General Info Flashcards
(354 cards)
1
Q
Most common anatomy of a bicuspid aortic valve?
A
- R/L Fusion
- R/N Fusion
- L/N Fusion
2
Q
Most common coronary artery pattern in D-TGA?
A
Usual coronary arteries
3
Q
Most common coronary artery abnormality seen in D-TGA?
A
Circumflex from RCA
4
Q
Fibrous continuity of which valves seen in majority of ToF?
A
Tricuspid/Aortic
5
Q
ToF in South American and Southeast Asian patients?
A
- Complete absence of infundibular septum
- Continuity between aortic/pulmonary valves
- Double committed sub-arterial VSD
6
Q
Mitral arcade?
A
Shortened/absent chordae
Leads to direct insertion of leaflets to papillary muscles
7
Q
What does kidney release in response to decreased renal perfusion?
A
Renin
8
Q
What does angiotensin II do?
A
Vasoconstriction
ADH/Vasopressin secretion
9
Q
Most common aortic arch malformation in asymptomatic patients?
A
Left arch with aberrant right subclavian
- 0.5% general population
- Common in trisomy 21
10
Q
Surgery for DORV with subpulmonary VSD?
A
Arterial switch with VSD closure
-Physiology is like TGA
11
Q
Most common additional defect seen in ToF?
A
ASD or PFO (80%)
Right aortic arch (25%)
12
Q
% of ToF with right aortic arch?
A
25%
13
Q
Most common type of AVSD in trisomy 21?
A
Complete AV canal defect
Rastelli Type A
14
Q
Most common reason for reoperation following partial or complete AV canal repairs?
A
AVVR (usually left)
15
Q
LVOTO occurs more commonly after which type of AVCD repair?
A
Partial
16
Q
Whale tail?
A
TAPVR to CS
17
Q
Primary heart field cells become what cardiac structures?
A
LV and bilateral atria
18
Q
Secondary heart field cells become what cardiac structures?
A
Majority of RV and conotruncal outflow tracts
19
Q
Migration of neural crest cells into developing heart causes what embryologic process?
A
Septation of conotruncal outflows into aorta and pulmonary arteries
20
Q
What fetal remnant delivers blood from hepatic veins to RA?
A
Ductus venosus/Ligamentum venosus
21
Q
What is position of sinus venosus ASD relative to fossa ovalis?
A
Superior/Posterior
22
Q
Where is sinus node found in left sided juxtaposition of the atrial appendages?
A
Anterior or Inferior
23
Q
Left juxtaposition of the atrial appendages is associated with that types of cardiac defects?
A
Abnormal ventriculo-arterial connections
24
Q
Where is sinus node found in right juxtraposition of the atrial appendages?
A
Normal
25
What types of lesions are seen in right juxtaposition of the atrial appendages
ASD, VSD, PDA (simple lesions)
26
What structure most likely damaged in patient with stridor following coarctation repair or surgical PDA ligation?
Left recurrent laryngeal nerve
27
Most common additional defect seen in HLHS and what % of patients?
Coarctation
| 80%
28
What shifts Hgb-O2 dissociation curve left?
Increased pH (Decreased H+)
Decreased CO2
Decreased 2,3-DPG
Decreased temp
29
What shifts Hgb-O2 dissociation curve right?
```
Exercise
Decreased pH (Increased H+)
Increased CO2
Increased temperature
Increased 2,3-DPG
```
30
Ca stored where?
Sarcoplasmic reticulum
31
Most common morphology of truncal valve
Tricuspid
32
Most common abnormal morphology of truncal valve
Quadricuspid (20%)
33
Truncal valve is most commonly in continuity with what other valve?
Mitral
34
Best anatomical hallmark of LA?
Valve of fossa ovalis (septum primum)
35
Triangle of Koch
Tricuspid valve annulus (septal leaflet)
CS ostium
Tendon of Todaro
36
Most reliable anatomical feature that distinguishes RV?
Level of insertion of AV valve
37
Which aortic arch do the PAs and ductus come from?
Left 6th
38
Majority of true aortic arch comes from which arch?
Left 4th
39
Which arch gives rise to proximal right subclavian?
Right 4th
40
Which coronary supplies posteromedial papillary muscle?
Right
41
Anterolateral papillary muscle is supplied by which coronary artery?
LAD and Circumflex
42
Fetal circulation, highest O2 content?
Umbilical vein (blood from Mom)
43
Fetal circulation, lowest O2 content?
Umbilical arteries
44
Ligament of Marshall
Remnant of left SVC
45
What are distinguishing features of RA?
Limbus of fossa ovalis
Connection of IVC
Large pyramidal appendage
46
What is Libman-Sacks endocarditis?
Non-infective endocarditis associated with SLE affecting mitral valve
47
Normal location of sinus node?
Subepicardial
| Junction of SVC and crista terminalis
48
Blood supply to sinus node?
RCA (60%)
| Circumflex (40%)
49
AV node blood supple
Dominant coronary artery- 80% RCA
50
Where is conduction system in inlet VSD?
Anterior/superior to defect
51
Gradient indicative for pulmonary balloon valvulplasty?
> 40mmHg (cath or echo)
-No gradient criteria is significant symptoms
52
Balloon size for pulmonary valvuloplasty
120-140%
53
Gradient indicative for aortic valvuloplasty?
- AS with decreased LV function regardless of gradient
- Cath peak >50
- Cath peak >40 with symptoms
54
Balloon size for aortic valvuloplasty?
80-90%
55
Goal gradient for aortic valvuloplasty?
<25-35mmHg by cath
56
Catheter based gradient indicative for recoarctation angioplasty?
- >20mmHg
| - <20 mmHg is significant collaterals, single ventricle, ventricular dysfunction
57
Balloon size for recoarctation?
-2-3 times stenosis, but <1mm larger than smallest proximal/distal vessel
58
Gradient indicative of need for branch PA angioplasty?
- >20-30mmHg
- RVP > 1/2 to 2/3 systemic
- Flow discrepancy
59
Balloon size for branch PA angioplasty?
- 3-4 times narrowest segment
| - 15-20% restenosis rate
60
Normal location of AV node?
Subendocardial
| Triangle of Koch
61
What population are sub-arterial VSDs most commonly seen in?
Asian
62
Which aortic cusp is most likely cause of AI in perimembranous and subarterial/supracristal VSDs?
Right
63
What type of VSD is a overriding AV always associated with?
Malalignment
64
Abdominal features of asplenia?
1. Midline liver
2. 2 Mirror image right lung lobes
3. Patent biliary tree with single gallbladder
4. Malrotation common
5. Aorta/IVC on same side of vertebral column
65
Abdominal features of polysplenia?
1. Visceral situs varies
2. Single gallbladder, but biliary atresia common
3. Interrupted IVC with azygous continuation
4. Multiple spleens on same side
66
Most common aortic-pulmonary position in DORV?
Side by side
| Aorta to right
67
Crescent shaped valve at ostium of CS?
Thebesian valve
68
What is Chiari network?
Weblike structure from enlarged/persistent Eustachian and/or Thebesian valve or remnant of the valve of the sinus venosis
Extends from crista terminalis to Eustacian/Thebesian valve
69
Coronary artery features seen in truncus?
- Left coronary system arises from posterolateral suface
- LAD usually small and displaced leftward
- RCA from anterolateral surface
- Conal branch of RCA usually large
- Left dominance in 25-30%
70
What coronary branch determines dominance?
Posterior descending
71
What % of population is right dominant v. left dominant v. co dominant for coronary arteres?
Right: 70%
Left: 10%
Co: 20%
72
Most common coronary artery anomaly?
- Left circumflex from right main (no clinical significance)
| - Left main from right sinus can be problematic if tracks between aorta and RVOT
73
Vertical vein embryology?
Connection between splanchnic plexus of lung buds and cardinal veins
-NOT a left SVC... this is more posterior than left SVC
74
Anterior indentation on barium esophagram?
Pulmonary artery sling
- Tracks between trachea and esophagus
- Associated with tracheal stenosis
75
CHD in Williams Syndrome?
- Supravalvar AS and PS
- Coronary stenosis
- Renal artery stenosis - Leads to HTN
76
Most common association with right aortic arch?
ToF
77
Gene in Alagille?
JAG-1
-Liver disease, right heart disease (pulmonary stenosis, both valvar and branches)
78
NOTCH 1 mutation?
Aortic valve pathology
79
PTPN11 mutation?
LEOPARD Syndrome
| Noonan Syndrome
80
TBX1 mutation?
22q11 or DiGeorge
81
Features of DiGeorge?
```
Hypocalcemia
Immunodeficiency
IAA type B
Truncus
ToF
```
82
TBX5 mutation?
Holt-Oram
- Limb anomalies (missing thumb)
- Heart defects (ASD)
- 75% have heart defects
83
How many papillary muscles with a classic parachute mitral valve versus most common type of parachute mitral valve?
1 with classic
2 with most common (asymmetric chordal attachments to one of the papillary muscles)
84
Features of sinus of valsalva aneurysms?
1. Mostly male
2. 2/3 right sinus
3. Associated with outlet VSD
4. Right sinus to RV rupture most common
5. Non-coronary to RA rupture second most common
85
Posterior indentation on esophagram?
Vascular ring
86
Restrictive cardiomyopathy?
- Reduced ventricular volumes
- Preserved ventricular function
- Normal wall thickness
- Dilated atria
87
What anesthetic to avoid in muscular dystrophy?
Succinylcholine
88
What post-op RV pressure should a VD be reopened following RV-PA conduit in PA/VSD?
> 70%
89
Most common cause of vascular ring?
Double aortic arch
90
2nd most common cause of vascular ring?
Right arch with diverticulum of Kommerrell
91
Embryonic origin of right arch with left sided diverticulum of Kommerell?
- Right dorsal aorta
- No left 4th
- Left 6th persists
92
Which aortic arches disappear?
1, 2, 5
93
Embryology of IAA?
Absence of both 4th arches
94
Most common arch anomaly?
- Left arch with retroesophageal right subclavian (aberrant right subclavian)
- 38% of Trisomy 21 patients
95
Embryonic origin of left aortic arch with retroesophageal right subclavian?
Dissolution of right 4th and right 6th arches
96
Embryonic origin of left aortic arch with diverticulum of Kommerell?
Dissolution of right 4th and persistence of right 6th arches
97
Embryologic origin of right arch with mirror image branching?
- No left dorsal aorta or right 6th
| - Persistence of left 6th and left 4th
98
Most common arrhythmia in restrictive cardiomyopathy?
- Atrial flutter
| - 2nd and 3rd degree heart block
99
Describe Barth syndrome?
- X-linked
- Hypotonia, neutropenia, dilated cardiomyopathy
- Diagnosed by elevated 3-methylglutaconic acid in urine
100
CS is derived from what?
Left common cardinal vein
101
Left SVC is derived from?
- Persistence of left sinus horn
| - Left anterior cardinal and left common cardinal veins
102
Right SVC is derived from?
- Right anterior cardinal vein
| - Right common cardinal vein
103
IVC is derived from?
Subcardinal and supracardinal veins
104
Which end of the PDA are vegetation seen with in endocarditis?
Pulmonary end
105
RF for conotruncal defects?
1. Diabetes
2. Maternal PKU
3. Retinoic acid
4. Trimethadione
106
What is the primary management for mild and moderate MR?
Medical: Diuretics and afterload reduction
107
What echo and ECG findings are seen in cor triatriatum?
RA and RV enlargement
108
Describe Scimitar Syndrome
- Anomalous connection of right pulmonary veins to IVC
| - Usually have right lung hypoplasia
109
What screening is recommended for 1st degree relatives of HCM patients?
- ECHO every 12-18 months starting at age 12
| - ECHO every 5 years after 21 if no hypertrophy is seen
110
Best imaging modality for partial pericardial defect?
MRI
- Absence of portion of pericardium
- 80% left sided
- Heart displaced to left
- Bulge of great vessels
111
Which aortic valve cusps most commonly prolapses with VSDs
Right (followed by non)
112
Classic ECG findings with Duchenne muscular dystrophy?
Deep Q in I, aVL, V5, V6
113
What is mitral arcade?
Shortened or absent chordae which leads to stenosis and insufficiency
114
Uhl's anomaly?
- Complete absence of RV myocardium with marked RV dilation (parchment heart)
- Large P waves with diminished RV voltages
- Cyanosis
- Normal endocardial potentials (differs from Ebstein's)
- Similar pressure wave contours form RA/RV/PA
115
Where is AV node in L-TGA?
Anterior aspect of atrioventricular ring neat atrial septum
-Normally located in posterior position in subendocardium within triangle of Koch
116
When is coarctation repair recommended in asymptomatic infants?
2-3 years
117
What is most likely cause of myocarditis/dilated cardiomyopathy in a patient from Latin or South America?
Chagas disease
-Trypanosoma Cruzi
-Otherwise viral or coxsackie B is most common
118
Most common cause of bacterial pericarditis?
1. S. Aureus
2. H. Influenzae
3. S. Penumonia
- Fluid will be predominantly neutrophils
- Most likely due to hematogenous dissemination from lung
- Very ill at presentation
- Need Abx + Drainage
- Abx: Nafcillin/Oxacillin or Vanc + 3rd gen cephalosporin
- Constrictive pericarditis is a more common complication with bacterial compared to viral
119
Most common cause of pericarditis?
-Viral: Coxsackievirus
- Fluid is predominantly lymphocytes
- NSAIDs for treatment
- Constrictive pericarditis is rare
120
S2 changes with varying degrees of AS?
- Mild-Mod: Normal splitting
| - Severe: Single S2 due to prolonged ejection time
121
Formula for wall stress?
Stress = (Pressure * Radius) / Wall Thickness
122
Poiseulle's Law of Resistance?
(8 * Viscosity * Length) / Radius ^4
123
Delayed gadolinium is ideal to assess for?
-Myocardial perfusion including infarction (hyperenhancement on delayed imaging)
124
Dark blood imaging good for?
Anatomic diagnosis and structure
125
Bright blood imaging good for?
Qualitative and quantitative assessment of ventricles and valves
126
Most common defects seen in rubella?
PS
PDA
VSD
127
Which type of IAA is most commonly seen in AP septation defects like AP window?
A (1/3 of IAA
| -Distal to left subclavian
128
Which type of IAA is most commonly seen in DiGeorge?
B
| -Between left subclavian and left carotid
129
Heart defect associated with WPW?
- Ebstein's
| - LAD and WPW suggests Ebstein's
130
What BP is seen with supravalvar AS?
Higher BP in right arm compared to left (due to Coanda effect)
131
Which genetic syndromes are associated with PAPVR?
Turner
| Noonan
132
What defects are most commonly seen in Noonan?
- PS
- Hypertrophic cardiomyopathy
- PTPN11 gene mutation
- 80% have CHD
133
Which defect is most commonly associated with cc-TGA?
- VSD (80%)
| - LVOTO (subpulmonary) (30-50%)
134
Most common cause of viral myocarditis?
Coxsackie
135
AR murmur heard best in what position?
Patient leaning forward with breath held after exhalation
136
What position is MR best heard from rheumatic fever?
- Left lateral decubitus
| - Holosystolic, apical, radiates to the left axilla
137
What genetic syndrome is associated with congenital polyvalvular dysplasia?
Trisomy 18
138
High pitched click immediately after S1 at the apex?
BAV
139
Down sloping ST segments in V1 and V2 are characteristic of what?
Brugada
- Na channelopathy
- Loss of function SCN5A mutation
- Cove type ST elevation in V1-V3
- Unmasked with class 1 Na channel blockers (flecainide, procainamide)
140
Auscultation findings with worsening PS?
Murmur peaks later in systole and split widens (P2 is delayed)
141
Most common cardiac tumor in children?
Rhabdomyomas
- Well circumscribed, noncapsulated, ventricular
- Benign, spontaneous resolution
142
What defects are highly associated with NEC?
HLHS, Truncus
143
Most common major manifestation of RF?
Migratory polyarthritis
Carditis
Chorea
144
Myxoma?
- Pedunculated
- Friable
- Most commonly in LA
- Classic Triad: Obstruction, embolism, systemic illness
145
Systemic venous baffle obstruction more common after?
Mustard
146
Most common CHD in Cri-Du-Chat?
VSD
| 5p Deletion
147
Recoarctation rate for infants undergoing angioplasty for native coarctation
60-65%
148
Ellis-can Creveld?
- Post axial polydactyly
- Dwarfism
- Abnormal fingernails
- Amish
- Large ASD/common atrium
149
Max total dose of non-ionic contrast during cath?
6mL/kg
150
Lung segment with highest blood flow?
RLL
151
Which systemic vein has the highest O2 saturation?
Renal
152
Which systemic vein has the lowest O2 saturation?
CS
153
Equation for FS?
(EDD-ESD)/EDD
| -FS maximal during 1st month
154
Causes of holodiastolic flow reversal in aorta?
- Large PDA
- Severe AI
- Systemic to pulmonary shunt
- Large AV fistual
155
Anatomic hallmarks of complete AVSD?
- Cleft in anterior leaflet of left AV valve
- AV valves at same level
- Left papillary muscles rotated laterally
156
Anatomic hallmarks of RA?
- Broad based appendage
| - Connections of the IVC and CS
157
Most diagnostic echo finding in pericardial tamponade?
Collapse of RV in diastole
158
WOW associated with what CHD?
Ebsteins
159
Morphologic features of LV?
- Higher insertion of AV valve
- Lack of AV valve chordal attachments to septum (septophobic)
- Smooth walled
- Elliptical shaped
160
Most common fetal arrhythmia?
PAC
161
Normal orientation of the aorta?
Right and posterior to PA
162
Typical aorta location in d-TGA?
Anterior and right to PA
163
Typical aorta location in L-TGA
Anterior and left to PA
164
Typical aorta location in DILV?
Left and anterior or PA
165
RIsk of fetal exposure to lithium?
Ebstein (1%)
166
Indomethacin exposure?
Ductal constriction
167
Heart defect associated with fetal alcohol snydrome?
VSD
168
Heart defect associated with isretinoin?
d-TGA
| -Conotruncal defects
169
Heart defect in fetal hydantoin syndrome from phenytoin?
Coarctation and LVOTO
170
Most common heart defect in diabetic pregnancies?
VSD
TGA
Truncus
ToF
171
Heart defect with ACEi use?
Septal defects
172
Heart defect with SSRI use?
Septal defects
| PPHTN
173
Tei index
(Isovolumetric contraction time + Isovolumetric relaxation time) / Ejection time
174
Most common complication of subpulmonic VSD?
AR from prolapse of right cusp
175
Most common type of VSD seen with coarctation?
Perimembranous
176
Treatment of choice for fetal tachycardia with evidence of hydrops?
Sotalol 80mg PO BID
177
Most common associated lesion in DORV?
PS or PA
178
Valsalva does what to venous return?
Decreases
179
Valsalva does what to a Stills murmur?
Softer (decreased venous return)
180
AAIR pacing is indicated for?
Sinus node dysfunction
181
Hyperthyroid patients are prone to which cardiac arrhythmia?
A-fib
182
Chagas disease causes what rhythm disturbance?
AV block
183
ECG changes in hyperkalemia?
1. Peaked T waves
2. Widened QRS
3. Flat P waves
4. VF or cardiac standstill is not treated
184
Valsalva does what to HOCM murmur?
Louder (decreased venous return)
185
Load independent method for assessing LV systolic function
Stress-velocity index
186
Treatment for neonatal atrial flutter?
Cardioversion
187
What HR is in an indication for pacemaker in absence of CHD?
< 50
188
M band is composed of?
Myosin only
189
I ban consists of
Actin
Troponin
Tropomyosin
190
What 2 myocyte proteins prevent actin and myosin interaction?
Troponin I
| Tropomyosin
191
What HR is an indication for pacemaker in presence of CHD?
< 70
192
Kearns Sayre Syndrome?
- Chronic opthalmoplegia
| - Heart block (pacemaker indicated with bifasicicular block)
193
A band consists of?
Actin and myosin (all filaments = A band)
194
Treatment for Torsades de Pointes
IV Mg Sulfate
195
Pompe's disease?
- HCM
- Short PR interval
- Glycogen storage disease
- Hepatomegaly
196
Typical ECG pattern seen in CPVT? Hos is it treated?
- Normal at rest
- Bidirectional VT with exercise or emotion
- Treat with beta blockers
197
What does amiodarone do the effect of warfarn, digoxin, cyclosporine and phentyoin?
Increases effect (will need to decrease dose)
198
Which immunotherapy is associated with the development of diabetes?
Tacrolimus
199
Amiodarone can have what effect on fetus?
Neonatal hypothyroidism
200
WPW most commonly seen what 2 forms of CHD?
Ebstein
L-TGA
(Also HOCM)
201
Brugada classic ECD findings and treatment?
- ST elevation in V1/V2
- T waves deeply coved negative
- Caused by congenital defect of Na Channels (SCN-5A)
- Causes young healthy people to develop VF, potential for death
- Procainamide challenge used for diagnosis
- Treatment = Defibrillator
202
Lyme carditis causes what conduction distrubance?
AV nodal conduction abnormalities (heart block)
-Treatment: Ceftriaxone, doxycycline, amoxicillin
203
Position of displaced conduction system in AV canal?
Posterior and inferior to VSD
204
What does a short HV interval (<25ms) indicate?
Accessory pathway to ventricles
205
LQTS1 typically presents during what?
Exercise
206
LQTS1 is caused by what mutation?
KCNQ1
207
Most likely diagnosis for tachycardias with RP longer than PR
- Atypical AVNRT
- PJRT (AVRT via slowly conducting accessory pathway
- Atrial tachycardia
- Atrial flutter
208
Short RP regular narrow complex tachycardia?
1. Typical AVNRT
2. Orthodromic SVT
3. Atrial tachycardia with AV block
4. Junctional tachycardia with 1:1 retrograde activation
209
Contradictions to exercise testing?
- Severe AS
- Recent MI or RF
- Severe pulmonary hypertension
210
Chief determinant of symptoms in primum ASD?
Degree of left AVVR
211
Risk of IE coincides with what abnormality of a valve?
Degree of regurgitation (not structure)
212
Paradoxical emboli are most common with what type of ASD?
Secundum (preferential flow from IVC goes towards mid-septum
213
What is the incidence of CHD in patients with Trisomy 21?
50% of patients with Down Syndrome have CHD (most commonly ACSD - 2/3)
214
Lab criteria for Kawasaki?
- Albumin < or equal to 3g/dL
- Anemia for age
- Plts > or equal to 450,000/mm
- WBC > or equal to 15,000/mm
- Elevation of ALT
- Sterile pyuria > or equal to 10 WBCs/hpf
215
AHA IE guidelines?
- Prosthetic valve
- Prosthetic material used for valve repair
- History of IE
- Completely repaired CHD with prosthetic material or device for 6 months
- Residual defect at side of prosthetic device
- Valvulopathy in transplanted heart
- Unrepaired cyanotic CHD
- NOT needed for GI/GU procedures
216
Most common organism in endocarditis?
Alpha hemolytic strep (S. Viridans)
217
Second most common cause of endocarditis?
S. Aureus
218
PCN allergic IE prophylacis?
Cephalexin, clindamycin, azithromycin or clarithromycin
219
Most common cause of sudden death in athletes?
HCM
220
Marfan Gene?
FBN1
221
Loeys-Dietz gene?
- AD
- TGF Beta 1 or 2
- Aterial tortuosity, hypertelorism, bifid uvula, clef palate, craniosynostosis, aortic dilation/dissection, pectus excavatum/carinatum, scoliosis, joint laxity, arachnodactyly, talipes equinovarus
-LDS2: Velety smooth, translusent skin, overlaps of LDS1
222
Ehlers Danlos Gene?
COL1A1/A2
| -Loose joints, chronic join pain, early onset arthrtiis
223
ECG of ALCAPA?
Deep, wide Q waves in I/aVL/V3-V6
224
High pitched early diastolic murmur?
AI
225
Early low pitched decrescendo diastolic murmur?
PI
226
Diastolic, mid to late peaking, low-pitched murmur
MS or TS
227
Gold standard for diagnosis and classification of myocarditis
Endomyocardial biopsy
228
Jones Criteria?
Major:
- Joints: Migratory polyarthritis
- Carditis: New-onset murmur (pericarditis)
- Nodules: Subcutaneous
- Erythema marginatum (red ring on trunk and extremity)
- Sydenham chorea
Minor: Prolonged PR, elevated ESR/CRP/Temp, arthralgias
229
Most common symptoms in rheumatic heart disease?
Migratory polyarthritis
230
BP does what with inspiration and expiration?
- Decreases with inspiration
- Increases with expiration
-Pulsus Paradoxus: Exaggerated change in BP with inspiration (tamponade)
231
Describe MR seen in rheumatic heart disease?
Posterolateral direction of jet
| Prolapse of anterior leaflet
232
PVR is lowest at what lung volume?
Functional residual capacity
233
Which VSD types are highest risk for post-op heart block?
Canal
| Perimembranous
234
MOA of heparin?
Potentiates effects of anti-thrombin III (inhibits thrombin formation and prevents conversion of fibrinogen to fibrin)
235
MOA of streptokinase?
Converts plasminogen to plasmin (which then cleaves fibrin)
This is a "clot buster"
236
MOA of tPA?
Binds to fibrin converting plasminogen to plasmin
237
MOA for enoxaparin?
LMWH- Catalyzes ATIII formation and activation
238
Drug of choice for suicidal RV after pulmonary valvotomy?
Beta blockers
239
Cyclosporine and tacrolimus mechanism?
Inhibit calcineurin,
| Decrease T-call activation
240
Major side effect of calcineurin inhibitors?
Nephrotoxicity
HTN
Gingival hyperplasia
Hirsuitism
241
Most common lipoprotein disorder in childhood?
Familial combined hyperlipidemia
242
MOA of sirolimus?
Antiproliferative
| Decrease T cell production
243
Anti-coagulation following mechanical mitral valve?
Warfarin (2.5-3.5) and ASA
244
Anticoagulation after AVR?
- Mechanical: Coumadin for INR 2-3 + low dose ASA
- If RF, increase INR to 2.5-3.5
- Bioprosthetic: ASA 75-100mg
- If RF, coumadin for INR 2-3
245
Most likely rhythm with an irregularly irregular wide complex rhythm in an otherwise health person?
- Pre-excited a-fib
- Avoid AV blocking agents (digoxin, Ca channel blockers, adenosine)
- Cardioversion is best option, amiodarone if medication used
246
Treatment of choice for CPVT VT/VF storm?
IV beta blocker
247
What type of AV block can be seen with long QT?
2:1 AV block
248
Most common indication for reoperation in truncus arteriosus?
RV-PA conduit failure
249
Where does the AV node course in membranous VSD?
Posterior-inferior rim
250
Double chambered RV typically seen with what type of VSD?
- Perimembranous
| - High association with discrete subaortic stenosis
251
Most common indication for re-operation in partial AVC defect?
- Left AVVR
| - LVOTO more common with partial AVCD
252
Deficiency of which rim of the ASD is an indication for surgical repair?
Posterior-inferior
-Also other abnormalities which would benefit from intracardiac repair (TR)
253
Which deficient ASD rim is most associated with erosions?
Retro-aortic (anterior/superior)
254
What is recommended following ASD device closure?
ASA + SBE ppx x6 months
255
What is recommended following PDA device closure?
SBE ppx x6 months
256
Conduit size needed for Melody valve?
16mm or greater
257
What is the AV node supplied by?
RCA
258
Embryological origin of the CS?
1. Left horn of sinus venosus
| 2. Proximal left common cardinal vein
259
What course of left main from right sinus is associated with sudden death?
If it passes between aorta and PA
260
LAD from right sinus is commonly seen in what heart defect?
-ToF (usually passes in front of RVOT)
261
Most frequently seen post-operative complications after atrial switch?
- Baffle leaks (10-20%)
- SVC obstruction (5-10%)
- IVC obstruction (1%)
262
Most common reason for re-operation following Ross procedure?
Pulmonary homograft failure
263
Most common indication for re-operation in complete AVCD?
MR
264
Strongest predictor for development of post-op MR following CAVC repair?
Post-op severe MR
265
What lab test need regularly checked while taking amiodarone?
LFTs, PFTs, TFTs
266
Most common short term complication following arterial switch procedure?
Supravalvar PS (LeCompte maneuver reduces incidence)
267
Most common long term complication in repaired Scimitar?
PV obstruction
268
Strongest predictor of CVA in Eisenmenger?
Microcytosis from Fe deficiency
269
Which syndrome is associated with hypertelorism and bifid uvula?
Loeys-DIetz
-Treat with ARBs
270
IPAH is associated with what gene abnormality?
BMPR2
271
What lab monitoring should be performed with endothelin antagonist?
Liver function
272
Which side is PA absent in ToF v. Truncus?
```
ToF = Opposite arch
Truncus = Same as arch
```
273
Most common associated lesions with Ebsteins?
1. ASD
2. PFO
3. VSD
274
1st aortic arch gives rise to?
Part of maxillary artery (branch of the external carotid)
275
2nd aortic arch gives rise to?
Stapedial and hyoid artery (facial arteries)
276
3rd aortic arch gives rise to?
Common carotid and proximal part of internal carotid
277
5th aortic arch gives rise to?
Nothing- it regresses
278
Phase 0 of AP?
Rapid depolarization, Na rapidly into cell, Ca slowly into cell
279
Phase 1 of AP?
Repolarization, inactivation of Na channels and opening of few K channels (K out)
280
Phase 2 of AP?
Ca in... Repolarization is slowed by influx of Ca via L-type Ca (slow) channels balancing theoutward K current (some Na involved in creating plateau)
281
Phase 3 of AP?
K out... Rapid repolarization
282
Phase 4 of AP?
Resting potential, K in
283
Where is I-band in sarcomere?
Light band, made up of actin and thin filaments
Z: Line in the center
284
Where is A-band in sarcomere?
Dark band, made of myosin and thick filaments which may overlap with actin filaments (has both myosin and actin)
285
What is H-zone in sarcomere?
Zone of myosin filaments only (no overlap with actin filaments) within the A-band
-The lighter stripe in the center of the A-band
286
Where is the M line in a sarcomere?
Middle of the H-band, myosin only
287
Describe T-tubules of sarcomere?
- Envelope the myofibril at the level of the Z disk forming couplings with the SR
- Allows transmission of teh AP to cellular interior
288
What embryological structure does the SVC arise from
Right common cardinal vein and anterior cardinal vein
289
What embryological structure does the outflow tract of the LV arise from?
Bulbus cordis
290
What fetal structures have the lowest O2 saturation?
CS and SVC
291
Where is the AV node in L-TGA?
- Anterior aspect of AV ring near atrial septum
| - Anterior to VSD if present
292
Where is AV node located in CAVC?
- Posterior to VSD
| - Inferior to CS
293
Where is AV node located in tricuspid atresia?
- Posterior to VSD
| - Floor of RA
294
Where is AV node located in DILV?
Anterior to AV ring
295
Hypokalemia changes?
- U wave
| - Prolonged QTc
296
Hyperkalemia changes?
- Peaked T-waves
- Prolonged QRS
- AV block
297
Characteristics of manhaim tachycardia?
Delta wave with normal PR interval
298
Hypokalemia increases arrhythmogenic effects?
Digoxin
299
Broad based T waves seen in long QT type?
LQT1
300
Low amplitude or notched T waves seen in long QT type?
LQT2
301
Long ST segment seen in long QT type?
LQT3
302
Exertion or swimming related sudden death?
LQT1
303
Auditory stimulation or postpartum sudden death seen in?
LQT2
304
Sleep related sudden death seen in?
LQT3
305
Beta blockers most effective in LQT...?
LQT1
306
Hyperthyroidism can cause what arrhythmia?
A-fib
307
What AH jump is associated with dual AV node physiology?
> 50msec
308
Short HV interval seen in?
Accessory pathways <25-35msec
309
What type of VSD must be there for straddling tricuspid valve and straddling mitral valve?
Tricuspid: Inlet
Mitral: Malalinged
310
Short PR interval seen with?
1. WPW
2. Glycogen storage disease
3. Normal
311
WPW seen commonly in waht CHD?
Ebsteins
L-TGA
HCM
312
Describe absence of a pulmonary artery in relationship to arch sidedness in truncus v. ToF
- Truncus: Same side as arch
| - ToF: Opposite side of arch
313
Ductus commonly absent in what CHD?
1. Truncus
| 2. ToF-Absent pulmonary valve
314
Main reason for reoperation in truncus?
Conduit replacement
315
What gradient and RV pressure is considered severe conduit stenosis?
> 50mmHg
| RVSP >75% systemic
316
ToF with AVSD is seen in what syndrome?
Trisomy 21
317
Most common cardiac tumor in children?
Rhabdomyoma
318
What cardiac tumor shows delayed enhancement on CMR?
FIbroma
319
Single, friable, pedunculated mass within LA? Treatment?
Myxoma, Resection
320
Multiple, well circumscribed, echo bright lesions seen within the ventricular myocardium are most likely?
Rhabdomyomas
321
What genetic syndrome should be ruled out in the presence of multiple ventricular tumors?
Tuberous sclerosis
322
"Spider cells" are found with which cardiac tumor?
Rhabdomyomas
323
Single, firm, intramural tumor seen within LV free wall or interventricular septum?
Fibroma (usually don't regress)
324
Cardia myxomas are associated with what?
Multiple lentigines syndrome
325
Most frequent malignant primary cardiac tumor?
Angiosarcoma - Hemorrhagic effusion seen
326
Coronary artery fistulas are most likely to empty where?
RV
327
What septal thickness is associated with sudden death in HOCM
> 30mm
328
Cyanosis and dyspnea on exertion in a Fontan when standing upright is concerning for?
Pulmonary AVM
329
LAD in newborn raises suspicion for?
- Tricuspid atresia
| - AVCD (superior axis)
330
Treatment for mild-mod and severe carditis in acute rhaumatic fever?
- High dose ASA for 4-6 weeks
| - Steroids for 2 weeks then taper and add ASA is severe
331
Norepinephrine MOA is predominantly?
- Alpha agonist - Vasoconstriction
| - Very little beta
332
Dobutamine MOA?
- Mostly beta 1
| - CO increases, SVR decreases
333
Left pressures > Right pressures?
Restrictive cardiomyopathy
334
RVSP > 50mmHg
Restrictive cardiomyopathy
335
LV pressure = RV pressure?
Constrictive cardiomyopathy
336
RVSP < 50mmHg
Constrictive cardiomyopathy
337
Pulmonary venous baffle obstruction more common after?
Senning
338
Systemic baffle obstruction more common after?
Mustard
339
Cyanosis following hybrid procedure most likely caused by?
Restrictive atrial septum
340
Most common associated defect with congenital absence of pericardium?
ASD
341
Most common VSD in DORV?
Subaortic
342
Estimated rate of erosion of ASD devices?
1/1000
343
Type 1/Alpha error?
- Incorrectly rejecting the null hypothesis
| - Saying there is a difference when there isn't
344
Type 2/Beta error?
- Incorrectly accepting the null hypothesis
| - Saying there is no difference when there is
345
First line therapy for idiopathic pulmonary HTN?
Ca channel blockers
346
Doppler gradients for PS?
Mild <40
Moderate 40-60
Severe > 60
347
Cath based gradient for coarctation intervention?
> 20mmHg
348
Most common associated defect with Ebstein?
ASD
349
Second most common associated defect with Ebstein's
PS
350
Clefts in mitral valve are in what leaflet?
Anterior
351
Embryology of normal left arch
- Left dorsal, left 4th, left 6th, right 4th
| - No right 6th or right dorsal
352
Embryology of right aortic arch with mirror image branching?
- Right dorsal, left 4th, left 6th, right 4th
| - No right 6th or left dorsal
353
Embryology of left arch with aberrant right subclavian?
- Left dorsal, left 4th, left 6th
| - No right dorsal, right 4th or right 6th
354
Embryology of right aortic arch with aberrant left subclavian/diverticulum of Komerell?
- Right dorsal, right 4th, left 6th
| - No left dorsal, left 4th
