protection
- Poor Coronary Transference
- Coronary Air
- Pulmonary Hypertension
- Left Atrial Dilation (caution with fluid boluses)
- Milirinone
This Complex Shunt has a common truncal valve and mixing of oxygenated and deoxygenated blood
Truncus Arteriosus
How common is Truncus Arteriosus?
Rare, usually only 1 % of Congenital Heart Defects
What is a characteristic of Truncus Arteriosus?
- Common arterial outlet for Aorta and Pulmonary Artery associated with SINGLE VALVE and VSD
- Mixed blood at arterial level with HIGH PULMONARY BLOOD FLOW leading to PULMONARY HYPERTENSION and eventually HEART FAILURE
How is Truncus Arteriosus surgically treated?
SEPARATION of PULMONARY circulation from SYSTEMIC circulation and close VSD with a VALVED CONDUIT
What is the post-operative mortality rate in surgically treating Truncus Arteriosus? What reasons?
High, 5-25%, due to:
- Truncal Valve Stenosis
- Coronary Abnormalities
- Pulmonary Hypertensive Crisis
- Low Birth Weight
This Complex Shunt has BOTH of the GREAT ARTERIES (Pulmonary Artery and Aorta) arising from the morphologic RIGHT VENTRICLE, often due to a LARGE VSD
Double Outlet Right Ventricle (DORV)
How common is Double Outlet Right Ventricle (DORV)?
Rare, around 1 % of Congenital Heart Defects
What 2 major genetic strands are associated with Double Outlet Right Ventricle (DORV)?
Trisomy 13 and Trisomy 18
Trisomy 13 and Trisomy 18 are associated with which Complex Shunt?
Double Outlet Right Ventricle (DORV)
If Pulmonary _______ is present, DORV resembles the physiology of ________ of ________.
- Stenosis
- Tetrology of Fallot
How much does the Pulmonary Valve have to be stenosed in order for DORV to resemble Tetrology of Fallot?
About 50%
Characteristics of this Complex Shunt include a VERY SMALL UNDERDEVELOPED LEFT VENTRICLE
Hypoplastic Left Heart Syndrome
What are characteristics of Hypoplastic Left Heart Syndrome?
- Very small underdeveloped LEFT VENTRICLE
- Mitral & Aortic valve STENOSIS/ATRESIA
- Hypoplastic Aortic Arch
- SINGLE VENTRICLE (RIGHT VENTRICLE)
How does pulmonary blood flow in patients with Hypoplastic Left Heart Syndrome (HLHS)? Systemic blood flow?
- Pulmonary blood flow from Left Atrium (LA) via Atrial Septal Defect (ASD) to Right Atrium (RA) and Right Ventricle (RV)
- Systemic blood flow from RV to Pulmonary Artery (PA) to Aorta via Patent Ductus Arteriosus (PDA)
How is blood supplied to the systemic circulation in patients with Hypoplastic Left Heart Syndrome (HLHS)?
The Patent Ductus Arteriosus (PDA) is the only flow to the systemic circulation (arising from the Pulmonary Artery to the Aorta)
What is the epidemiology of Hypoplastic Left Heart Syndrome (HLHS)? When it is diagnosed?
- In the U.S, 2 out of 10,000 live births
- Usually during prenatal diagnosis
How do neonates present when suspecting HLHS?
- Tachypnea
- Tachycardia
- Cyanosis
- Systolic Murmur
How many surgeries must be performed to convert HLHS? What is it converted into?
- 3
- SINGLE-VENTRICLE TYPE CIRCULATION