Pediatric Cardiology & Congenital Heart Disease - Quiz 5 Flashcards

1
Q

What are the most common Congenital Heart Defects?

A

Bicuspid Aortic Valve

Ventricular Septal Defect (VSD)

Atrial Septal Defect (Secundum)

Cyanotic Lesions

TOF

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What are some Environmental Factors that contribute to Congenital Heart Defects?

A

Chronic Maternal Disease

Maternal Meds, Drug, & ETOH Abuse

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is Retinoic Acid?

A

Found in cosmetics to treat skin problems, but has teratogenic properties, so preggos should avoid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the result of the Recirculation of Pulmonary Venous Blood?

A

L –> R Shunt

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What results with the Recirculation of Systemic Venous Blood?

A

R –> L Shunt

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What happens w/ Single-Ventricular Physiology?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Life is not compatible w/ Parallel Blood Flow Circuits w/ Transposition of the Great Vessels (TGA). How is this problem solved?

A

Intercirculatory Mixing provided by communication via a ASD, VSD, PFO, or PDA.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

In regards to Intercirculatory Mixing, what determines the Arterial Saturation?

A

Volumes & Sats of Recirculated Systemic & Effective Systemic Venous Blood reaching the Aorta

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is Fontan Physiology?

A

When Systemic Venous Blood passively flows directly into the Pulmonary Circulation, then returns to a common atrium and single venticle.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Which heart conditions cause Cyanotic Heart Disease w/ Decreased Pulmonary Flow?

A

TOF

Tricuspid Atresia

Univentricular Heart w/ Pulm. Stenosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Which heart conditions cause Cyanotic Heart Disease w/ Increased Pulmonary Flow?

A

Transposition of Great Arteries (TGA)

&

Total Anomalous Pulmonary Venous Return

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Which heart conditions cause Acyanotic Heart Disease d/t a L–>R Shunt Lesion?

A

VSD

ASD

AVSD

PDA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Which heart conditions cause Acyanotic Heart Disease d/t an Obstructive Lesion?

A

Aortic Stenosis

Pulm. Valve Stenosis

Coarctation of Aorta

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the One-Ventricle Lesion heart conditions?

A

Hypoplastic Left Heart Syndrome

Tricuspid Atresia

Double Inlet LV

Unbalance AVSD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are the Two-Ventricle heart conditions?

A

Truncus Arteriosus

TOF w/ Pulm. Atresia

Severe Neonatal Aortic Stenosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are the LV Obstructive Lesion heart conditions?

A

Mitral Stenosis

Aortic Stenosis

Coarctation

Mixing of Systemic & Pulm. Venous Blood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is the PO2 & O2 Sat in the Placenta?

A

PO2: 32-35 mmHg

O2 Sat: 80%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

In Fetal Circulation, what is the PO2 & O2 Sat when the blood in the IVC joins the SVC Drainage?

A

PO2: 12-14 mmHg

O2 Sat: 40%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

In Fetal Circulation what is the PO2 & O2 Sat of the blood traveling from the Ascending Aorta to the Brain, Coronary Arteries & Upper Limbs?

A

PO2: 20-22 mmHg

O2 Sat: 65%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is the PO2 & O2 Sat for the Blood in the Descending Aorta in regards to Fetal Circulation?

A

PO2: 20-22 mmHg

O2 Sat: 55%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Why is the P50 Lower in Fetal Hgb?

A

To help w/ O2 Uptake in the Placenta

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

With Fetal Circulation, How does the RV compare to the LV?

A

Same Size & Thickness

RV has Higher Output @ 1.3 : 1

Pumps 450 mL/kg/min

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What causes the Foramen Ovale to close?

A

When LA pressure is more than RA Pressure causing the FO’s Flap Valve to close against the Septum Secundum

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Functional Closure of the Ductus Arteriosus happens in 10-72 hrs after birth. What else happens at this time?

A

↑Arterial O2 Tension

↓Prostaglandins

Lung Expansion releases Bradykinin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
What happens to Cardiac Output after Birth?
Increases 30-80%
26
What is the Main Purpose of Transitional Circulation?
Separate Systemic & Pulm Circulation Close FO & DA (Reversible for a few days)
27
What can cause the FO to Reopen?
Crying & Pain Hypoxia Hypercarbia Acidosis Lung Disease Sepsis
28
How long does it take for the Anatomic Closure of the FO?
3 months & 1 year when the Septum Primum & Secundum adhese
29
How long does it take for the Anatomic Closure of the DA?
1 - 3 months when the Ligamentum Arteriosum forms
30
What can cause the Ductus Arteriosus to Reopen?
Decrease in O2 Tension
31
What is Alprostadil used for?
Prostalglandin E1 - used to open the DA | (and also to treat ED)
32
What are signs of a PDA?
Hyperactive Precordium Bounding Pulse Wide Pulse Pressure Hepatomegaly Tachypnea Tachycardia
33
What med can be given to close a PDA?
Indomethacin - prostaglandin inhibitor
34
What is the Blood Volume of a Premi?
100 mL/kg
35
What is the Blood Volume for a \< 3 month old?
90 mL/kg
36
What is the Blood Volume for a baby older than 12 months?
Same as Adult 70 mL/kg
37
What is the Blood Volume for a 3-12 month old baby?
80 mL/kg
38
With Fetal & Newborn hearts, how is Cardiac Output increased?
By Increasing HR b/c increasing preload has little effect
39
What is the Normal Respiratory Rate & Heart Rate for an Infant?
RR: 30 - 60 HR: 120 - 160
40
What is the Normal BP for a Premi vs. a Neonate?
**Premi**: 50/25 **Neonate**: 70/40
41
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**
42
Why should Hypoxia & Pressors be avoided in babies?
To prevent R--\>L Shutning & RV Dysfunction
43
What type of Heart Defects are Volume Overload Lesions?
ASD VSD AVSD PDA Truncus Arteriosus L--\>R Shunting
44
With Volume Overload Lesions, where would the Lesions need to be to cause Right Heart Dilatation?
Proximal to Mitral Valve Distal = Left Heart Dilatation
45
How are Volume Overload Lesions treated?
Diuretics Afterload Reduction Surgery
46
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
47
What are signs of ASD?
Mostly Asymptomatic Rarely CHF Easily Fatigued Mild Growth Failure No Cyanosis unless Pulm. HTN
48
How is an ASD fixed?
Surgery/Catheter Closure when kid is 2-5 y.o to avoid late complications
49
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
50
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
51
What causes the L--\>R shunt w/ VSDs?
PVR is less than SVR, NOT Higher LV pressure
52
At what size is it considered a Large VSD?
\> 6 mm & will need repair 50% of Small VSDs close on their own
53
What are symptoms of Large VSDs?
CHF Failure to Thrive Resp. Infections Exercise Intolerance Hyperactive Precordium
54
What is the treatment for VSDs?
**Small VSDs** - No Surgery, just Endocarditis Prophylaxis **Large VSDs** - Start w/ afterload reducers & diuretics, then surgery
55
How does a AVSD develop?
Incomplete Endocardial Cushion Fusion
56
What genetic disease is AVSD common seen in?
Trisomy 21 (Down's)
57
What are signs & symptoms of AVSD?
(Looks like ASD) CHF Pulm. Infections Failure to Thrive Easily Fatigued
58
What is the treatment for AVSD?
Surgery Always Treat CHF Pulmonary Banding
59
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
60
What genetic syndrome is Coarctation seen in?
Turner's Syndrome
61
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
62
What is the treatment for Aorta Coarctation?
Maintain Ductus w/ Prostaglandin E Surgery to prevent LV Dysfunction Angioplasty Balloon Angioplasty for Re-Coarctation
63
What should be given for Pulmonary Blood Flow Obstruction?
Prostaglandin E1 to manage cyanosis
64
What happens w/ Parallel Circulation?
RV ejects into Aorta LV ejects into Pulm. Circulation Mixing occurs at Atrial, Ventricle, or Ductal Levels
65
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
66
What happens w/ Double Inlet LV?
Both Atria empty into LV
67
What Four critieria are needed to be considered a Tetrology of Fallot?
(Most common Cyanotic CHD) VSD Overriding Aorta RVOT Obstruction RV Hypertrophy
68
What happens in a Tet Spell?
Dynamic Narrowing w/ Hypercyanotic Episodes & ↑R--\>L Shunt
69
What can cause a Tet Spell?
Crying Feeding Anesthesia Surgical Stimulation Acidosis
70
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
71
What syndromes are commonly associated with TOF?
DiGeorge & Trisomy 21
72
For a PDA case, what might be given to the patient?
Blood ABx Vitamin K
73
What are the complications associated w/ PDA surgery?
Difficulty Ventilation Desaturation Hemorrhage Accidental Aorta/Pulm. Artery Ligation
74
What monitoring devices should be included along with the standard monitors for a PDA?
Two Pulse Ox - One on Right Hand & Lower Limb
75
During PDA surgery, what might be the cause if the Pulse Ox on the Lower limb is lost?
Clamping of the Aorta
76
What signs would you see at birth for a kid with a Hypoplastic Left Heart?
Tachypnea Tachycardia Cyanosis Systolic Murmur
77
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
78
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
79
What happens during the Norwood Stage 2 Hypoplastic Left Heart Surgery?
Disconnect BT Shunt Make Glenn Shunt by connecting SVC to PA
80
What happens during the Norwood Stage 3 Hypoplastic Left Heart Surgery?
Fontan SVC & IVC connected to PA
81
What the normal Saturation for a BT shunt?
75%
82
What's the normal Saturation for a Glen Shunt?
85%
83
What's the normal Saturation for a Fontan?
95%
84
How is Transposition of the Great Arteries fixed?
Arterial Switch Procedure - arterial trunk relocated to respective ventricles & coronary arteries relocated to aortic root
85
How is a Truncus Arteriosus repaired?
Close the VSD w/ Homograft
86
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
87
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
88
How does the QRS axis appear at birth?
Right Sided QRS, then Left sided by 1 month of age
89
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
90
How would an SVR Decrease **or** PVR Increase affect a R --\> L shunt?
Increases the R --\> L Shunt
91
What is associated with Kawasaki Disease?
Mucocutaneous Lymph Node Syndrome Vasculitis Dilated Coronary Arteries Aneurysm MI Covid-19
92
What are signs and symptoms of Trisomy 21?
Small Size Short Neck Small & Low Ears Macroglossia Mandibular Hypoplasia Narrow Nasopharyx Hypotonia
93
What conditions are commonly associated w/ Trisomy 21?
OSA MR Spine Disorders Thyroid Disease Subglottic Stenosis
94
How does Trisomy 21 affect the CV system?
50% pts have CV Defects ASD VSD TOF PDA Pulm. HTN Bradycardia w/ Anesthesia
95
What are the signs & symptoms of the X-Linked disorder Turner Syndrome?
Webbing Micrognathia Short Stature Lymphedema Ovarian Failure
96
How does Turner Syndrome affect the CV System?
Aortic Coarctation Biscuspid Aortic Valve HTN
97
What conditions are commonly associated w/ Turner Syndrome?
Obesity DM Hypothyroidism Liver Disease