Pediatric Cardiology & Congenital Heart Disease - Quiz 5 Flashcards
What are the most common Congenital Heart Defects?
Bicuspid Aortic Valve
Ventricular Septal Defect (VSD)
Atrial Septal Defect (Secundum)
Cyanotic Lesions
TOF
What are some Environmental Factors that contribute to Congenital Heart Defects?
Chronic Maternal Disease
Maternal Meds, Drug, & ETOH Abuse
What is Retinoic Acid?
Found in cosmetics to treat skin problems, but has teratogenic properties, so preggos should avoid
What is the result of the Recirculation of Pulmonary Venous Blood?
L –> R Shunt
What results with the Recirculation of Systemic Venous Blood?
R –> L Shunt
What happens w/ Single-Ventricular Physiology?
Ventricle pumps out Mix of Pulm. & Systemic Blood
Distribution depends on Intra/Extracardiac Resistance in 2 parallel circuits
O2 Sat is the same in both Aorta & Pulm. Artery
Life is not compatible w/ Parallel Blood Flow Circuits w/ Transposition of the Great Vessels (TGA). How is this problem solved?
Intercirculatory Mixing provided by communication via a ASD, VSD, PFO, or PDA.
In regards to Intercirculatory Mixing, what determines the Arterial Saturation?
Volumes & Sats of Recirculated Systemic & Effective Systemic Venous Blood reaching the Aorta
What is Fontan Physiology?
When Systemic Venous Blood passively flows directly into the Pulmonary Circulation, then returns to a common atrium and single venticle.
Which heart conditions cause Cyanotic Heart Disease w/ Decreased Pulmonary Flow?
TOF
Tricuspid Atresia
Univentricular Heart w/ Pulm. Stenosis
Which heart conditions cause Cyanotic Heart Disease w/ Increased Pulmonary Flow?
Transposition of Great Arteries (TGA)
&
Total Anomalous Pulmonary Venous Return
Which heart conditions cause Acyanotic Heart Disease d/t a L–>R Shunt Lesion?
VSD
ASD
AVSD
PDA
Which heart conditions cause Acyanotic Heart Disease d/t an Obstructive Lesion?
Aortic Stenosis
Pulm. Valve Stenosis
Coarctation of Aorta
What are the One-Ventricle Lesion heart conditions?
Hypoplastic Left Heart Syndrome
Tricuspid Atresia
Double Inlet LV
Unbalance AVSD
What are the Two-Ventricle heart conditions?
Truncus Arteriosus
TOF w/ Pulm. Atresia
Severe Neonatal Aortic Stenosis
What are the LV Obstructive Lesion heart conditions?
Mitral Stenosis
Aortic Stenosis
Coarctation
Mixing of Systemic & Pulm. Venous Blood
What is the PO2 & O2 Sat in the Placenta?
PO2: 32-35 mmHg
O2 Sat: 80%
In Fetal Circulation, what is the PO2 & O2 Sat when the blood in the IVC joins the SVC Drainage?
PO2: 12-14 mmHg
O2 Sat: 40%
In Fetal Circulation what is the PO2 & O2 Sat of the blood traveling from the Ascending Aorta to the Brain, Coronary Arteries & Upper Limbs?
PO2: 20-22 mmHg
O2 Sat: 65%
What is the PO2 & O2 Sat for the Blood in the Descending Aorta in regards to Fetal Circulation?
PO2: 20-22 mmHg
O2 Sat: 55%
Why is the P50 Lower in Fetal Hgb?
To help w/ O2 Uptake in the Placenta
With Fetal Circulation, How does the RV compare to the LV?
Same Size & Thickness
RV has Higher Output @ 1.3 : 1
Pumps 450 mL/kg/min
What causes the Foramen Ovale to close?
When LA pressure is more than RA Pressure causing the FO’s Flap Valve to close against the Septum Secundum
Functional Closure of the Ductus Arteriosus happens in 10-72 hrs after birth. What else happens at this time?
↑Arterial O2 Tension
↓Prostaglandins
Lung Expansion releases Bradykinin
What happens to Cardiac Output after Birth?
Increases 30-80%
What is the Main Purpose of Transitional Circulation?
Separate Systemic & Pulm Circulation
Close FO & DA (Reversible for a few days)
What can cause the FO to Reopen?
Crying & Pain
Hypoxia
Hypercarbia
Acidosis
Lung Disease
Sepsis
How long does it take for the Anatomic Closure of the FO?
3 months & 1 year when the Septum Primum & Secundum adhese
How long does it take for the Anatomic Closure of the DA?
1 - 3 months when the Ligamentum Arteriosum forms
What can cause the Ductus Arteriosus to Reopen?
Decrease in O2 Tension
What is Alprostadil used for?
Prostalglandin E1 - used to open the DA
(and also to treat ED)
What are signs of a PDA?
Hyperactive Precordium
Bounding Pulse
Wide Pulse Pressure
Hepatomegaly
Tachypnea
Tachycardia
What med can be given to close a PDA?
Indomethacin - prostaglandin inhibitor
What is the Blood Volume of a Premi?
100 mL/kg
What is the Blood Volume for a < 3 month old?
90 mL/kg
What is the Blood Volume for a baby older than 12 months?
Same as Adult 70 mL/kg
What is the Blood Volume for a 3-12 month old baby?
80 mL/kg
With Fetal & Newborn hearts, how is Cardiac Output increased?
By Increasing HR b/c increasing preload has little effect
What is the Normal Respiratory Rate & Heart Rate for an Infant?
RR: 30 - 60
HR: 120 - 160
What is the Normal BP for a Premi vs. a Neonate?
Premi: 50/25
Neonate: 70/40
PVR starts to fall at birth but does not reach adult level until ______
PVR starts to fall at birth but does not reach adult level until 6 MONTHS
Why should Hypoxia & Pressors be avoided in babies?
To prevent R–>L Shutning & RV Dysfunction
What type of Heart Defects are Volume Overload Lesions?
ASD
VSD
AVSD
PDA
Truncus Arteriosus
L–>R Shunting
With Volume Overload Lesions, where would the Lesions need to be to cause Right Heart Dilatation?
Proximal to Mitral Valve
Distal = Left Heart Dilatation
How are Volume Overload Lesions treated?
Diuretics
Afterload Reduction
Surgery
What are the major types of ASD?
- Secundum ASD - @ Fossa Ovalis - Most common
- Primum ASD - Lower, Form of AVSD, MV Cleft
- Sinus Venosus ASD - High, r/t Partial Anomalous Venous Return - Least Common
What are signs of ASD?
Mostly Asymptomatic
Rarely CHF
Easily Fatigued
Mild Growth Failure
No Cyanosis unless Pulm. HTN
How is an ASD fixed?
Surgery/Catheter Closure when kid is 2-5 y.o to avoid late complications
When is Surgical Repair of an ASD indicated if the child is younger than 2 y.o?
When the kid has CHF or Severe Pulm. HTN
It’s too late when shunt is reversed while having Pulm. HTN
What are the different types of VSDs?
- Perimembranous - Most Common
- Infundibular (Subpulmonary/Supracristal) - Involves RVOT
- Muscular - single/multiple
- AVSD - Inlet VSD, Involves AV Valve Abnormalities
What causes the L–>R shunt w/ VSDs?
PVR is less than SVR, NOT Higher LV pressure
At what size is it considered a Large VSD?
> 6 mm & will need repair
50% of Small VSDs close on their own
What are symptoms of Large VSDs?
CHF
Failure to Thrive
Resp. Infections
Exercise Intolerance
Hyperactive Precordium
What is the treatment for VSDs?
Small VSDs - No Surgery, just Endocarditis Prophylaxis
Large VSDs - Start w/ afterload reducers & diuretics, then surgery
How does a AVSD develop?
Incomplete Endocardial Cushion Fusion
What genetic disease is AVSD common seen in?
Trisomy 21 (Down’s)
What are signs & symptoms of AVSD?
(Looks like ASD)
CHF
Pulm. Infections
Failure to Thrive
Easily Fatigued
What is the treatment for AVSD?
Surgery Always
Treat CHF
Pulmonary Banding
What is the Coarctation of the Aorta?
Narrowing of Aorta at any point from the Transverse Arch to Iliac Bifurcation
98% Juxtaductal
Commonly associated w/ Bicuspid Aortic Valve
What genetic syndrome is Coarctation seen in?
Turner’s Syndrome
What are signs & symptoms of Aorta Coarctation?
Weak or Absent Fem Pulses
Higher BP in Upper Extremities than Lower
Pulse Differences b/t R & L Arms
Cyanosis if Ductus is Open
Lower Extremity Hypoperfusion
Cardiomegaly & Rib Notching
What is the treatment for Aorta Coarctation?
Maintain Ductus w/ Prostaglandin E
Surgery to prevent LV Dysfunction
Angioplasty
Balloon Angioplasty for Re-Coarctation
What should be given for Pulmonary Blood Flow Obstruction?
Prostaglandin E1 to manage cyanosis
What happens w/ Parallel Circulation?
RV ejects into Aorta
LV ejects into Pulm. Circulation
Mixing occurs at Atrial, Ventricle, or Ductal Levels
What is needed to Maintain Ductal Patency and to Enlarge the Atrial Shunt?
Prostaglandin E1 to maintain Ductus Patency
&
Balloon Septostomy to Enlarge Atrial Shunt
What happens w/ Double Inlet LV?
Both Atria empty into LV
What Four critieria are needed to be considered a Tetrology of Fallot?
(Most common Cyanotic CHD)
VSD
Overriding Aorta
RVOT Obstruction
RV Hypertrophy
What happens in a Tet Spell?
Dynamic Narrowing w/ Hypercyanotic Episodes
&
↑R–>L Shunt
What can cause a Tet Spell?
Crying
Feeding
Anesthesia
Surgical Stimulation
Acidosis
What is included in the Urgent Intervention for a Tet Spell?
Phenylephrine 0.5-1 mcg/kg
Norepinephrine 0.5 mcg/kg
100% O2
Fluid Bolus
Sedation
Bicarb
B-Blockers
What syndromes are commonly associated with TOF?
DiGeorge & Trisomy 21
For a PDA case, what might be given to the patient?
Blood
ABx
Vitamin K
What are the complications associated w/ PDA surgery?
Difficulty Ventilation
Desaturation
Hemorrhage
Accidental Aorta/Pulm. Artery Ligation
What monitoring devices should be included along with the standard monitors for a PDA?
Two Pulse Ox - One on Right Hand & Lower Limb
During PDA surgery, what might be the cause if the Pulse Ox on the Lower limb is lost?
Clamping of the Aorta
What signs would you see at birth for a kid with a Hypoplastic Left Heart?
Tachypnea
Tachycardia
Cyanosis
Systolic Murmur
What is the Surgical Goal for a Hypoplastic Left Heart?
Transition to Single Ventricle Circulation where the Pulm. Blood Flow is supplied by the SVC & IVC
What happens in the Norwood Stage 1 of Hypoplastic Left Heart Surgery?
Move the Aortic Arch so that it comes from the Pulm. Trunk
Pulm. Valve becomes Aortic Valve
BT Shunts blood from Subclavian Artery to PA
Sano Mod Shunts RV to PA
What happens during the Norwood Stage 2 Hypoplastic Left Heart Surgery?
Disconnect BT Shunt
Make Glenn Shunt by connecting SVC to PA
What happens during the Norwood Stage 3 Hypoplastic Left Heart Surgery?
Fontan
SVC & IVC connected to PA
What the normal Saturation for a BT shunt?
75%
What’s the normal Saturation for a Glen Shunt?
85%
What’s the normal Saturation for a Fontan?
95%
How is Transposition of the Great Arteries fixed?
Arterial Switch Procedure - arterial trunk relocated to respective ventricles & coronary arteries relocated to aortic root
How is a Truncus Arteriosus repaired?
Close the VSD w/ Homograft
Which med is good for sick neonatal hearts?
Milrinone - PDE3 Inhibitor that increases cAMP & Calcium to relax smooth muscles and enhance both parts of the cardiac cycle
A newborn has T-Waves upright in all leads, but gets inverted in V1-V4 by one week of age, then back upright at adolescence. What is indicated if they dont invert at one week?
Right Ventricular Hypertrophy
How does the QRS axis appear at birth?
Right Sided QRS, then Left sided by 1 month of age
How does an Infant’s Cardiac Output compare to an Adult’s?
Healthy Full Term Infant has 2-3x the Cardiac Output of an Adult
How would an SVR Decrease or PVR Increase affect a R –> L shunt?
Increases the R –> L Shunt
What is associated with Kawasaki Disease?
Mucocutaneous Lymph Node Syndrome
Vasculitis
Dilated Coronary Arteries
Aneurysm
MI
Covid-19
What are signs and symptoms of Trisomy 21?
Small Size
Short Neck
Small & Low Ears
Macroglossia
Mandibular Hypoplasia
Narrow Nasopharyx
Hypotonia
What conditions are commonly associated w/ Trisomy 21?
OSA
MR
Spine Disorders
Thyroid Disease
Subglottic Stenosis
How does Trisomy 21 affect the CV system?
50% pts have CV Defects
ASD
VSD
TOF
PDA
Pulm. HTN
Bradycardia w/ Anesthesia
What are the signs & symptoms of the X-Linked disorder Turner Syndrome?
Webbing
Micrognathia
Short Stature
Lymphedema
Ovarian Failure
How does Turner Syndrome affect the CV System?
Aortic Coarctation
Biscuspid Aortic Valve
HTN
What conditions are commonly associated w/ Turner Syndrome?
Obesity
DM
Hypothyroidism
Liver Disease