Cardiology Flashcards
1
Q
From what embryologic structure does the heart form?
A
Mesoderm
First system to function in utero
2
Q
What embryologic structures form the tube of the heart structure and when?
A
Two sheets of mesodermal angiogenic cells (Day 15) –>
Upper sheet enlarges to encircle other sheet (Day 17)–>
Beating initiated in upper tube (Day 20)
3
Q
How and when does the heart tube structure form ventricles?
A
Tube bends to the right (D-loop) (Day 21)–>
Chambers form (Day 22)–>
Ventricles migrate to side-by-side (Day 28)
4
Q
How and when does cardiac septation occur?
A
Atrial septum: grows within atria and forms 2 septum (Day 34)
Ventricular septum: Cells near inferior single ventricle grow upward to form septum (Day 38-46)
5
Q
When is cardiac structure complete?
A
7-8 weeks
6
Q
Describe the position of the atria relative to the viscera
A
1st letter:
S- solitus
I - inversus
A- ambiguous
7
Q
Describe the position of the ventricle:
A
2nd letter:
D-loop: right ventricle on right side
L-loop: mirror image
8
Q
Describe the position of the great arteries:
A
3rd letter:
S- solitus (aorta to right and posterior of pulmonary artery)
I- inversus/mirror image
9
Q
What are the cardiac designations for:
- Normal
- TGA (Right and left)
- Situs inversus totalis
A
Normal: SDS
D-TGA: SDD (right)
L-TGA: SLL (left)
Situs inversus: ILI
10
Q
When do cardiac defects occur during embryogenesis?
A
Most by 8 weeks
11
Q
Cardiac morphogenesis- when does it occur and why?
A
Can occur progressively throughout pregnancy
Occurs due to acquired conditions or decreased blood flow
12
Q
Where is most blood volume in the fetal heart?
A
Right ventricle
13
Q
Where is the smallest blood volume in the fetal heart?
A
Right atrium
14
Q
What percentage of fetal blood volume shunts through the PDA?
A
60%
15
Q
What percentage of fetal blood flow goes to the lungs?
A
10%
16
Q
What percentage of fetal blood volume is each of the 4 heart chambers?
A
Right atrium (20-25%)–>
Left atrium (27%)–>
Left ventricle (34%)–>
Right ventricle (65-70%)
17
Q
Blood from the upper body drains to the ____ ventricle which then supplies the _____ body
A
Right
Lower
18
Q
Blood from the lower body and placenta drain to the_____ then 1/3 cross the PFO to ___________ and 2/3 supplies the upper body
A
IVC
Cerebral and coronary arteries
19
Q
The ______ ventricle supplies the majority of cardiac output in the fetus
A
Right
20
Q
The highest oxygen content in the fetus is in the __________
A
Umbilical veins (70%)
21
Q
Cardiac output equals
A
Systemic blood pressure/
Total peripheral vascular resistance
OR
HR X Stroke volume
22
Q
Heart rate or stroke volume have a bigger impact on cardiac output?
A
Heart rate
23
Q
What 3 things keep the ductus arteriosus open in utero?
A
Prostaglandin 2
Prostacyclin
Thromboxane A2
24
Q
What medication maintains an open doctor’s arteriosus?
A
Prostaglandin 1
25
The lowest oxygenation content in the fetus is in the
Umbilical artery
26
Fetal hemoglobin contributes to lower pO2 tolerance by
Higher oxygen affinity
Low p50
Left shift in oxyhemoglobin curve (easier to release O2)
Increased hemoglobin levels for increased O2 carrying capacity
Anaerobic metabolism
27
Calculation of cardiac output:
HR X SV
OR
SBP/TPVR
28
Right ventricle stroke work is roughly equal to _____ left ventricle stroke work
1/6
29
An increased cardiac contraction resulting from increased preload/stretch is described by the
Frank Starling principle
30
Cardiac contractility is increased by
Catecholamines
| Thyroid hormone
31
Pulmonary: systemic shunt calculation:
sO2 (ao) - sO2 (SVC)/
| sO2 (LA or Pulm v) - sO2 (PA)
32
Pulmonary vascular resistance calculation:
P PA- P LA/
| P blood flow
33
Systemic vascular resistance calculation
P Ao - P RA/
| Sys blood flow
34
Cyanosis is visible if hemoglobin decreases by
3 to 5 g reduced Hgb
35
What causes differential cyanosis and what is it?
Critical coarctation with PDA and increased PVR
| Right to shunting causing cyanosis in the lower body more than the upper body
36
What is reverse differential cyanosis?
```
Upper body more cyanotic than lower body
Due to TGA with intact septum, associated with pulmonary hypertension,
interrupted aortic arch,
or coarctation of the aorta,
and PDA
```
37
PaO2s associated with critical preductal coarctation of the aorta with PDA and increased PVR are
Right radial paO2 = 250. ^^^^^
Umbilical artery paO2 = 45. vvvvv
38
PaO2s associated with TGA with intact ventricular septum, PDA and (PHTN, interrupted aortic arch, or preductal CoA) are
Right radial paO2 = 50
Umbilical artery paO2 = 250
39
PaO2s associated with infradiaphragmatic TAPVR are
Umbilical artery paO2 = 90
Umbilical venous paO2 = 250
40
S1 heart sound represent
Closure of mitral and tricuspid valves
41
A widely split s1 can represent
Right bundle branch block
| Ebstein's anomaly
42
S2 represents
Closure of the aortic valve in pulmonary valve
43
A widely split S2 can represent
ASD
| PAPVR
44
A single S2 can represent
Pulmonary hypertension
45
VSD murmur is characterized by
Holostystolic murmur beginning with S1 and continuing to S2
46
A crescendo ejection murmur can represent
Stenotic aortic or pulmonic valves
47
Valvular regurgitation sounds like a
Blowing murmur
48
Diastolic murmurs are _____ and can represent ________
Always pathologic
Aortic regurgitation
Pulmonic regurgitation
Tricuspid or mitral stenosis
49
Continuous murmurs occur with
```
PDA
AV fistula
Venous hum
Collateral vessels
Truncus arteriosus
Aortopulmonary window
```
50
Narrow pulse pressure occurs with
Pericardial tamponade
Aortic stenosis
Intravascular depletion
51
Wide pulse pressure occurs with
```
PDA
Thyrotoxicosis
AV fistula
Aortic regurgitation
Truncus arteriosus
```
52
Mean blood pressure is calculated as
Diastolic blood pressure + 1/3 ( systolic blood pressure - diastolic blood pressure)
53
Tachycardia in early compensated shock is due to
Catecholamine release
54
In neonates the most common type of shock is
Hypovolemic shock
55
Kerley B lines indicate
Congestive heart failure, linear densities in lungs due to interstitial edema
56
Recurrence of congenital heart disease in a subsequent sibling is
2-5%
57
Risk of congenital heart disease in a child to a mother with Congenital heart disease is
If the father had CHD
~7%
~2%
58
Most inheritable type of congenital heart defects are
Left-sided obstructive lesions
59
The most common CHD is ______ at ____ percentage
VSD
| 16%
60
Most common cyanotic heart disease presenting in the first week of life is _____ at the _____ percentages
TGA
| 5-10%
61
Cyanotic heart disease most likely to present in the first week of life and cause mortality is
HLHS
| 2%
62
Most common cyanotic heart disease beyond infancy is
TOF
| 8-10%
63
Name the cyanotic heart disease listed in the 5T/DO/ESP
```
Truncus arteriosus
Transposition of the great arteries
Tricuspid atresia
Tetralogy of Fallot
TAPVR
DORV
Ebstein's anomaly
Single ventricle
Pulmonary atresia
```
64
CHF related to congenital heart defects tend to be ______ type of defects
Obstructive
```
HLHS with restrictive atrial defect
Severe TR or PR
Large systemic aortovenous fistula
Obstructed TAPVR
TGA
Ebstein's anomaly
Critical AS or PS
Preductal CoA
```
65
TGA is more common in
Males
66
About 50% of TGA will also be associated with a
VSD
67
The more common form of TGA has the aortic valve positioned
Interior, inferior, and to the right of the pulmonary valve
68
LTGA is also known as
Congenitally corrected TGA
Aortic valve is anterior and left of the pulmonary valve
69
Which type of TGA is most likely to have severe cyanosis at birth?
D-TGA
70
Which congenital heart defect has the appearance of egg on a string on x-ray and no murmur?
TGA
71
EKG in TGA tends to show
Right QRS axis
Right ventricular hypertrophy
Right atrial hypertrophy
72
Immediate management of TGA includes
PGE1
Rashkind (balloon septostomy)
Treat CHF
Arterial switch (Jatene) and VSD/PS repairs at older age
73
25% of tetralogy of fallot have
Right aortic arch
74
Four abnormalities associated with tetralogy of fallot are
VSD
RVOT
RVH
OAo
75
What distinguishes a pink tet from a blue tet?
Severity of right ventricular outflow tract obstruction
Decreased pulmonary blood flow, decrease pulmonary veins return to left atrium
76
How does increased pulmonary vascular resistance and decreased systemic vascular resistance lead to a tet spell?
Changes in pulmonary and systemic resistance lead to increase right to left shunting which causes decreased pulmonary blood flow-->
Decreased blood flow through the pulmonary arteries decreases the PO2 causing acidosis and increased carbon dioxide
77
What methods counteract a tet spell?
```
Knees to chest
Morphine
Bicarb
Vasoconstrictors
Propranolol or esmolol
Fluid bolus
```
78
Surgical management of TOF involves
Blalock Taussig shunt
| VSD closure and RVOT obstruction repair
79
Survival with pulmonary atresia is dependent on the presence of
ASD or PFO with PDA
80
RVH occurs in pulmonary Atresia due to
RVOT
81
EKG for pulmonary Atresia will show
Normal QRS
LVH>> RVH
RAH 70%
82
In addition to PGE, PA is treated with
Angiography to determine anatomy
| BT shunt +/-RVOT reconstruction (if not RV dependent)
83
Truncus arteriosus is associated with
DiGeorge syndrome
| TA'GD
84
Truncus arteriosus increases the risk of
Right aortic arch
| Interrupted aortic arch
85
What other defect is always associated with the truncus archeriosis?
VSD
86
Which type of TA is most common and has the main pulmonary artery branch from the truncus then split?
Type 1, 50-70%
87
What is the difference between type 2 and 3 truncus arteriosus?
Type 2 PA branches posteriorly
| Type 3 PA branches laterally, least common type
88
Truncus arteriosus is marked clinically by
```
Cyanosis
CHF
Wide pulse pressure
Bounding pulses
Pansystolic murmur
Single S2
```
89
Truncus arteriosus is associated with right aortic arch ____%
50%
90
Surgical repair plan for truncus arteriosus is
Early, complete repair
91
Tricuspid Atresia without a _____ has a worse severity.
VSD- poor RV development, ductal dependent
92
30% of tricuspid Atresia also have
Great arteries transposed
93
Clinical findings in tricuspid Atresia are
```
Severe cyanosis
CHF
Systolic murmur and single S2
+/- Pulmonary vascular markings if PBF
Left superior QRS
LV>>RV
Arterial hypertrophy
```
94
Following PGE1, surgical plan for tricuspid Atresia is
Rashkind (balloon septostomy)
| PA banding if ++ PBF
95
The Congenital heart defect associated with maternal lithium use
Ebstein's anomaly
96
A heart defect with an enlarged right atrium, displaced tricuspid valve, small right ventricle, and 80% with an ASD is
Ebstein's anomaly
97
20% of ebstein's anomaly have this arrhythmia
WPW
98
The high mortality of ebstein's anomaly is attempted treatment with:
PGE1
Treat CHF
Airway stabilization
If severe symptoms, surgical intervention
99
A non-cardiac complication of ebstein's anomaly is
Airway compromise due to dramatic cardiomegaly
100
Asplenia or polysplenia are increased in which congenital heart defect?
Single ventricle
101
Single ventricle usually looks like
Single left ventricle with left transposition (Aorta comes off small leftward RV)
Absent ventricular septum (complete mixing)
102
Symptoms of single ventricle dependent on:
PBF-
- Increased: CHF, mild cyanosis, enlarged heart/PVM
- Decreased: mod-severe cyanosis, mild CHF, normal heart size/PVM
103
Surgical treatments for single ventricle are
CHF-> PA banding
Palliative -> Glenn procedure
Definitive -> Fontan procedure
104
TAPVR is defined as
Pulmonary veins drain to RA
| PV can be supracardiac, cardiac, or infracardiac
105
PV draining to coronary sinus in TAPVR is
Cardiac
106
PV draining to portal vein or IVC in TAPVR is
Infracardiac
*Most likely to be obstructive
107
The TAPVR type with the worst severity is
Infracardiac/obstructive
| Surgical repair urgently
108
Treating TAPVR with PGE1 can cause
worsened pulmonary congestion/edema
109
If the aorta and pulmonary artery both come from the RV, this is ________.
The other anomalies associated with DORV are
Double outlet right ventricle
VSD
Great arteries can be side by side or transposed, +/- PS
110
Clinical presentation in DORV is dependent on
Size/type of VSD
+/- PS
EKG: RVH
111
Arrhythmia associated with DORV is
First degree heart block
112
What kind of surgical repair is indicated for DORV?
Depends on severity of VSD and PS
113
What is the most common cause of CHF after the 2nd week of life?
VSD
114
Most common type of VSD is
Perimembranous (70%)
115
Inlet and outlet VSD's each make up ____% of all VSD's.
~7%
116
Describe the presentation of a mod -large VSD
Clinically silent for 2-3 days, then CHF, poor feeding, respiratory distress, then harsh holostystolic murmur
117
Definitive surgery for VSD is indicated when
Significant left to right shunt (2:1)
Severe CHF
Poor growth
Increased pulmonary artery pressure
118
Second most common cardiac defect is
ASD (6-11%)
119
ASD is more common in
Females
120
RV overload occurs in ASD because
Left to right shunting due to increased RV >> LV compliance
121
In contrast to VSD, EKG in ASD shows
RVH, RAD
122
Surgery in ASD is
At 2-5 years, definitive closure if RV overload
123
Molecules that prompt PDA closure
PGFa, acetylcholine, bradykinin, oxygen
124
Physiology of a PDA mimics a
VSD
125
A continuous or systolic machinery murmur suggests a
PDA
126
Complete AV canal defect is associated with
Trisomy 21
127
Associated defects with AV canal defects are
PDA and TOF (10%)
128
Primum ASD, inlet VSD, and common AV valve is a
Complete AV canal defect
129
Complete and partial AV canal defects are differentiated by
Partial:
+/- cleft in MV
No VSD
Normal TV
130
Symptoms in AV canal defects are
Dependent on ASD/VSD contributions
131
AV canal murmur is
Systolic due to VSD
| +/- apical diastolic murmur, +/- gallop
132
Surgical correction of AV canal defect involves
ASD/VSD closure
| AV valve separation
133
Partial anomalous pulmonary venous return is
One or more PV drain into RA
| RIGHT>>> LEFT (2:1)
134
In PAPVR, left pulmonary veins most often drain
To the innominate vein
135
Clinically PAPVR mimics
ASD
136
Pulmonary congestion in PAPVR is dependent on
Number of anomalous veins
ASD qualities
PVR
137
Similar to ASD, PAPVR clinically has
ASD murmur
RVH, RAE
increased PVM
EKG: RVH, RV conduction delay
138
Does PAPVR need surgical correction?
Only for clinically significant left to right shunt
139
Obstructive cardiac lesions are
Coarctation of the aorta
Pulmonic stenosis
Aortic stenosis
Infracardiac TAPVR
140
Coarctation of the Aorta of associated with
Turners syndrome (30% of Turner's patients)
141
Cardiac defects associated with CoA
Bicuspid aortic valve
| VSD
142
The form of CoA with the worst severity is
Preductal
Presents after birth
Differential cyanosis
Shock following PDA closure
Associated with other defects
143
The CoA most likely to have collateral vessels:
Juxtaductal and postductal
144
Rib notching in CoA is a sign of
Collateral vessels
145
Juxta- and post- ductal CoA commonly presents with
Hypertension or BP gradient
146
CoA murmur is
Systolic rejection at left interscapular area
| +/- AR bicuspid valve murmur
147
CoA surgery involves
End to end anastomosis
+/- balloon angioplasty
Surgery often followed by hypertension
148
Pulmonic stenosis and aortic stenosis are each approximately ___% of CHD
5%
149
_________ stenosis is more common in males.
Aortic (4:1)
150
Williams syndrome is associated with ________ stenosis
Both pulmonic and aortic stenosis, supravalvular
151
Subvalvular pulmonic stenosis is associated with ______
TOF
152
Bicuspid aortic valve is associated with ______ ________ stenosis
Valvular aortic stenosis
153
Valvular positioning of pulmonic and aortic stenosis can result in
Post-stenotic dilation
154
If mild, both pulmonic and aortic stenosis present
```
Asymptomatic
No cyanosis
Election click
Split S2
Normal heart size
```
155
Severe pulmonic and aortic stenosis both require
PGE1
| balloon valvuloplasty
156
Pulmonic stenosis murmur
ULSB Systolic ejection with radiation to back
| Grade proportional to stenosis
157
Aortic stenosis murmur
URSB, left midsternal systolic ejection murmur, radiation to LLSB
Grade is INDEPENDENT of degree of stenosis
Increased stenosis--> 2nd R intercostal thrill
158
```
Aortic valve regurgitation:
Phys
Presentation
CXR/EKG
Management
```
Incr preload/SV-> aorta dilation
Wide pulse pressure, bounding pulses. Early diastolic murmur.
LVE, dilated Aorta. ST depression, T wave inversion
Treat CHF, valve repair
159
```
Mitral valve regurgitation
Phys
Presentation
CXR/EKG
Management
```
LA/LV overload--> hypertrophy
Systolic blowing & diastolic murmurs at apex
Increased LV
Treat CHF, valvuloplasty vs MV replacement
160
```
Tricuspid valve regurgitation
Phys
Presentation
CXR/EKG
Management
```
```
RA/RV overload
RV dysfunction+/- cyanosis if R-L
Systolic blowing murmur, diastolic murmur at LLSB
Hepatic congestion
RAH
Conservative, surgery only if necessary
```
161
HLHS is most common in
Males
162
The second most common CHD with cyanosis in the guest week of life is
HLHS
163
Aortic or mitral valve atresia/stenosis, hypoplastic LV and aortic arch hypoplasia is
HLHS
164
Patients with HLHS shows symptoms when
The PDA closes-->
| CHF, metabolic acidosis, shock
165
HLHS EKG/CXR show
+PVM, cardiomegaly, RVH, RAD
166
Management of HLHS
PGE1, inotropes->
balloon septostomy -->
Norwood--->
heart transplant if needed
167
Hypertrophic cardiomyopathy is associated with
Pompe
Hurler
Noonan
IDM
168
Dilated cardiomyopathy is associated with
Myocarditis
Carnitine deficiency
Abnormal perfusion
Post-arrhythmia
169
Cardiac dysfunction in dilated cardiomyopathy is due to
Decreased ventricular function due to overstretch/dilation of LA/LV
170
Cardiomyopathy with CHF and MR murmur is
Dilated cardiomyopathy
171
The least common form of cardiomyopathy is
Restrictive
172
Marked arterial>>ventricular dilation with abnormal ventricular filling is
Restrictive cardiomyopathy
173
EKG in HCM shows
LVH, ST and T wave changes
| Possible arrhythmia
174
If obstructive, HCM should be treated with
Preload
Myomectomy
Avoid digoxin, inotropes
175
An S4 gallop is seen in
Dilated cardiomyopathy
176
EKG In dilated cardiomyopathy shows
Increased LV, ST and T wave changes
Q waves
possible arrhythmia
177
Dilated cardiomyopathy should be treated with
Vasodilators
Treat CHF
Possible anticoagulation
Possible heart transplant
178
Restrictive cardiomyopathy can mimic
Pulmonary hypertension
179
Persistent pulmonary overcirculation, pulmonary vasculature remodeling, increased PVR and RA/RV enlargement are due to
Eisenmengers complex- progression of untreated CHD
180
Treatment of eisenmengers complex includes
Nifedipine
Prostacyclin
Nitric oxide
Lung transplant
181
Acquired pulmonary vasoconstriction leading to RVH and RV dysfunction that results from severe lung disease is
Cor pulmonale
182
Although irreversible, Cor pulmonale is treated with
Addressing underlying disease, diuretics, pulmonary vasodilators, oxygen
183
Pulsus paradoxus from rapid fluid accumulation is
Pericardial effusion--> cardiac tamponade
184
Pericardial effusion can be caused by
Pericarditis
Severe anemia/CHF
Post cardiac surgery
CVL leakage
185
Treatment of pericardial effusion
Pericardiocentesis
| Treat underlying disease
186
Left coronary artery originating from pulmonary artery is
Anomalous origin of the LCA from the PA (ALCAPA)
187
4 stages of ALCAPA are marked by
1- elevated PVR, adequate perfusion
2- decreased PVR and ALCA flow, increased dependence on collaterals
3- asymptomatic phase, collaterals maintaining perfusion
4- decrease PVR-> decrease collateral flow to ALCA (pulmonary-coronary steal)--> LV ischemia, LAD infarction, pulmonary congestion
188
Clinical presentation of ALCAPA is
```
Onset 2-3 months
Respiratory distress
Feeding intolerance
FTT
Transient ischemia: pallor, paroxysmal crying, diaphoresis
```
189
When ALCAPA reaches stage 4 (CHF), EKG shows
Deep Q waves (I, aVL, v4, v5, v6)
| ST elevations v4-6
190
Diagnosis and treatment of ALCAPA include
EKG (showing infarction)
Echo with Doppler
Cardiac Cath if echo non-confirmatory (incr Pulm:syst blood flow)
ALCA anastamosis to aorta
191
Prognosis of ALCAPA is
Dependent on early intervention before significant myocardial injury
May require transplant
High mortality of undiagnosed
192
In general, in utero cardiac tumors can cause
Hydrops
Fetal arrhythmia
Can be asymptomatic
193
The most common cardiac tumor is
Rhabdomyoma
194
Qualities and treatment of rhabdomyomas
Usually multiple
Commonly involve ventricle and septum
Associated with tuberous sclerosis
Surgery if outflow obstruction
195
A well circumscribed, fibrous single cardiac tumor is
Fibroma
| Most in LV
196
A cardiac tumor found in adulthood in the LA that can cause inflow obstruction
Myxoma
197
A rare cardiac tumor of cardiac myocytes is
Sarcoma
198
An intrapericardial tumor that can cause pericardial effusion is
Teratoma
199
The cardiac malformations associated with asplenia are
Always severe
200
Bilateral ______ sidedness is associated with asplenia.
Right
201
Bilateral left sidedness and less severe cardiac malformations are associated with
Polysplenia
202
TAPVR, bilateral SVC, AVC, TGA, PS/PA, single ventricle, dextrocardia are associated with both asplenia and polysplenia, but ________ are associated specifically with asplenia.
Aorta/IVC juxtaposition (100%)
203
Non cardiac sequelae of asplenia are
```
2 right lungs, gallbladders
Malrotation
Howell-Jolly & Heinz bodies
Strep pneumo
Cyanosis
```
Poor prognosis
204
Non cardiac sequelae of polysplenia are
2 left lungs
Biliary atresia
Malrotation
Cyanosis
Poor prognosis
205
Fibromuscular septum divining the LA to two compartments is
Cor triatriatum
206
Cor triatriatum presents
```
Decreased peripheral pulses
Tachypnea
FTT
pulmonary edema
Loud P2
Increased PVM
EKG: RAD, RVH
```
207
Treatment of Cor triatriatum
Treat pulmonary edema
| Surgery if necessary
208
Cyanide heart disease more prone to pulmonary over circulation are
TAPVR
HLHS
TGA/VSD
Truncus arteriosus
209
Chest x-ray finding of a boot shaped heart is concerning for
Tetralogy of Fallot
210
Chest x-ray finding of an egg on a string is concerning for
D-TGA
211
Chest x-ray finding of a snowman shaped heart is concerning for
TAPVR, supracardiac
212
Chest x-ray finding of an extremely large heart with decreased pulmonary markings is
Ebstein's anomaly
213
Chest x-ray findings of a small heart with increased pulmonary markings is
Obstructive TAPVR
214
Chest x-ray findings concerning for right aortic arch with increased pulmonary markings is
Truncus arteriosus
215
Chest x-ray findings concerning for right aortic arch with decreased pulmonary markings is
Tetralogy of Fallot
216
QTc calculation
QT/ √RR interval
217
In infants over 6 months, normal QT is
<0.45s
218
RV hypertrophy on EKG is
V1: increased R, persistent upright T, increased R/S ratio, possible Q wave
219
LV hypertrophy on EKG is
V6: increased R, Q wave (+v5), peaked T waves, increased R/S ratio
220
Neonates <1 months and those with TOF have an EKG axis
+90- +/- 180°
221
Infants with tricuspid Atresia or AV canal have an EKG axis
0- -90°
222
Infants older than 1 month or with PA with intact septum have any EKG axis
0- +90°
223
Upright T wave in V1 after 72h suggests
RVH
224
Normally, p wave is ______° and QRS/T are ________°
0-90°
100-150°
225
Arrhythmia affect ___% of fetuses
1-2%
226
Fetal magnetocardiography (FMCG) increases into about arrhythmias with
Observation of QRS and QT interval, beat variability, and presence of T waves
227
Most tachyarrhythmias are
SVT (70-80%)
228
SVT typically presents at _____ weeks
28-32
229
Factors that determine perversion to groups in SVT
Prematurity and duration of SVT
230
Top 3 treatments for fetal SVT:
Digoxin
Amiodarone
Procainamide/flecainide/sotalol
231
Side effect of procainamide for fetal SVT
Can induce contractions
232
Flecainide and sotalol are limited to 2nd line treatment for SVT due to increased
Mortality
7-15% flecainide
30% sotalol
233
For tachyarrhythmias, a treatment that works in SVT but not Atrial flutter is
Amiodarone
234
Both tachyarrhythmias are associated with an increased risk of
Necrotizing enterocolitis
235
Cardiac change associated with maternal thyrotoxicosis
Sinus tachycardia
236
AV block is associated with ______ and presents at ___ weeks
Maternal SSA/SSB antibodies
Any gestational age
237
AV block with maternal antibodies is at risk for
```
Needing cardiac pacing at birth
Cardiomyopathy b(30%)
```
238
AV block with a poorer prognosis has
No maternal antibodies
Additional cardiac defects
Higher risk for hydrops/CHF/demise
+/- a-/poly- splenia
239
There is an increased risk of SVT in the first month of life if the fetus has
Fetal ectopy
240
PACs are associated with
Hyperthyroidism
CHD
Cardiomyopathy
Central line misplacement
241
PVC's can be caused by
```
Digoxin toxicity
Infection
Electrolyte abnormalities
Hypoxemia
Acidosis
CHD
Aminophylline/caffeine
Myocarditis
```
242
Premature junctional contractions
Lack p waves with normal QRS
243
WPW, atrial flutter, atrial fibrillation, SVT, and ventricular tachycardia are examples of ______, the most common type of tachyarrhythmia.
Reentrant tachycardia
244
Reentrant tachycardia can be treated with
DC cardioversion
245
Junctional ectopic tachycardia, ectopic atrial tachycardia and ventricular tachycardia are _______ tachyarrhythmia.
Abnormal automatic focus
| Typically refractory to DC conversion
246
Abnormal p waves with atrial ectopic focus and rapid rate is
Ectopic atrial tachycardia
| Treated with beta blocker/antiarrhythmic
247
Junctional ectopic tachycardia happens most often _______ with EKG __________
Post operatively
| Ventricular rate faster than atrial rate
248
Junctional ectopic tachycardia happens most often _______ with EKG __________
Post operatively
| Ventricular rate faster than atrial rate
249
Junctional ectopic tachycardia is treated with
Normalize electrolytes
Limit inotropes
Arterial pacing+/- amiodarone, procainamide
250
Adenosine or ice given to an infant with atrial flutter causes
Allowed HR that can reveal sawtooth pattern
251
Atrial flutter is treated with
Digoxin
| DC synchronized cardioversion/esophageal pacing if unstable
252
Atrial rate of 360+ bpm is __________ and treated with _____________
Atrial fibrillation
| DC defibrillation, digoxin
253
Tachycardia with a wide QRS should be assumed to be
Ventricular tachycardia
254
Ventricular tachycardia is usually treated with
Lidocaine
If unstable, DC cardioversion
255
Causes of ventricular tachycardia
```
CHD
Electrolyte abnormalities
Hypoxemia
Myocarditis
Cardiac tumors
Digoxin toxicity
Prolonged QT
Cardiomyopathy
```
256
Rapid irregular rate with abnormal QRS
Ventricular fibrillation
257
Causes of ventricular fibrillation
```
CHD
Prolonged hypoxia
Hyperkalemia
Myocarditis
Medications
Cardiomyopathy
Tumors
```
258
Prolonged PR is a
First degree AV block
259
Increasing PR interval leading to a dropped atrial impulse is
second degree AV block
Mobitz I
Wenckebach
260
Abrupt missing atrial beat without change in PR interval is
Second degree AV block
| Mobitz II
261
Dissociated AV construction with independent ventricular and atrial rates
Complete AV block
Third degree AV block
Associated with LTGA, AVC, lupus
262
Outcome of compete heart block
Poor prognosis of HR <55, prolonged QT, wife QRS, ventricular dysfunction
pacing required if symptomatic
263
RBBB is associated with
Ebstein's anomaly
264
Prolonged QT interval is ______ and can lead to _______
>0.45s
Ventricular tachycardia, SIDS
265
Treatment for prolonged QT is
Propranolol
| Pacing if necessary
266
SA node injury after cardiac surgery is
Sick sinus syndrome
| Can be associated with atrial flutter/fibrillation
267
Cardioversion dosing
0.25-0.5j/kg
Max dosing 2j/kg
268
Defibrillation dosing
1-2 J/Kg
| Max dosing 4j/kg
269
A prolonged QRS with slurring is
WPW
270
WPW is associated with
form of SVT
Ebstein's anomaly
L-TGA
271
WPW is treated similarly to
SVT
If stable, vagal maneuvers, adenosine, digoxin, propranolol
If unstable, synchronized DC cardioversion
272
Verapamil is contraindicated in infants less than 12 months due to
Increased risk of sudden death
273
EKG changes in hypercalcemia are
Shortened QT interval
274
EKG changes in hypocalcemia are
Prolonged QT interval
275
EKG changes in hyperkalemia
Peaked T wave, short QT, depressed ST-->
Prolonged PR, wide QRS, flat P wave-->
Absent P, sinusoidal QRS, systole/ventricular fibrillation
276
EKG changes in hypokalemia
Wide QRS, depressed ST, biphasic T-> u wave
--> Flat T, prominent U, prolonged PR, sinoatrial block
277
Shortening fraction calculation
LV diastolic dimension- LV systolic dimension/
LV diastolic dimension
X 100
278
On echo, AVC is best viewed
By apical view
279
On echo, ASD is best viewed
by subcostal view
280
Aortic arch is best seen on echo by
Suprasternal notch view
281
Left heart and valves are best seen on echo by
Parasternal view
282
Optimal timing of fetal echo is
18-32w
Transvaginal can be fine as early as 10 weeks
283
CHD difficult to visualize on fetal echo are
CoA,
Minor valve anomalies
VSD
ASD
284
Cardiac A1 receptors are located ______ and cause _______
Vascular smooth muscle
Cardiac myocytes
Increase contractility
Vasoconstriction
285
Cardiac a2 receptors are located ________ and cause _________
CNS
Sympathetic nerves
Block NE release
Inhibits sympathetic response
Vascular smooth muscle relaxation
286
Cardiac B1 receptors are located _____ and cause ________
Sinoatrial node
Cardiac muscle
Conduction cells
```
Increased HR (SA node)
Increased contractility
```
287
Mechanism of action of no milrinone/amrinone is
Phosphodiesterase inhibitor
| Leads to accumulation of cAMP
288
Effects of phosphodiesterase inhibitors are
Chronic inotropy
Decreases SVR
Some pulmonary vasodilation
289
cAMP accumulation by phosphodiesterease inhibitors causes
Inhibited cAMP breakdown leading to increased calcium cellular entry, increased contractility, relaxation of vascular smooth muscle
290
Mechanism of action of digoxin is
Inhibits sodium potassium ATPase pump
| Increases calcium influx
291
What are the clinical effects of digoxin
Negative chronotropic
Inotropy
Decreased SVR
292
N/K ATPase pump inhibition by digoxin causes
Decreased afterload
| Anti-arrhythmic via decreased AV conduction
293
Digoxin toxicity can cause
```
GI symptoms
Bradycardia
Prolonged PR
AV block
Hyperkalemia, hypercalcemia
```
294
Mechanism of action of dobutamine
Acts on beta 1 >>> beta 2 receptors
295
Clinical effects of dobutamine are
Chronotropy
Inotropy
Decreased SVR
296
Dobutamine acts as a
Synthetic catecholamine
297
Limited effects on afterload make dobutamine useful in settings of
Cardiogenic shock and myocardial dysfunction
298
Mechanism of action of dopamine
Endogenous catecholamine
Inhibits N/K ATPase and Na/H pump
Epinephrine and norepinephrine precursor
Promotes endogenous release of norepinephrine
299
Clinical effects of dopamine
++ Chronotrope
Inotropy
Dose dependent SVR increase
300
Clinical benefits of dopamine are
LOW DOSE: Renal vasculature dilation
MODERATE DOSE: B1 and dopaminergic receptors -> inotropy, ^HR
HIGH DOSE: B1 and A1
301
Mechanism of epinephrine
Potent vasopressor
| B1, B2 >> a
302
Clinically effects of epinephrine
Strong Chronotropy
Inotropy
Dose dependent SVR increase
Can improve coronary artery perfusion
303
Epinephrine adverse effects include
Hypokalemia
Local tissue ischemia
Renal vascular ischemia
Severe hypertension
304
Mechanism of isoproterenol
Synthetic catecholamine
| B only
305
Clinical effects of isoproterenol
+++++ Chronotropy
--> useful for complete heart block
Inotropy
Decreased SVR
Not helpful in shock
306
Effects of nitroprusside
Decreased SVR
Vasodilator
Increases intracranial pressure
Can cause cyanide toxicity, tissue necrosis
307
Mechanism of norepinephrine
A> B1 > B2
Chronotropy, inotropy
Increases SVR
308
Adverse effects of norepinephrine
```
Second line to epinephrine
Profound vasoconstriction
Renal vasoconstriction
Hypocalcemia
Hypoglycemia
```
309
Mechanism of indomethacin
Cyclooxygenase inhibitor
Blocks prostaglandin synthesis
60-80% successful PDA closure, 30% relapse
310
Mechanism of prostaglandin
Vasodilator, maintains PDA
311
6-15% of patients receiving prostaglandin will have
Apnea
312
PGE1 relatively contraindicated in
Obstructive TAPVR
HLHS with intact septum
TGA, restrictive septum
Mitral valve Atresia
313
Endocarditis prophylaxis is indicated for
```
Unrepaired CCHD
Repaired CCHD with prosthetic material 6 months post-op
Incompletely repaired CCHD
Cardiac transplant
Prosthetic valve
Prior bacterial endocarditis
Rheumatic heart disease
HCM
MV prolapse+ regurg
```
314
Fetal valvuloplasty is offered to prevent HLHS for patients with:
Critical AS <26 weeks without progression to LV hypoplasia
315
Fetal valvuloplasty is offered to prevent hypoplastic right ventricle to patients with
PA with intact ventricular septum
316
Fetal ASD creation is offered to palliate HLHS to patients with
HLHS with intact septum
Or
TGA with intact septum
317
The rashkind procedure is a ________ and is used in
Septostomy
D-TGA
Severe MS
HLHS with inadequate mixing
318
The Blalock-taussig procedure creates _________ and is used for
Shunt between subclavian artery and ipsilateral pulmonary artery
Increases PBF
TA, PA, TOF with severe PS
319
The Glenn procedure creates _________ and is used for
Connection between SVC and PA to increase PBF without RV
TA
Single ventricle with severe PS
320
Mustard, Senning, Rastelli and Jatene are all procedures used for treating
D-TGA
321
The mustard procedure creates
An intraatrial baffle after atrial septum removed
SVC/IVC -> LA -> LV -> PA
322
Senning procedure differs from the Mustard
Because the baffle is made using atrial septum and RA wall instead of pericardium
323
The Rastelli procedure
Patches the VSD to decrease deoxygenated blood mixing into aorta.
Used in d-TGA with VSD and PS
324
The procedure to switch the arteries position in d-TGA is the
Jatene procedure
325
The procedure to reroute the SVC and IVC to the PA is
Fontan
Used in tricuspid Atresia and single ventricle
326
The 3 stages of the Norwood to repair HLHS are
1- septostomy, MPA division, hypoplastic aorta repair, shunt to increase PBF
2- Bidirectional Glenn, shunt removed, SVC connected to PA
3- Modified Fontan (SVC/IVC to PA)
327
Syndromes with a >50% likelihood of CHD
C- Carpenter and CHARGE
E- Ellis van Creveld
H- Holt-Oram
N- Noonan
T- Tri 13, 18, 21
V- VACTERL
W- Williams
328
Most common CHD in carpenter syndrome
VSD, ASD
329
Most common CHD in cat eye syndrome
TAPVR
330
Most common CHD in CHARGE
TOF
331
Most common CHD in Cornelia-de-Lange
VSD
332
Most common CHD in cri du chat
Varies
333
Most common CHD in DiGeorge
Aortic arch (Truncus arteriosus)
334
Most common CHD in Ehler-Danlos
Aortic root dilation, MV prolapse
335
Most common CHD in Ellis van Creveld
Common atrium
336
Most common CHD in glycogen storage diseases 2a (Pompe)
HCM
337
Most common CHD in Goldenhar
VSD, PDA, TOF
338
Most common CHD in Holt-Oram
ASD
339
Most common CHD in homocysteinuria
Arterial/venous thromboses
340
Most common CHD in Hurler
Thickened valves
CAD
HCM
341
Most common CHD in Klinefelter
TOF
| MV prolapse
342
Most common CHD in Klippel-Feil
VSD
343
Most common CHD in Marfan
Aortic dilation
Aneurysm
MV prolapse
344
Most common CHD in Meckel-Gruber
ASD, VSD
345
Most common CHD in Noonan
Dysplastic pulmonary valve
| LVH, HCM
346
Most common CHD in Rubenstein -Taybi
VSD, ASD
347
Most common CHD in TAR (thrombocytopenia, absent radius)
TOF
| ASD
348
Most common CHD in trisomy 13
VSD/PDA (80-90%)
| 50% with other anomalies
349
Most common CHD in trisomy 18
VSD, PDA, PS, CoA
350
Most common CHD in trisomy 21
Complete AVC
VSD
PDA
351
Most common CHD in Turner
Bicuspid aortic valve
CoA
AS
352
Most common CHD in VACTERL
VSD
| TOF/CoA
353
Most common CHD in Williams
Supravalvular subaortic stenosis
354
Infectious causes of myocarditis are
Coxsackie B
Parvovirus B19
Rubella
355
CHD due to rubella are
AS
PS
TOF
356
Maternal medications that can cause VSD are
```
Ethanol
Hydantoin
Phenytoin
Trimethadione
Valproic acid
```
357
Maternal medications that can cause ASD are
```
Ethanol
Hydantoin
Phenytoin
Trimethadione
Valproic acid
```
358
Maternal medications that can increase risk of HLHS
Retinoic acid
| Trimethadione
359
Maternal medications that increase risk of pulmonary hypertension
Aspirin
| SSRI
360
Thalidomide can cause
Conotruncal malformations
361
The medication that can cause TGA is
Retinoic acid
362
_____ % of mother's of an infant with complete heart block have lupus
30-60%
