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Congenital Heart Disease
Test 3
126
Nursing
Undergraduate 3
08/04/2014

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Term
Why are congenital heart diseases important in pediatrics?
Definition
Common
Serious
Often treatable
Term
What is the incidence of congenital heart disease?
Definition
One of the most common causes of morbidity & mortality in the NICU
Occurs in ~ 1% of all live births
Nearly half of the cases are diagnosed during first week of life
Term
What are the characteristics of congenital heart disease?
Definition
Failure of the heart to follow the correct sequence of embryologic events
Occurs during the first trimester of fetal development
Resultant abnormal or incomplete structural contributions may lead to minor or major deformities
Term
What is congenital heart disease?
Definition
Congenital heart disease is the failure of the heart to follow the correct sequence of emryologic events during the first trimester of fetal development. Resultant abnormal or incomplete structural contributions may lead to minor congenital cardiac anomalies that can be managed conservatively, or to major deformities that may require immediate medical and/or surgical intervention
Term
What circulatory changes occur at birth?
Definition
Colossal decrease in pulmonary vascular resistance
Pronounced rise in systemic vascular resistance
Decrease in blood flow to the right atrium
Abrupt rise in pulmonary arterial blood flow
Functional closure of foramen ovale & ductus arteriosus
Term
What are the immediate alterations that occur at birth?
Definition
1. A colossal decrease in pulmonary vascular resistance associated with the expansion of the lungs.
2. A pronounced rise in systemic vascular resistance associated with the elimination of the low-resistance placental circulation.
3. A decrease in blood flow to the right atrium because of the abolition of umbilical venous return.
4. An abrupt rise- as much as tenfold- in pulmonary arterial blood flow, which is promptly translated into an increase in left arterial volume and pressure.
5. Functional closure of the valve of the foramen ovale because of the rise in left atrial pressure and the fall in right atrial pressure.
6. Constriction of the ductus arteriosus in response to rises in systemic arterial oxygen tension and declining pulmonary vascular resistance.
Term
What is the gross anatomy of the heart?
Definition
4 chambers, left separated from right
Valves
Outflow tracts
Term
What are the basic principles of heart physiology?
Definition
A chamber that has to pump against higher than normal pressure can fail with time.
If a chamber does not pump efficiently, may cause “backing up” of blood (congestion) in the circulation that feeds that chamber. This also causes strain on chamber that is pumping against the congestion.
Term
What is an example of primary disturbance/blood flow?
Definition
If lesion, for instance, VSD, blood will flow from area with higher pressure (LV) to lower pressure (RV)
Short term, this disturbance causes increased volume delivered to RV (dilation), and increased volume delivered to pulmonary circulation.
Long term, this disturbance causes changes in pulmonary vasculature, increased pulm resistance, RV failure
Term
How does the infant and Child present with CHD?
Definition
Critically ill Neonate with inadequate pulmonary blood flow (PBF) or systemic blood flow (SBF)
Heart Murmur
Arrythmia


PRE NATAL DIAGNOSIS
Term
What are the clinical findings of childhood CHD?
Definition
Poor feeding
Growth failure
Sweating
Tachycardia
Cardiomegaly
Gallop rhythm
Pulsus paradoxus
Pulsus alternans
Term
What are the manifestations of pulmonary edema/congestion?
Definition
Tachypnea
Wheezing
Rales
Dyspnea
Cough
Cyanosis
Term
What are the manifestations of Systemic venous congestion?
Definition
Hepatomegaly
Neck vein distension
Peripheral edema
Term
What is the Evaluation of CHD patients?
Definition
History
Physical examination
ECG
CXR
Blood gases
Hyperoxia test
Echocardiogram
Cardiac catheterization
CT angiogram
MRI
Term
What is a hyperoxia test?
Definition
Done when patient is cyanotic

RA Arterial PaO2 by ABG while patient is breathing room air
RA Arterial PaO2 by ABG after inhaling 100% O2 for 10 minutes
A “failed” hyperoxia test is a neonatal emergency - urgent intervention.
Term
What are the Po2 levels of a hyperoxia test?
Definition
pO2 < 100; cyanotic CHD likely
pO2 100-250; cyanotic CHD possible
pO2 > 250; cyanotic CHD unlikely
Term
What is the Evaluation of CHD patients History?
Definition
Color (ever turned blue)
How does baby feed (tiring, sweating, breathing heavy/fast)
Cough
Weight gain
Exercise tolerance (older children)
Syncope/dizziness
Term
What vitals are taken with Evaluation of CHD patients Physical Exam?
Definition
heart rate, blood pressure, respiratory rate, oxygen saturations, growth curves
Term
What cardiovascular assessment is done with Evaluation of CHD patients Physical Exam?
Definition
color, precordial activity, jugular venous distension, pulses in all extremities, thrill, S1, S2 (split?), S3, S4, capillary refill, edema, murmur (turbulence - systolic/diastolic, intensity, character, location, radiation).
know location of where to best hear valves (aortic, pulmonary, tricuspid, mitral)
Term
What chest assessment is done for eval of CHD?
Definition
effort, retractions, wheezes, rhales, crackles
Term
What abdominal assessment is done with evaluation of CHD?
Definition
liver and speen size
Term
What are the 2 classifications of CHD lesions?
Definition
Acyanotic lesions
Cyanotic lesions
Term
What are the characteristics of Acyanotic lesions?
Definition
Ventricular Septal Defect (VSD)
Atrial Septal Defect (ASD)
Patent Ductus Arteriosus (PDA)
Atrioventricular Septal Defect (AVSD)
Pulmonary Stenosis (PS)
Aortic Stenosis (AS)
Coarctation of the Aorta
Term
What are the characteristics of Cyanotic lesions?
Definition
Truncus Arteriosus
Transposition of the Great Arteries (TGA)
Tricuspid Atresia
Tetralogy of Fallot
Total Anomalous Pulmonary Venous Return (TAPVR)
Term
Which type of CHD lesion is more common?
Definition
Uncommon, each <1% of CHD, pulmonary atresia, Ebstein’s anomaly, truncus arteriosus, single ventricle, double outlet right ventricle

Acyanotic more common than cyanotic
Term
What are the classifications of excessive pulmonary blood flow?
Definition
VSD, ASD, PDA -> ALL HOLES! Letting more blood through because of the pressure
Term
What is Inadequate Pulmonary Blood Flow?
Definition
Tetralogy of Fallot
Term
What is Inadequate or obstruction to systemic Blood flow?
Definition
HLH, Coarctation of Aorta -> Coarctation = STENOSIS! Narrowing of the aorta
Term
What is Abnormal Mixing?
Definition
TGA
Term
What are the characteristics of Ventricular septal defect?
Definition
Membranous
Muscular
15-50% close spontaneously
Murmur
Congestive failure
SBE prophylaxis
Term
What kind of lesion of VSD?
Definition
Acyanotic Lesion with increased pulm blood flow
Most common form of CHD,
20-25% of all CHD

(VSD)
Term
What are the ECG readings of VSD?
Definition
ECG: small VSD nrml,
med VSD  LVH +/- LAE
large VSD BVH +/- LAE
Term
What are the CXR readings of VSD?
Definition
CXR: increased pulm vasculature (depends on LR shunt), possible cardiomegally
Term
What are the exam findings of VSD?
Definition
2-5 / 6 holosystolic murmur loudest at LLSB,
possible thrill at LLSB, P2 may be increased
(large VSD and pulm HTN), hyperdynamic precordium
Term
What is ASD?
Definition
Acyanotic Lesion with increased pulm blood flow (ASD)
Term
What are the exam findings of ASD?
Definition
wide, fixed split S2
2-3 / 6 SEM at LUSB (from increased blood flow across pulm flow tract)
Term
What are the ECG findings of ASD?
Definition
ECG:
small ASD nrml
hemodynamically signif ASD RAD
and mild RVH or RBBB
Term
What are the CXR findings of ASD?
Definition
CXR:
possible cardiomegally and increased pulm vascular markings
Term
What is PDA?
Definition
Acyanotic Lesion with increased pulm blood flow (PDA)
Term
What are exam findings of PDA?
Definition
1-4 continuous “machinery-like” murmur loudest at LUSB
Term
What are the ECG findings of PDA?
Definition
Small to moderate PDA: Normal or LVH
Large PDA: BVH
CXR:
Term
What are the CXR findings with PDA?
Definition
Possible cardiomegaly with increased pulmonary vascular markings
Term
Who is PDA common in?
Definition
More common in premature infants
Term
What is AVSD?
Definition
Acyanotic Lesion with increased pulm blood flow (AVSD)
Term
What are exam findings of AVSD?
Definition
Hyperactive precordium with systolic thrill at LLSB and loud S2
Possible holosystolic regurgitant murmur (MR)
Possible middiastolic rumble
Term
What are ECG findings with AVSD?
Definition
Superior QRS axis
possible RVH and LVH
Term
What are the CXR findings with AVSD?
Definition
Cardiomegaly with increased pulmonary vascular markings
Term
Who does AVSD occur often in?
Definition
30-60% occur in Down Syndrome
Term
What is Pulmonary Stenosis ?
Definition
Acyanotic Lesion
Term
What are the exam findings of Pulmonary Stenosis?
Definition
Ejection click at LUSB with valvular PS
Click intensity decreases with inspiration, increases with expiration
Widely split S2, diminished P2 intensity
Systolic ejection murmur with radiation to back and sides
Term
What are the ECG findings of Pulmonary Stenosis?
Definition
Mild PS: normal
Moderate to severe: RAD and RVH
Term
What are the CXR findings of Pulmonary Stenosis?
Definition
Normal heart size with normal to decreased pulmonary vascular markings
Term
What is Aortic Stenosis ?
Definition
Acyanotic Lesion
Term
What are the exam findings of Aortic Stenosis?
Definition
Systolic thrill at RUSB
Ejection click, not varying with respiration
Harsh SEM (2-4 / 6) RUSB with radiation to neck
Possible early diastolic murmur (AR)
Narrow pulse pressure if severe AS
Term
What are the ECG findings of Aortic Stenosis?
Definition
Mild AS: normal, moderate to severe: LVH
Term
What are the CXR findings of Aortic Stenosis?
Definition
Normal
Term
What are coarctation of the aorta?
Definition
Turners
Isolated
With intracardiac defects
Surgical repair
Balloon angioplasty
Term
What are the exam findings of coarctation of aorta?
Definition
2-3 / 6 SEM at LUSB
If Bicuspid valve, ejection click at RUSB
BP in upper extr > BP in lower extr
Pulse ox difference > 5% between upper extr and lower extr suggests coarc
Term
What is coarctation of the aorta?
Definition
Acyanotic Lesion with Inadequate systemic Blood flow or obstruction
Term
What are the ECG findings of coarctation of the aorta?
Definition
Infancy: RVH or RBBB
Children: LVH
Term
What are the CXR findings of coarctation of the aorta?
Definition
Cariomegaly
Pulmonary venous congestion
Rib notching after age 5 from collateral circulation development
Term
What is the infant presentation of coarctation of the aorta?
Definition
CHF
Term
What is the childhood presentation of coarctation of the aorta?
Definition
HTN/mumur
Term
What are the cyanotic lesions?
Definition
Truncus Arteriosus
Transposition of the Great Arteries (TGA)
Tricuspid Atresia
Tetralogy of Fallot
Total Anomalous Pulmonary Venous Return (TAPVR)
Term
What is transposition of the great arteries?
Definition
Reversal of anatomic relation of great arteries
Ventriculo-arterial discordance
Balloon atrial septostomy
Arterial switch (Jatene)
Mustard/Senning
Term
What is TGA?
Definition
Cyanotic Lesion with abnromal mixing
Term
What are exam findings of TGA?
Definition
Can have extreme cyanosis
Single S2, loud
If associated VSD or PS, murmur
Term
What are ECG findings of TGA?
Definition
RVH because RV acting as systemic ventricle
upright T wave in V1 after 3 days old may be only abnormality
Term
What are CXR findings of TGA?
Definition
Classic finding: “Egg on a string” with cardiomegaly
May have increased pulmonary vascular markings
Term
What is balloon arterial septostomy?
Definition
Creates connection between systemic and pulmonary circulations– TGA, pulm atresia with intact atrial septum
Term
What is Tricuspid Atresia?
Definition
Cyanotic lesion

Lesion: absent tricuspid valve and hypoplastic RV and PA.
Term
What are the exam findings of Tricuspid Atresia?
Definition
Single S2
If VSD, 2-3 /6 systolic regurgitation murmur at LLSB
Term
What must tricuspid atresia have to survive?
Definition
ASD, PDA, or VSD for survival.
Term
What are the ECG findings of Tri. Atresia?
Definition
Right atrial enlargement or common atrial enlargement and LVH
Term
What are the CXR findings of Tri. Atresia?
Definition
Normal or slightly enlarged heart
May have “boot shaped” heart
Term
What is Tetralogy of Fallot ?
Definition
Ventricular septal defect
Overriding aorta
Subpulmonary stenosis
Right ventricular hypertrophy


Cyanotic Lesion with inadequate pulmonary blood flow
Term
How is Tetralogy of Fallot corrected?
Definition
Surgical REpair
Term
What are common findings with Tetralogy of Fallot?
Definition
Large VSD
RVOT obstruction
RVH
Overriding aorta
Term
What does the degree of RVOT obstruction determine?
Definition
amount of shunting across VSD, pulm flow, and cyanosis.
Term
What are the manifestations of MILD ps?
Definition
L to R shunting, no cyanosis
Term
What are the manifestations of SEVERE ps?
Definition
R to L shunting, cyanosis
Term
What are common exam findings of Tetralogy of Fallot ?
Definition
loud systolic murmur along LSB, loud single S2, possible thrill.
Term
What are common ECG findings of Tetralogy of Fallot ?
Definition
RAD and RVH
Term
What are the CXR findings of Tetralogy of Fallot?
Definition
boot shaped heart”
normal size
+/- increased pulmonary vascular markings depending on pulm blood flow (degree of RVOT)
Term
What are TET spells that are associated with Tetralogy of Fallot?
Definition
Occur in young infants
Increased RVOT obstruction or decreased systemic resistance leads to R  L shunting across VSD, resulting in decreased pulm blood flow
Term
What are the manifestations of TET spells?
Definition
Cyanosis, decreasing murmur
Term
How are TET spells treated?
Definition
calming child, oxygen, knee chest position, morphine. All aimed at increasing systemic resistanc, decreasing pulmonary resistance to promote pulmonary bood flow
Term
What is Total Anomalous Pulmonary Venous Return (TAPVR) ?
Definition
Cyanotic Lesion

Pulmonary veins drain into RA or other location instead of LA. Must have ASD or PFO for survival.
Term
What is Supracardiac Total Anomalous Pulmonary Venous Return (TAPVR)?
Definition
PV into SVC
Term
What is Cardiac Total Anomalous Pulmonary Venous Return (TAPVR)?
Definition
: PV into coronary sinus or RA
Term
What is sub diaphragmatic Total Anomalous Pulmonary Venous Return (TAPVR)?
Definition
PV into IVC, portal vein, ductus venosus or hepatic vein
Term
What are exam findings of Total Anomalous Pulmonary Venous Return (TAPVR) ?
Definition
Hyperdyanmic RV impulse
S2 fixed and widely split
2-3 / 6 SEM at LUSB and middiastolic rumble at LLSB
Term
What are ECG findings of Total Anomalous Pulmonary Venous Return (TAPVR) ?
Definition
RAD, RVH
Term
What are CXR findings of Total Anomalous Pulmonary Venous Return (TAPVR) ?
Definition
Cardiomegaly
Increased pulmonary vascular markings
Term
What is the most common chromosomal defect?
Definition
Trisomy 21

Most frequent chromosomal defect affecting live-born infants (1 in 660 live births)
Term
What are the most common congenital Heart Malformations associated with Trisomy 21?
Definition
Atrio-ventricular septal defect
Ventricular septal defect
Atrial septal defect
Patent ductus arteriosus
Term
What are the cardiac effects of FAS?
Definition
Half of patients with FAS have congenital heart disease: VSD, ASD, TOF.
Term
What are the cardiac effects of Turner Syndrome?
Definition
Coarctation of aorta, bicuspid aortic valve, aortic stenosis
Term
What is the dominant cardiac defect in downs?
Definition
AVSD, VSD, ASD
Term
What are the dominant defects in noonan syndrome?
Definition
PS
Term
What is the dominant cardiac defect of Turner Syndrome?
Definition
Coarctation
Term
What is the dominant cardiac defect of Marfan syndrome?
Definition
Aortic Root Dilatation
Term
What is the dominant cardiac defect of William's syndrome?
Definition
Supravalvular AS
Term
What is the dominant cardiac defect of FAS?
Definition
VSD, ASD
Term
What is the dominant cardiac defect of DiGeorge syndrome?
Definition
Interrupted aortic arch, TOF
Term
What is the dominant cardiac defect of CHARGE syndrome?
Definition
AV canal, VSD, ASD
Term
If you are severely compromised with sickle cell trait, what can happen?
Definition
Your trait can turn into sickle cell anemia!
Term
Name five Cyanotic Congenital Heart Malformations that start with the letter “T” ?
Definition
Tetralogy of Fallot
Transposition of the Great arteries
Tricuspid atresia
TAPVR
Truncus arteriosus
Term
Name two congenital Heart malformations in the newborn that are dependent on their patent ductus for survival?
Definition

Tetraolgy of the Heart

Transposition of the Great Arteries 

Term
How is patency of the PDA maintained?
Definition
Prostaglandin Prostaglandins are utilized to maintain the patency of the ductus arteriosus until surgical ligation is performed. When surgical ligation is not indicated, prostaglandin inhibitors (eg, nonsteroid antiinflammatory drugs [NSAIDs]) are used to close the ductus arteriosus.
Term
What was the first interventional Catheterization technique done in infants and children?
Definition
Balloon atrial septostomy
Term
Which side during utero does NOT receive a lot of blood?
Definition
RIGHT because it's pulmonary!
They are not using their lungs!
Term
[image]
Definition
Atrial Septal Defect
Term
[image]
Definition
Patent Ductus Arteriosis
Term
[image]
Definition
Pulmonary Stenosis
Term
[image]
Definition
Aortic Stenosis
Acyanotic Lesion
Term
[image]
Definition
Coarctation of Aorta
Term
[image]
Definition
Tricuspid Atresia
Cyanotic Lesion
Term
[image]
Definition
Tetralogy of Fallot

Ventricular septal defect
Overriding aorta
Subpulmonary stenosis
Right ventricular hypertrophy
Term
[image]
Definition
Total Anomalous Pulmonary Venous Return (TAPVR) Cyanotic Lesion
Term
C. A. is a 10 day old male who presents to the emergency department with a 2 day history of poor feeding and lethargy. The child is pale, heart rate is 102 beats per minute and his blood pressure in the left upper extremity is 100/48. His cry is weak

How do you evaluate this patient?
Definition
Take vitals
Temp
Listen
Term
What do you think his cardiac condition is?
Definition
Cyanosis?
HPT?
Murmur?
Tachycardia?

Ductus closed after 10 days
Term
What is your differential diagnosis?
Definition
Blood Gas
O2 Sat Monitor
Term
How do you initiate therapy?
Definition
Oxygen
Term
What is surgical/ interventional option?
Definition
Balloon Arterial Sepsostomy
Term
What is the long term prognosis?
Definition
Good
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