Pediatric Cardiology Flashcards
1
Q
What congenital heart defect does PDA stand for?
A
Patent Ductus Arterious
2
Q
What congenital heart defect does ASD stand for?
A
Atrial Septal Defect
3
Q
What congenital heart defect does VSD stand for?
A
Ventricular Septal Defect
4
Q
What congenital heart defect does TET stand for?
A
Tetrology of Fallot
5
Q
What congenital heart defect does Coarc stand for?
A
Coarctation of the Aorta
6
Q
A ____ heart defect is defined as a defect that occurs as a result of abnormal development of the heart and related structures during development
A
Congenital
7
Q
Congenital heart defects affect between __-__ babies per 1000 live births
A
4-10
8
Q
Congenital heart defects are the leading cause of ___ (excluding prematurity) during the first year of life
A
Death
9
Q
During the first year of life, ___% of infants with heart defects require treatment or diet within the first year
A
35
10
Q
The cause of the heart defects is only known in about ___% of cases
A
10
11
Q
In the United States, there seems to be an ____ in cases of congenital heart defects; the cause is not known but it is thought that we just have better detection methods today
A
Increase
12
Q
The risk for development of congenital heart disease increases when there is an increase in the number of ___ ___ relatives affected
A
First degree
13
Q
What heart defects can be caused by trisomy 13?
A
-VSD
-PDA
-ASD
14
Q
What heart defects can be caused by trisomy 21?
A
-Endocardial cushion defect
-VSD
-PDA
-ASD
-TET
-Transportation of great vessels
-Coarctation of the aorta
15
Q
What types of heart defects might be caused by Turner’s Syndrome?
A
-Coarctation of the aorta
-PDA
-Pulmonary stenosis
-Septal defects
16
Q
What types of heart defects might be caused by Cru du chat Syndrome?
A
-PDA
-Mixed defects
17
Q
About ___% of congenital heart defects are thought to be caused by a combination of genetic and environmental factors
A
90
18
Q
Because of the improved treatment methods, many children are growing up and having children; the risk of a child having a congenital defect is ___-___%
A
5-15%
19
Q
If two siblings have cardiac anomalies, the risk is about ___% for the next child, but if three children are affected, the risk jumps to ____%
A
9; 50
20
Q
Intrauterine infections, along with viral infections and Rubella, may cause what types of heart defects?
A
-PDA
-Pulmonary stenosis
-Coarctation of aorta
21
Q
___ ____ can infect endothelial cells, but the specific cardiac effects are unknown
A
Herpesvirus cytomegalovirus
22
Q
Diabetes might cause what types of heart defects?
A
-VSD
-Cardiomegaly
-Transposition of great vessels
23
Q
What types of heart defects might be caused by PKU?
A
-PDA
-Coarctation of the aorta
24
Q
Hypercalcemia may cause what types of heart defects?
A
-Supravalvular aortic stenosis
-Pulmonary stenosis
25
Alcohol abuse may cause what heart defects?
-TET
-ASD
-VDS
26
Warfarin use may cause what heart defects?
-ASD
-PDA
27
Increased maternal age increased risk for what cardiac defects?
-VSD
-TET
28
Prematurity increases risk of what heart defects?
-PDA
-VSD
29
Living at a high altitude increase risk for what heart defects?
-PDA
-ASD
30
Approximately the ___-___ day of fetal life, two lateral endothelial heart tubes fuse to form a single endocardial tube
21st-22nd
31
By the ___ day, the tube thickens and the first fetal heart contractions occur
28th
32
The tube them coils to the right and by the end of the ___ week, the tube is completely coiled and the major chambers are identified
4th
33
By the 22/23rd day, what structures can be identified in the heart?
-1st aortic arch
-Bulbus cordis
-Ventricle
-Atrium
34
By the 23/24th day, what structures can be identified in the heart?
-Truncus arteriosus
-Bulbus cordis
-Ventricle
-Atrium
-Sinus venosus
35
By the 24/25th day, what structures can be identified in the heart?
-1st and 2nd aortic arches
-Truncus arteriosus
-Ventricle
-Premature left atrium
36
The cranial end of this primitive heart is known as the ___ ___ which will further divide into the great vessels
Truncus arteriosus
37
The caudal end of the primitive heart will become the...
Superior and inferior cava
38
____ is also occuring at this time which creates the beginning of the heart valves
Septation
39
The heart is completed by __ __ after conception, therefor some congenital defects are formed before the pregnancy is recognized
6 weeks
40
Fetal ____ is anatomically and physiologically different from postnatal
Circulation
41
Oxygenation occurs in the ____ and not in the lungs
Placenta
42
Fetal lungs are filled with fluid and have low ___ ___ tension which produces pulmonary vasoconstriction
Arterial O2
43
There is ___ resistance to pump blood into the fetal lungs, but we don't want much in there anyway
High
44
Because of the pulmonary constriction, only ___% of the fetal cardiac output is received by the pulmonary circulation
8
45
Fetal systemic vascular resistance is low, and nearly half of the blood flow enters the ____ which provides low resistance to flow
Placenta
46
The fetus is ____ with an O2 saturation of approximately 60-70%; however the fetus is not hypoxic because of the extremely high cardiac output
Hypoxemic
47
The cardiac output of a fetus averages about ___-__ ml/kg/min
400-500
48
The best oxygenated blood from the placenta travels to the fetal ___
Brain
49
The fetal blood is oxygenated in the placenta, which is a less efficient oxygenator than the ___
Lungs
50
The oxygenated blood enters the fetus through the ___ ___ to the liver
Umbilical vein
51
About 50% of oxygenated blood enters the ___ ___, bypassing the hepatic circulation and flowing into the inferior vena cava
Ductus venosis
52
When this blood reaches the right atrium, it is diverted by the ___ ___ toward the atrial septum, and flows through the foramen ovale into the left atrium
Crista dividens
53
The blood then passes through the left ventricle and ___ ___ to perfuse the head and upper extremities
Ascending aorta
54
This pathway allows the best-oxygenated blood from the placenta to perfuse the ___ ___
Fetal brain
55
Venous blood from the heat and upper extremities results to the fetal heart through the ___ ___ ___, enters the right atrium and ventricle, and flows into the pulmonary artery
Superior vena cava
56
Since pulmonary vascular resistance is high, this blood is diverted through the ductus arteriosus into the ___ ____
Descending aorta
57
Ultimately, much of this blood will return to the ____ through the umbilical cord to be reoxygenated
Placenta
58
Within the first few breaths, the lungs are filled with air and it becomes a more efficient ____
Oxygenator
59
With this in increase in O2 and decrease in CO2, the ___ ___ ___ starts to drop
Pulmonary vascular resistance
60
As the peripheral vascular resistance starts to decrease, right atrium and right ventricle pressures ____
Decrease
61
Systemic vascular resistance increases due to the elimination of the placenta, therefore increasing ___ ___ and ___ ___ pressure
Left atrium, left ventricle
62
This change in pressure gradient functionally closes the ___ ___
Foramen ovale
63
Anatomic closure, due to ___ deposits, is completed within the first few months of life
Fibrin
64
The change in O2 and CO2 levels is caused by constriction of the ___ ___
Ductus arteriosus
65
Another factor in constriction of the ductus arteriosus is the sudden drop in ____
PGE1
66
The ductus is usually functionally closed by ___ to ___ hours after birth with anatomical closure within ___-___ days of life
15-18 hours; 10-21 days
67
If a defect is present, the duct can stay open or be reopened with medications like _____
Prostaglandin
68
Due to the vasoconstriction of the umbilical arteries and the elimination of blood flow through the ___ ___, by 7 days post-natal, there is no flow through the duct
Ductus venosus
69
The ductus venosus because then becomes the ___ ___
Ligamentum venosum
70
There is marked change in ___ ___ after birth
Pulmonary resistance
71
The change in pulmonary resistance in a neonate is ___ and related to the infant's condition
Variable
72
Problems with increased peripheral vascular resistance that occur are related to factors that cause constriction of the ___ ___
Pulmonary bed
73
Constriction of the pulmonary bed can result in ___ ___ (2/1000 live births- children may be very sick)
Pulmonary hypertension
74
With a ____ lesion, there is usually an obstruction to pulmonary blood flow
Cyanotic
75
With a cyanotic lesion, blood shunts from ___ to ___, decreasing blood flow to the pulmonary circulation
Right to left
76
Cyanotic lesions cause a mixing of ____ and ___ blood
Oxygenated and deoxygenated
77
With an ____ lesion, blood usually shunts from left to right
Acyanotic
78
With acyanotic lesions, blood moves from ____ pressure to ___ pressure
High to low
79
Usually, acyanotic lesions cause ___ ___ of pulmonary blood flow
Over circulation
80
____ lesions are a stenotic area with restrictive blood flow
Obstructive
81
With obstructive lesions, there is hypertension ___ to obstruction
Proximally
82
With obstructive lesions, there is hypoperfusion ____ from obstruction
Distally
83
A patent ductus arteriosus (PDA) is caused by...
Failure of ductus arterious to close
84
With PDA, blood shunts normally from ___ to ___
Left to right
85
Is a PDA a cyanotic or acyanotic defect?
Acyanotic
86
A PDA can be part of a ____ defect
Complex
87
A PDA can be ___ ___ is more complex defects
Medically necessary
88
With a PDA, there is increased blood flow to the ____, resulting in increased workload on the left side of the heart
Lungs
89
Clinical manifestations of PDA:
-Continuous machinery-type murmur heart beat at the upper left sternal border through systole and diastole (aortic area)
-If the lesion is significant, infants will also have bounding pulses, and active precordium (you can see their heart beating), a thrill upon palpation, and signs and symptoms of CHF from the pulmonary overcirculation
90
A small PDA is usually ____
Asymptomatic
91
Atrial septal defects (ASD) are caused by...
Improper formation of the septal wall
92
Is an ASD cyanotic or acyanotic?
Acyanotic
93
An ASD causes a failure of the ___ ___ to close
Foramen Ovale
94
ASD is the ___ most common congenital heart defect
4th
95
___ ___ ___ is an opening found low in the septum that may be associated with AV valve abnormality
Ostium primum defect
96
___ ___ ___ is an opening in the center of the septum and is the most common
Ostium secundum defect
97
___ ___ ___ is an opening high up in the atrial septum near the superior vena cava and right atrium junction
Sinus venosus defect
98
With ASD, blood is shunted ___ to ___ because of the higher pressure of the left atrial chamber
Left to right
99
ASD might cause right atrium and right ventricle ___
Enlargement
100
Children with ASD are usually ____
Asymptomatic
101
With an ASD, you will hear a ____-____ systolic ejection murmur due to increase blood flow the pulmonary valves
Crescendo-decrescendo
102
Erb's point is...
2nd-3rd intercostal space, left sternal border
103
With an ASD, you might also hear a wide fixed splitting of the ____ heart sound due to volume overload in the right ventricle
Second
104
Volume overload causes a prolonged ___ ___ and delay of pulmonic valve closure resulting in the splitting sound
Ejection time
105
Small ASD might go undetected until adulthood when ____-like symptoms appear
Stroke
106
A ventricular septal defect (VDS) is a defect in the ___ ___
Septal wall
107
Is a VSD cyanotic or acyanotic?
Acyanotic
108
Those with VSD are usually asymptomatic until there is a fall in ___ ___ ___ at 2-4 weeks of age
Peripheral vascular resistance
109
A VSD is the most common congenital heart defect, accounting for approximately ___-___% of all defects
25-33%
110
With VSD, blood is shunted from ___ to ___
Left to right
111
The amount of shunting with VSD is dependent on the size of the defect as well as the degree of ___ ___
Pulmonary resistance
112
With VSD, blood is shunted on ____, so blood goes right out the pulmonary artery into pulmonary circulation
Systole
113
With VSD, you will see ____ of the main pulmonary artery, left atrium, and left ventricle
Enlargement
114
With VSD, ___ ___ hypertrophy occurs to effectively pump the additional volume
Left ventricle
115
With VSD, eventually the heart can't handle the increased volume and ___ ___ ____ develops
Congestive heart failure
116
Clinical manifestations in children with VSDs depend on...
-Age of the child
-Size of the defect
-Level of pulmonary resistance
117
Newborns with VSD are relatively ___
Asymptomatic
118
The high ___ ___ ___ equalizes pressure in the ventricles
Peripheral vascular resistance
119
Once peripheral vascular resistance drops, left-right shunting occurs resulting in...
Murmur (load, holosystolic left sternal border
120
Large VSDs lead to symptoms like...
-Symptoms of congestive heart failure
-Poor weight gain
-Loud, harsh, holosystolic murmur
-Systolic thrill at left lower sternal border
121
Adults with unrepaired VSD may develop ___ ___ ___ ___ which may cause cyanosis and clubbing
Pulmonary vascular obstructive disease
122
Tetralogy of fallot (TET) is the most common ____ defect
Cyanotic
123
TET is what four defects in one?
-VSD
-Pulmonary stenosis
-Right ventricular hypertrophy
-Overriding aorta
124
TET occurs __-__% of the time
9-14%
125
Pathophysiology of TET varies widely based on...
-Degree of pulmonary stenosis
-Size of the VSD
-Pulmonary and systemic resistance to flow
126
If pulmonary resistance is high with TET, you will see a ___ to ___ shunt
Right to left
127
If pulmonary stenosis is minor and pressure is lower, you will see a ___ to ___ shunt
Left to right
128
Pulmonary stenosis decreases the amount of blood flow to the ____
Lungs
129
Pulmonary stenosis causes an increase in pressure which results in a __ to ___ shunt
Right to left
130
People with pulmonary stenosis have a mixing of deoxygenated blood with a decreased amount of oxygenated blood from the lungs; the body compensates by increased the amount of ___ ___ ___ (polycethmia)
Red blood cells
131
Clinical manifestations of tetralogy of fallot:
-While the PDA is open, the newborn has adequate pulmonary blood flow
-Once the duct closes, cyanosis occurs
-Over time, you may see clubbing of fingers and toes
132
A tet spell might cause...
Sudden onset of dyspnea, cyanosis, and restlessness which occurs with crying or exertion
133
One theory about tet spells is that right ventricle outflow tract goes into spasm, increasing pulmonary resistance and __-__ shunting
Right-left
134
A compensatory mechanism for older children with tet spell is to ___, which increases systemic vascular resistance while decreasing venous return to the heart
Squat
135
The decrease in venous return that happens when squatting allows for more ___ ___ to be available and the increase in systemic vascular resistance helps reverse the shunt to more left-right
Oxygenated blood
136
Coarctation of the aorta (Coarc) is severe ____ of the arch of the aorta
Narrowing
137
The ____ and ____ of the coarc predict presentation
Location and narrowing
138
Coarc commonly occurs because of an abnormal ___ ___ ___ that constricts at the time of ductal closure
Contractile ductal tissue
139
Coarc causes ___ pressures above the site of stenosis and ____ pressures below the site
Higher; lower
140
You may see ___ ___ ___ is the coarc is pre-ductal
Congestive heart failure
141
The high pressure above the coarc are seen in the ___ ___ served by the arteries where the pressure is higher
Systemic circulation
142
Clinical manifestations of coarc in newborns:
-Usually present with signs of congestive heart failure
-Once the ductus closes, infants deteriorate rapidly with the development of hypotension, acidosis, and shock
143
Older children with coarc might not be diagnosed until ____ is noted
Hypertension
144
In older children with coarc, you would see ____ in upper extremities and ____ in lower extremities
Hypertension; hypotension
145
If pulses are affected by the coarc, we will see...
-Mottled skin
-Leg cramps during exercise from anorexia
146
Hypertension may cause...
-Dizziness
-Headache
-Fainting
-Epistaxis
147
___ ___ is a narrowing of the right ventricle outflow tract
Pulmonary stenosis
148
There is a decrease in ____ blood flow with pulmonary stenosis
Pulmonary
149
Pulmonary stenosis can be...
-Supravalvular
-Subvalvular
-Valvular
150
___ ___ ___ is a result of the stenosis
Right ventricular hypertrophy
151
If the backup into the right atrium is severe with pulmonary stenosis, you may see reopening of the __ __, causing ASD
Foramen ovale
152
Clinical manifestations of pulmonary stenosis depends on ___
Severity
153
Systolic ejection murmur at the __ ___ ___ ___ reflects obstruction to flow
Left upper sternal border
154
With pulmonary stenosis, you might palpate a ___
Thrill
155
If child has moderate stenosis, they may have ___ ___ because of insufficient pulmonary blood flow
Exertional dyspnea
156
Severe pulmonary stenosis results in...
-Cyanosis
-Right sided failure
157
Is a transposition of the great arteries cyanotic or acyanotic?
Cyanotic
158
With a transposition of the great arteries, the ___ and ___ ___ are switched
Aorta and pulmonary artery
159
Transposition of the great arteries is called a ___ defect because of the dependence upon mixing of the pulmonary and systemic circulation for survival
Mixing
160
Patients with transposition of the great arteries must have one of these things to survive:
-Patent ductus arteriosus
-ASD
-VSD
161
With transposition of the great arteries, there is mild cyanosis shortly after birth which worsens during the first day when the ___ ____ closes
Ductus arteriosus
162
When the ductus arteriosus closes, it causes hypoxemia which causes symptoms like...
-Metabolic acidosis
-Tachycardia
-Tachypnea
163
The presence of a ___ or ___ ___ allows for more mixing with transposition of the great arteries
PDA or septal defect
164
If someone with transposition of the great arteries has an intact septum, you will not hear a ____
Murmur
165
Fixing a transposition of the great arteries is a tricky procedure because the __ __ must be moved along with the ___
Coronary arteries, aorta
166
___ ___ is also known as mucocutaneous lymph node syndrome
Kawasaki Disease
167
Kawasaki disease is acute self-limiting ____ that may result in cardiac complications
Vasculitis
168
Are males or females more affected by Kawasaki disease?
Males (slightly)
169
Peak incidence of Kawasaki disease is in what seasons?
-Winter
-Spring
170
Kawasaki disease is a primary disease of ___ ___
Young children
171
___% of cases of Kawasaki disease are in children less than 5 years of age
80%
172
The first reported cause of Kawasaki was reported in ___ by Dr. Thomisakyu Kawasaki
1967
173
Kawasaki occurs throughout the world but the greatest number of cases are reported in ____
Japan
174
What U.S state has the highest incidence of Kawasaki?
Hawaii
175
Kawasaki disease occurs in ___ stages
4
176
What happens in stage I of Kawasaki disease (0-12 days)?
Small capillaries, arterioles, venules, and the heart become inflamed
177
What happens in stage 2 of Kawasaki disease (12-25 days)?
Inflammation spreads to larger vessels
178
What happens in stage 3 of Kawasaki disease (26-40 days)?
Coronary arteries begin to thicken and inflammation resolves
179
What happens in stage 4 of Kawasaki disease (40 days and beyond)?
Vessels develop scarring, calcification, and stenosis of coronaries
180
Symptoms of the acute phase of Kawasaki:
-Fever (most common)
-Conjuntivitis (begins within days of fever onset)
-Strawberry tongue
-Rash
-Lymphadenopathy (occurs in 50-70%)
-Irritability
181
Symptoms of the subacute phase of Kawasaki:
-Fever ends
-Clinical signs resolve
-Desquamation of palms and soles
-Thrombocytosis (high platelet count)
182
During the subacute phase, the child is at the highest risk for developing ___ ___
Coronary aneurysm
183
Symptoms of the convalescent phase of Kawasaki:
-Elevation of erythrocyte sedimentation rate and platelet count
-Possible arthritis
184
The convalescent phase lasts until all ____ return to normal
Labs
185
Eisenmenger's Syndrome is a defect of the ___ ___ (ASD or VSD) with severe pulmonary hypertension, hypertrophy of the right ventricle, and latent or overt cyanosis
Interventricular septum
186
Because of the VSD with Eisenmenger's, there is an ___ in blood flow to the lungs and an ___ in blood pressure in the lungs
Increase; increase
187
When the high blood pressure becomes an irreversible problems on its own, the direction of blood through the ___ in the heart is reversed
Hole
188
The reversal of the direction of shunting ____ pressure resulting in a narrowing of the arteries
Increases
189
Over time, these changes are irreversible due to ___ of the arteries
Inflammation
190
Much of the lung arteries become ___, leading to increase in pulmonary blood vessel resistance
Occluded
191
Once the resistance is so high, the shunt reverses to ___ to ___
Right to left
192
Symptoms of Eisenmenger's syndrome:
-Cyanosis
-Clubbing
-Dyspnea and SOB
-Hemoptysis
-Activity intolerance
-Palpitations
-Syncope
-Erythrocytosis
-Sudden death
-Symptoms of pulmonary hypertension
-Symptoms of heart failure
