Exam 3 CHD 1-3 Flashcards
What is the most common form of congenital pathology?
Congenital Heart Disease (0.3-1.2% of live births)
What is the most common cardiac defect?
Bicuspid Aortic Valve (30% of all Congenital Heart Diseases, 1% of all life births)
What is the second most common cardiac defect?
VSD
What is the third most common cardiac defect?
ASD
What kind of lesion accounts for about 6% of all Congenital Heart Disease cases? And what disease is the one that predominates these types of lesions?
Cyanotic. Tetralogy of Fallot predominates.
What are some of the most common congenital heart diseases that people are surviving to adulthood with?
Bicuspid Aortic Valve Coarctation of the Aorta Pulmonic Valve Stenosis Ostium Secundum ASD VSD PDA
Chromosomal abnormalities are associated with ____% of congenital CV lesions. What are these (4)?
10%
Trisomy 21 (Down Syndrome) Trisomy 13 (Patau Syndrome) Trisomy 18 ( Edwards Syndrome) Turner's Syndrome
Congenital Hear Diseases due to a defect in chromosome 22 are groups as “Catch 22” disorders. What does CATCH 22 Stand for?
C- Cardiac Defects A- Abnormal Facies T- Thymic Hypoplasia C- Cleft Palate H- Hypocalcemia 22- The chromosome defect associated with these features.
What are the L to R shunt lesions?
Atrial Septal Defects Ventricular Septal Defects Atrioventricular Canal Defects Patent Ductus Arteriosus Aortopulmonary Window
What are the R to L shunt lesions?
Tetralogy of Fallot
Ebstein’s Anomaly
Pulmonary Stenosis in conjunction with atrial or ventricular septal defects (high PVR causes R to L Shunt)
Eisenmenger’s Syndrome
What is the Transposition Physiology lesion?
Dextro-Transposition of the Great Arteries
What are the One-Ventricle Lesions?
Hypoplastic Left Heart Syndrome
Tricuspid Atresia
Double Inlet Left Ventricle
What are the Two-Ventricle Lesions?
Truncus Arteriosus
Tetralogy of Fallot with Pulmonary Atresia
Severe Neonatal Aortic Stenosis
Left Ventricular Obstructive Lesions
Mitral Stenosis (Valvular, Cor Triatriatum) Aortic Stenosis (Valvular, Subvalvular (Subarotic Membrane) Supravalvular (Williams-Beuren Syndrome)
Coarctation Lesions
Shone’s syndrome (mitral stenosis, aortic stenosis, coarctation)
Mixing of Systemic and Pulmonary Venous Blood with Series Circulation
Partial anomalous pulmonary Venous Return (PAPVR)
Total anomalous pulmonary Venous Return (TAPVR)
Tables GALORE
List the Lesions that cause Outflow Tract Obstruction
LV- Coarctation of the Aorta and Aortic Stenosis
RV- Pulmonic Valve Stenosis
Lesions causing L to R Shunting
VSD PDA ASD Endocardial cushion defect Partial anomalous pulm. ven. return
Lesions causing R to L shunting
TOF Pulm. Atresia Tricuspid Atresia Transposition of the Great Arteries Truncus Arteriosus Single Ventricle Double - Outlet RV Total Anomalous Pulm. Ven. Return Hypoplastic Left Heart
Volume overload Lesions
ASD VSD AV Septal Defect PDA Truncus Arteriosus
Obstruction to Systemic Blood Flow
Aortic Stenosis
Coarctation of the Aorta
Interrupted Aortic Arch
Hypoplastic LH syndrome
Obstruction to Pulmonary Blood Flow
Pulmonary Stenosis
ToF
Pulmonary Atresia
Parallel Circulation
D- Transposition of the Great Arteries
Single Ventricle Lesions
Tricuspid Atresia
Double Inlet LV
Unbalanced AV Septal Defect
Intrinsic Myocardial Disorders
Cardiomyopathy
Myocarditis
What are the Cyanotic Defects?
TOF
Transposition of the Great Vessels
Hypoplastic Left Heart
Hypoplastic Right Heart
What are the acyanotic defects?
All the other ones that are not (TOF,TGV,HLH,HRH)
What are the 5 differences in Fetal and Adult Circulation?
- Placenta is the organ of respiration
- Fetal Circulation is parallel; adult is in series
- R-L shunting occurs across FO and DA
- PVR is high in fetus (lungs collapsed and full of fluid)
- SVR is low in fetal circulation (placenta is a large, low resistance vascular bed).
Acyanotic CHD is characterized by which type of shunt?
L to R Shunt
Pathophysiology of Acyanotic CHD (or L-R shunt)
Increased Pulmonary Blood Flow —> Increased PVR—> Intimal Hyperplasia and Vascular Remodeling
What is the end result of L-R Shunt (or Acyanotic CHD)
Pulmonary HTN, RVH, and CHF
Atrial Septal Defect (ASD) accounts for 1/3 of CHD in adults, it is 2-3x more frequent in _____
females
List the types of ASD
Patent Foramen Ovale Ostium Secundum Ostium Primum Sinus Venosus (near SVC or IVC junction) Coronary Sinus Common Atrium
Physiologic consequences of all ASDs are the same. Direction and Magnitude of shunt depends on __________&___________.
Size of Defect and Ventricular Compliance
Defect of ______ diameter has little to no effect.
<0.5cm
Defect of _____ diameter has large and significant effects.
> 2 cm
When pulmonary blood flow is ____x systemic blood flow, ASD should be closed.
1.5x
Symptoms of a large ASD includes:
Dyspnea on Exertion SVTs RHF Paradoxical Embolism Recurrent Pulmonary Infections
Anesthetic considerations with surgical repair of ASD
GA with invasive monitoring
Access to CPB and ability to treat potential heart block
Anesthetic considerations with percutaneous (endovascular) repair of ASD
Deep Sedation or GA
Noninvasive or minimal monitoring
For surgical correction of ASD, extubation should occur (early or late)
Early- Most extubated in OR or early ICU. Use small doses of opioids or Remifentanil/Propofol Gtts.
Which type of Ventricular Septal Defect (VSD) is the most common (70-80%), known as conoventricular, perimembranous or infracristal, is associated with valvular aneurysms or insufficiency and located lower in septum, just below crista terminalis.
Type 2
What type of VSD is known as endocardial cushion, inlet or canal-type, accounts for 5% of VSDs, is a AV canal defect, and located below mitral and tricuspid valves.
Type 3
What type of VSD accounts for 10-20% of VSDs, known as “muscular”, associated with multiple lesions, located deep in muscular portion of septum and can have single to multiple perforations.
Type 4
Which type of VSD is known as conal, sub-arterial, supracristal outlet, subpulmonic or infundibular, account for 5% of VSDs, located high in the interventricular septum (just below the pulmonic valve above the crista terminalis), and is associated with aortic insufficiency.
Type 1
Which type of VSD typically occurs with Down Syndrome?
Type 3
What is the most common type of VSD and the incidence %.
Type 2, account for 70-80% of all VSDs
What PVR:SVR ratio is indicative for small VSD?
Qp:Qs 1.4:1 or less
What PVR:SVR ratio is indicative for moderately restrictive VSD?
Qp:Qs 1.4:1-2.2:1
What type of shunt is known as a restrictive shunt?
What Sx are associated with this type?
Small (1.4:1 or less) “the amount of the shunt is restricted by the size of the defect”. No Sx
What PVR:SVR ratio is associated with a nonrestrictive VSD?
Qp:Qs 2.2:1 or greater.
What Sx progression is associated with Moderatly to nonrestrictive VSD?
Equalization of RV and LV pressures —> Volume and Pressure Overload of Pulmonary System (Pulm HTN).
End Result: L-R Shunt becomes a R-L shunt which leads to hypoxia as blood bypasses the lungs.
Can surgical correction be performed in Pt with Nonrestrictive VSD?
None—Will cause RH failure. So best to correct early before disease progression.
What heart sound is associated with VSDs?
Holosystolic Murmur heard best at the Left Sternal Border.
What tests can help determine the severity of VSDs?
CXR- May show cardiomegaly
EKG- May show enlarged cardiac silhouette.
Echo- used to evaluate most to evaluate presence, directionality & severity of VSD
Anesthetic management concerns with VSD
Small (restricted) defect (<1.4:1) can be closed percutaneously.
Most undergo surgical closure
Post-surgical complications with VSD correction
A-V block V-Tach CHF Pulmonary HTN Inotropic support may be needed
Atrioventricular Septal Defect (AVSD) is what type of VSD?
Type 3
AV Canal Defect, or Endocardial Cushion Defect is other names for
Atrioventricular Septal Defect (AVSD)
What are the two common types of AVSDs
Partial and Complete
Describe the two common types of AVSDs
Partial- Primum ASD with cleft in anterior MV leaflet
Complete- Large septal defect with atrial and ventricular components, common AV valve.
What 3 disorders are associated with AVSDs?
Trisomy 21
Tetralogy of Fallot
DiGeorge Syndrome
What type of shunt with AVSDs?
L to R shunt
AVSD is (cyanotic/acyanotic) disorder?
Acyanotic
What hemodynamic changes are associated with AVSDs?
AV valve regurgitation and Shunting (L to R)
Can result in Eisenmengers Syndrome (L to R –> R to L cyanotic shunting).
Anesthetic Management with AVSD
Small defect w/o Mitral Regurg- Inhalation Induction
IV Induction use Ketamine 1-2mg/kg, Fentanyl 5-10mcg/kg, Rocuronium 1mg/kg
Maintenance-Volatile agents and narcotics
Emergence- Will probably require Postop Ventilation.
More Anesthetic Management with AVSD
Inotropes may be needed Postop Pulmonary HTN may occur TEE helpful in assessing repair Heart Block may occur postop Associated with Trisomy 21- So all anesthetic management needs for Down Syndrome
Disorder where blood flows from the Aorta to the Pulmonary Artery (L to R Shunt)
Patent Ductus Arteriosus (PDA)
Amount of shunt in PDA depends on:
Size of ductus, pressure difference between aorta & PA, & ratio between PVR & SVR
Sx with mild moderate shunting with PDA:
asymptomatic
Sx with large PDA shunt:
Ventricular hypertrophy & pulmonary hypertension
Leads to- Heart failure, failure to thrive, aneurysmal dilatation of ductus, Eisenmenger syndrome
AVSDs are more common among
Down Syndrome (Trisomy 21)
PDAs are more common among
Preterm Infants
What is the 1st line Treatment for PDA?
Indomethacin (cyclooxygenase inhibitor, decreases the production of prostaglandins) (Prostaglandins keep the ductus open)
Infants with PDA, who fail to respond to Indomethacin Tx, will require:
Surgical closure
Preop requirement with surgical correction of PDA
Crossmatched blood available
Antibiotics
Vitamin K
What are the perioperative risks associated with PDA repair?
Difficulty ventilating/desaturation; accidental extubation; tearing PDA; recurrent laryngeal nerve damage; phrenic nerve paralysis; chylothorax; ligation of aorta or PA; endocarditis; paradoxical air embolism
Standard monitoring for PDA repair
2 pulse ox (1 right hand, 1 on lower ext)
1 BP cuffs (1 right arm, 1 on lower ext)
Anesthetic management considerations with PDA repair
Have dedicated IV for fluids and drugs (Duh)
High-dose opioids are good option (30-50mcg/kg)
Muscle Relaxation
ETT with small air leak
Avoid Decreasing PVR, Myocardial Contractility and SVR
Use Dopamine to maintain BP
Rule of Thumb with determining where to keep MAP for infants
Keep MAP equal to Gestational Age
PDA repair induction agents
Ketamine 1-2mg/kg, Rocuronium 1mg/kg, fentanyl 2-5mcg/kg
or Sevo+N2O/O2 Inhalation induction
Maintenance agent for PDA repair
Sevo or ISO
Emergence from PDA repair
Older patient with isolated PDA may be extubated awake in OR. Most others and especially neonates will be kept on vent. postop.
What is the major left-sided obstructive lesion occurring at valvular, subvalvular, supravalvular or a combination of these locations?
Aortic Stenosis
Aortic Stenosis is frequently due to _________
Bicuspid Aortic Valve
Aortic Stenosis is often _____ until adulthood
asymptomatic
A mean aortic valve gradient of ___ mmHg is considered mild.
20
A mean aortic valve gradient of ___mmHg is considered severe.
40
Infants with severe aortic stenosis (which is usually subvalvular) will have these problems
Feeding Difficulties
Poor Growth
Heart Failure
Supravalvular Aortic Stenosis is associated with
Round forehead, prominent facial bones, pursed upper lip and Williams syndrome
Both Subvalvular and Supravalvular Lesions have a high risk of
Sudden Death under Anesthesia
Patho of Aortic Stenosis
O2 Supply/Demand Imbalances, LV Hypertrophy and LV Failure.
Impaired coronary blood flow d/t low CPP and increased workload on LV —> ischemia
The ____ ventricle is more sensitive to afterload than the ____ ventricle.
The RIGHT ventricle is more sensitive to afterload then the LEFT ventricle.
What are the 4 types of Pulmonic Stenosis?
Valvular Pulmonary Stenosis
Supravalvular Pulmonary Stenosis
Subvalvular (infundibular) Pulmonary Stenosis
Branch Peripheral Pulmonary Stenosis
Pulmonary artery just above pulmonary valve is narrowed. Associated with ASD, VSD, PDA, and ToF.
What is Supravalvular Pulmonary Stenosis?
Valve leaflets art thickened and/or narrowed. Usually occurs in isolation of other congenital issues.
What is Valvular Pulmonary Stenosis?
Right or Left Pulmonary Artery (or both) is narrowed
What is Branch Peripheral Pulmonic Stenosis?
Muscle under valve area is thickened, narrowing tract from the RV. Associated with VSD.
What is Subvalvular (infundibular) Pulmonary Stenosis?
_______ Gradient is used for classification of Pulmonary Stenosis Classification, rather than Mean Gradient.
Peak Gradient
Peak Gradient that is associated with Mild Pulmonic Stenosis.
<36
Peak Gradient that is associated with Severe Pulmonic Stenosis?
> 64
List the Signs Sx of Pulmonic Stenosis and what will heard on auscultation and seen on ECG.
Sx- Right sided HF Sx (Dyspnea, JVD, Peripheral Edema and Asities)
Auscultation- Systolic Ejection Murmur at 2nd ICS
ECG- Right Ventricular Hypertrophy
Anesthetic Management of Pulmonic Stenosis
CPB or Valvuloplasty
Avoid Increases in RV O2 Requirements
Narrowing of the Aorta, close to the Ductus Arteriosus
What is Coarctation of the Aorta?
What are the 3 forms of Coarctation of the Aorta
Preductal
Juxtaductal
Postductal
Coarctation of the Aorta is more common in (Males or Females)?
Males
What is the most common (NEONATAL) form of Coarctation of the Aorta?
Preductal
What is the most common form of Coarctation of the Aorta? (Silly but yet in the slides)
Postductal
What type of Coarctation of the Aorta is associated with minimal collateral blood flow below coarctation and requires prostaglandin to maintain PDA?
Preductal
What types of CoA does have collateral blood flow below the coarctation?
Juxtaductal and Postductal
CoA accounts for ___% of all CHDs.
5% (so stupid)
Which group has the most adverse outcomes of CoA?
Neonatal or >1 year
Neonatal
Coa= Few Collaterals, poor LV function, weak femoral pulses, and progressive acidosis? (neonatal or > 1 year)
Neonatal
All forms of CoA have adverse effects, list them all:
Systolic HTN CHF Aortic Dissection Premature CAD Intracerebral Hemorrhage (aneurysm rupture)
Sx/Signs of CoA
Headache, Dizziness, Palpitation, Epistaxis
On exam: BP changes between upper and lower ext, widened pulse pressure, and diminished femoral pulse.
Newborn: selective cyanosis of lower extremities but with pink upper extremities and face.
CoA- Auscultation, BP, ECG, and CXR findings
Murmur along LEFT STERNAL BORDER
Different BP in Right Arm and Left Leg
LVH on ECG
Notching in posterior part of ribs (d/t increased collateral BF in the intercostal arteries) on CXR
Possibly see “Reversed E sign” on CXR (from coarctation and post stenotic dilatation)
Definitive Dx for Coa
Ultrasound, CT, MRI
Discuss Repair strategies with Coa
Done in infancy or early childhood before HTN develops- (if done after HTN, it may continue)
Repair can be done Percutaneously or Surgically
Percutaneously: Balloon Dilatation and Stent placement
Surgically: Patch or end to end Anastomosis
No CPB, but High Aortic Cross Clamp- managing 2 circulations.
Coa- IV access needs?
One for IV induction drugs
One for Prostaglandin E1
Surgical Approach for CoA?
Left Thoracotomy
Infants are cooled to a temp of ____ C to reduced spinal cord injury in CoA repair.
34-35 C
Most common Nerve Injury with CoA?
Left Laryngeal Nerve- Presents with Horseness or Stridor
Phrenic nerve injury is not as common during COA repair, but if occurs, can mean ______ may be needed.
Ventilatory support
CoA Induction for Neonate
Fentanyl 2-5mcg/kg IV, Roc 0.6-1 mg/kg IV
Low dose Isoflurane (0.3-0.5% if tolerates)
(Maintain HR, Preload, & Contractility to maintain CO)
Avoid Increases/Decreases in SVR and Avoid Myocardial Depression
Maintenance of Neonate for CoA (Fentanyl dose and what to do with prostaglandin).
Fentanyl 10-20mcg/kg, volatile agent, continue prostaglandin.
Emergence considerations with CoA
Keep SBP < ______
Consider ____ and _____ if cardiac function compromised.
Will require postop _____ . ______ tubes will be in place.
SBP < 120
dopamine and milrinone
ventilation
chest
Older children with CoA are (sicker/not as sick) as the neonate
not as sick- but will require same vascular lines and same precautions to protect spinal cord during CoA repair.
CoA- Treat postop HTN with
Nitroprusside and B-Blockers
Use cuffed tube with older children b/c extubation occurs______, compared to the neonate.
early
ToF, Truncus Arteriosus, Tricuspid Atresia, Hypoplastic Left Heart Syndrome, Anomalous Pulmonary Venous Connection and Eisenmenger syndrome are what types of CHD?
Cyanotic
Major characteristic of Cyanotic CHDs? (shunt direction and outcomes)
Right to Left Shunt–> Decreased pulmonary blood flow and hypoxia.
Severity of hypoxemia is determined by
pulmonary to systemic blood flow (Qp:Qs<1)
Associated problems with cyanotic CHDs
Erythrocytosis and Hyperviscosity
May have abnormal coag studies
HF, Pulm. HTN, Dysrhythmias, Dec lung compliance, altered response to hypoxia and hypercarbia, renal insufficiency.
What is the Most Common CYANOTIC Lesion?
Tetralogy of Fallot (ToF) accounts for 6-11% of all CHDs
What are the 4 features of ToF?
- Perimembranous VSD
- Aorta Overriding VSD
- RVOTO
- RVH
What are the 5 features of Pentrology of Fallot?
- Perimembranous VSD
- Aorta Overriding VSD
- RVOTO (aka pulmonary stenosis)
- RVH
- ASD
4 major defects of To4 (same as the features, but worded better from another slide)
- Pulmonary Stenosis (RVOTO)
- RVH
- Overriding Aorta
- VSD
Spell or Episodes associated with ToF
Tet Spells (Hypercyanotic Episodes)
Classic presentation of Tet Spells (Hypercyanotic Episodes)
Cyanosis, Hyperpnea, Possible LOC, Stroke, Seizures, or even Death.
Tet Spells (Hypercyanotic Episodes) occur ding periods of _____
Stress (crying, feeding, exercise, surgical stress, and anesthesia.
Proposed mechanism of Tet Spells
Spasm of the infundibular portion of the RVOT along with peripheral vasodilation and hyperventilation.
Susceptible period for Tet Spells?
2-3 months, however, can occur anytime during 1st year of life.
Squatting causes what during Tet Spells
Increased Peripheral Vascular Resistance and Decreased R to L Shunting.
Management of Tet Spells:
Phenylephrine 100mcg/ml, initial dose 0.5-1.0mcg/kg 100% O2 IV Fluid Bolus Sedation/Analgesia NaHCO3 Vasoconstrictors Beta-blockers
Additional Management of Cyanotic Spell (as from the chart)
Moist O2 Inhalation Knee Chest Morphine Sulphate 0.1-0.2mg/kg. IM/IV IV Propanolol 0.5mg/kg NaHCo3 (0.02mg/kg) Phenylephrine 0.02mg/kg Ketamine 1-3mg/kg IV Gen. Anesthesia Emergency Surgical Intervention
Surgical Management for ToF
Trend is early repair
Initial palliation surgery sometimes performed- Appoach is Thoracotomy, but can be sternotomy…with no CPB.
Anesthetic Goals for TOF
Maintain intravascular volume and SVR.
Avoid Increased PVR, especially from acidosis and excessive AW pressures. Ketamine is often used.
Postoperative Course for ToF
Potential for pulmonary reguritation
PFO sometimes left open as a “pop-up” valve to reduce RV pressures
RBBB is common
The palliative surgical Tx for ToF is the
Systemic to Pulmonary Shunt (Modified Blalock-Taussig Shunt)
Explain the Systemic - to - Pulmonary Shunt (Modified Blalock-Taussig Shunt)
Shunt from subclavian artery to branch pulmonary artery
What is always a risk during induction for these kids?
Hypercyanotic Spell During Induction
With TOF, avoid muscle relaxants that causes _____ release.
Histamine
TOF Tx- Place arterial line NOT ON THE SIDE where ________.
Shunt will be placed. Subclavian Artery will be clamped and pressure tracing will be lost.
Avoid _____ during ToF repair
Hypercarbia (This increases PVR and increases the R to L shunt)
Too much inotrope in ToF can worsen _____ postop.
RVOTO
_______ and ________ may precipitate Junctional Tachycardia
Pyrexia and Excessive Beta Adrenergic Stimulation
Best Inhalation Induction Agent with ToF?
Sevo
Best IV Induction Agent with ToF?
Ketamine 1-2mg/kg, Fentanyl 10mcg/kg, Roc 1mg/kg
Maintenance during ToF correction?
Iso or Sevo, Maintain Preload, HR, Contractility, and Treat Hypercyanotic Spells.
ToF surgery will probably need what postop?
Postop Ventilation.
