2. Malformations Flashcards
(419 cards)
1
Q
Inheritance of Alagille?
A
AD
2
Q
- Liver disease (bile duct paucity)
- Cholestasis
- CHD
- Skeletal abnormalities
- Ocular abnormalities
- Broad forehead, pointed chin
A
Alagille
3
Q
What kind of cardiac lesions are seen in Alagille?
A
Right-sided lesions
- Peripheral PS
- PS
- ToF
*Can see left-sided lesions and septal defects too
4
Q
Notch ligand
JAG1
A
Alagille
5
Q
NOTCH1
A
AoV pathology
6
Q
PTPN11
A
Noonan
7
Q
Hypertelorism, ptosis, short stature, webbed neck, skeletal anomalies, bleeding diathesis, lymphatic disorders, mental retardation, cryptorchidism, CHD
A
Noonan
8
Q
Types of CHD in Noonan
A
HCM PV stenosis ASD (primum/secundum) VSD ToF PA stenosis CoA Partial AVSD Polyvalvulopathy
9
Q
TBX1
A
DiGeorge
10
Q
Hypocalcemia, immunodeficiency, CHD
A
DiGeorge
11
Q
Types of CHD seen in DiGeorge?
A
IAA-Type B
Truncus
ToF
12
Q
GATA4
A
Septation defects
13
Q
Rubella in 1st trimester increases fetal risk for what CHD?
A
- PS (valvar, supravalvar, peripheral)
- PDA
*ToF also reported
14
Q
True or False: Excessive caffeine during pregnancy increases risk for CHD
A
False
15
Q
CHD associated with maternal PKU?
A
- Left-sided defects
- Septal defects
- ToF
16
Q
- Microcephaly
- Mental retardation
- High phenylalanine
A
PKU
17
Q
7q11.23 deletion
A
Williams
18
Q
Hypercalcemia, skeletal and renal anomalies, cognitive deficits, social, “elfin” facies
A
Williams
19
Q
Types of CHD seen in Williams?
A
- Supravalvar AS
2. Supravalvar PS
20
Q
3/6 SEM, friendly, stellate irises, long philtrum, depressed nasal bridge, prominent lower lip, enamel hypoplasia
A
Williams
21
Q
How is the deletion associated with Williams detected?
A
FISH (not by routine karyotype)
*7q11.23 deletion
22
Q
In Williams, does the supravalvar PS or AS tend to improve with time?
A
PS improves with time
AS usually progresses
23
Q
True or False: SCD has been described in Williams
A
True
*Suspected causes include coronary artery stenosis and severe biventricular outflow tract obstruction (myocardial ischemia, decreased CO, arrhythmia)
24
Q
Why are patients with William’s syndrome prone to develop HTN?
A
Renal artery stenosis
25
Growth retardation, short palpebral fissures, small mouth, micrognathia, prominent occiput, short sternum, small nipples, clenched hands, disorganized/hypoplastic palmar creases, hyperconvex nails, rocker bottom feet
T18
26
What is the % incidence of CHD in T18?
>90%
27
Most common CHD in T18?
1. Perimembranous VSD
2. ToF
3. DORV
4. Polyvalvular dysplasia
28
What % of children with T18 die within 1st year of life?
90%
*Usually not from heart disease
29
Most common CHD associated with 22q11 deletion?
1. ToF
2. IAA Type B
3. Truncus
4. Perimembranous VSD
5. Aortic arch anomalies
*Wide range however that can include PS, ASD, heterotaxy, HLHS
30
What can develop is a large ASD is left unrepaired?
Severe irreversible pulmonary HTN
31
In which gender is there a higher preponderance for the development of pulmonary HTN from an unrepaired ASD?
Female
32
Above what diameter ASD is spontaneous closure rare?
>8mm
33
What may need to be done prior to repair of any type of ASD that is not discovered until adolescence or adulthood?
Cath to assess for pulmonary HTN and vasoreactivity
34
Sinus venosus ASDs
- 5-10% of ASDs
- Posterior/Superior to fossa ovalis
- Commonly associated with anomalous connection of R pulmonary veins to either RA or SVC near caval-atrial junction
35
What is seen on ECG for a sinus venosus ASD?
Frontal plane P-wave axis <30 degrees (up to 1/2 patients)
36
Which types of ASDs have a normal P-wave axis?
1. Secundum
2. Primum
3. PFO
4. Unroofed CS
*Sinus venosus ASDs can have
37
In which defect are the papillary muscles closer together with the anterior muscle being closer to septum than normal and posterior muscle being further from the septum than normal?
Complete AV canal defect
38
How many leaflets does a common AV valve have?
5
*Beneath 5 commissures are 5 papillary muscles
39
In a complete AVCD are the left sided papillary muscles closer together or further apart as compared to a normal heart?
Closer
40
Which AV valve leaflet is smaller than normal in a complete AVCD?
The left lateral
41
How are the left sided papillary muscles in a complete AVCD rotated and what effect does this have on their position?
Counterclockwise
- Posterior further from septum
- Anterior closer to septum
42
What is the potential result of the change in left sided papillary muscle arrangement in complete AVCD?
Progressive LVOT obstruction
| also the large anterolateral muscle bundle
43
Components of a partial AVSD?
1. Primum ASD
| 2. Cleft anterior MV leaflet
44
True or False: A primum ASD results in earlier and more severe symptoms as compared to a secundum ASD
True
45
What is the most common reason for reoperation in partial AVSD?
Repair of recurrent or residual MR/MS
46
What do you need to think about in a patient with AVSD and a SEM?
LVOTO
47
Progressive LVOT obstruction is more common in patients with what type of AVSD?
Partial- 2 AV valve orifices
48
What is different about the aortic valve in AVSD?
Displaced anteriorly (versus being wedged between mitral/tricuspid annuli)
- Creates elongated/gooseneck deformity of LVOT
- LVOT obstruction can happen in any form of AVSD
49
Is LVOTO more common in AVSD with a common AV valve or 2 AV valve orifices?
Common
50
What % of patients with AVSD require reoperation for LVOTO?
10%
51
List mechanisms for progressive LVOTO in AVSD?
- Attachments of superior bridging leaflet into ventricular septum
- Extension of anterolateral papillary muscle into LVOT
- Discrete fibrous subaortic stenosis
- Tissue from aneurysm of membranous septum bowing into LVOT
52
Post-op from ASD/VSD repair hearing widely split S2 and no murmur... what is the cause?
RBBB
53
True or False: Prominent splitting of S2 can be heard with a small VSD
True
-Usually normal, but can have this sometimes
54
What should you consider in a VSD when you have systolic murmur that transmits to the LUSB or Apex?
LUSB: PS
Apex: MR
55
What limits flow across a large/unrestrictive VSD?
Relative resistances of systemic/pulmonary circulations
56
What anomalies of the tricuspid valve can be seen in a perimembranous VSD with L-R shunting?
Redundant septal leaflet tissue (this can partially or completely occlude the defect)
57
What happens to LV mass and volume after a VSD repair?
Decrease
| Volume decreases at a greater rate than mass
58
Patients who develop Eisenmenger physiology begin manifesting cyanosis before what age?
2
59
What can happen to the aortic valve with an unrepaired outlet or perimembranous VSD?
Prolapse of one of the aortic cusps
-Leaflet partially closes the VSD and eliminates shunting, but the AI is progressive
60
What can be seen in an unrepaired outlet VSD?
- Deficiency of muscular/fibrous supports below the AoV with herniation of the right coronary leaflet through the VSD
- Aortic commissures are usually normal
- Results in aortic insufficiency
61
What can be seen in an unrepaired perimembranous VSD?
- Herniation of the right (or less commonly non) coronary cusp
- Frequent abnormalities of aortic commissures (usually right/non)
- Results in aortic insufficiency
- May have associated infundibular PS
62
Relationship of the bundle of His to an inlet VSD?
Bundle passes anterior-superior to defect
63
Relationship of perimembranous VSD to bundle of His?
Bundle of His is subendocarial and courses along the posterior-inferior margin of the defect
64
Where is the conduction tissue in relation to a muscular or outlet VSD?
Usually far removed unless the defect extends into the perimembranous area.
65
NEC + PDA
Surgical closure of PDA
66
Potential complication of unrepaired PDA?
Endarteritis or endocarditis
*PDA can account for up to 15% of all endocarditis cases
67
Most common organisms for endocarditis with PDA?
S. Viridans
| S. Aureus
68
Where are vegetations most commonly seen in a PDA?
PA end of the duct
69
Which event is most responsible for early, functional closure of ductus arteriosus?
Medial smooth muscle cell migration in the wall of the ductus
70
When do the 2 phases of post-natal closure of the PDA occur?
1st: Within 12 hours after birth
2nd: 2-3 weeks
71
Describe the 2 phases of post-natal closure of the PDA
- 1st: Contraction and cellular migration of the medial smooth muscle in the wall of the ductus arteriosus that causes the vessel walls to become thick and protrude into the vessel lumen
- 2nd: Infolding of the endothelium, disruption and fragmentation of the internal elastic lamina, proliferation of the subintimal layers, and hemorrhage and necrosis in the subintimal region. There is connective tissue formation and replacement of muscle fibers with fibrosis with subsequent permanent sealing of the lumen… forms the ligamentum arteriosum
72
Theoretical benefits of ibuprofen over indomethacin for closure of PDA in a premature infant?
- Similar rates of ductal closure
| - Fewer negative effects on renal function, cerebral vasculature and cerebral blood flow
73
Which has higher risk of intraventricular hemorrhage in PDA closure... indomethacin or ibuprofen?
Equivocal
74
What is the use of ibuprofen as prophylaxis for PDA closure associated with an increased risk of?
Pulmonary HTN
75
What is the standard surgical approach for ALCAPA?
Direct reimplantation of the origin of the LCA into the aorta
76
What is an alternative approach for repair of ALCAPA?
Takeuchi
-AP window created and a tunnel created that directs blood from aorta to left coronary ostium
77
What is a risk factor for mortality and late reoperation in ALCAPA?
Mitral insufficiency
*Due to papillary muscle infarction and dysfunction
78
What determines specific features in a sinus of Valsalva fistula from the aorta to the RA?
Site of entry to heart
*Localized weakness in wall of sinus of Valsalva causes aneurysmal bulging... if this ruptures, size of fistula determines how large shunt will be
79
What type of shunting results from a sinus of Valsalva fistula emptying into the left heart v. right?
Right: Right to left shunt
Left: No shunt (O2 blood back to LV)
80
Describe the murmur in a small sinus of Valsalva fistula
- Continuous murmur
- Maximal intensity in the 3rd or 4th intercostal space near the sternal edge
- If fistula enters RA, murmur may be maximal to right of sternum
81
Describe exam findings in a large sinus of Valsalva fistula
- Wide pulse pressure
- Collapsing pulse
- LVH
- If fistula enters right side, there will be RV hyperactivity
- If fistula enters LV, may have a to-and-fro murmur and simulate aortic incompetence
- Occasionally, there is only a diastolic murmur in fistulae entering the LV (or the high pressure RV in neonate)
82
Which sinus does the coronary in ALCAPA usually arise from?
Left posterior facing sinus on PA
83
Describe pathophysiology in ALCAPA
- In fetal life, pressures and oxygen saturations are similar in the aorta and pulmonary artery so myocardial perfusion is normal
- After birth, PAs have low pressures and desaturated blood so myocardial perfusion is compromised
- Myocardial ischemia subsequently occurs and this is worsened with exertion in feeding or crying
- As time passes, infarction of the anterolateral LV free wall occurs. Mital valve papillary muscles are affected and MR develops
84
True or False: Anomalous coronary artery origins from the wrong sinus of Valsalva are generally asymptomatic in infancy?
True
85
What is the most common coronary artery anomaly?
Origin of left circumflex from right main (1/3 of all major coronary arterial anomalies)
86
True or False: A left circumflex from the right coronary artery has no general clinical significance in the absence of intracardiac surgery
True
87
Why is an origin of LCA from the right sinus of Valsalva important clinically?
- If passes between aorta and RVOT, risk for sudden death during or just after vigorous exercise
- Many cases, ostium of the LCA is slit-like… increases risk further
88
What is often seen with aneurysms in the sinus of Valsalva?
VSD (50% of the time)
*Especially right sinus aneurysms associated with defects of the outlet septum
89
What causes an aneurysm of the sinus of Valsalva?
- Localized weakness of the wall leads to aneurysmal bulging
- Localized aneurysms are usually congenital with thinning just above the annulus at the leaflet hinge
- Can follow IE
90
Which gender is more affected by aneurysm of the sinus of Valsalva?
Male (75%)
91
Where are sinus of Valsalva aneurysms more frequently located?
- 65% located in right aortic sinus
- 25% in non-coronary sinus
- 10% in left aortic sinus
92
What circumstance is most common for rupture of sinus of Valsalva aneurysm?
-Rupture of right sinus into RV in setting of an outlet VSD
- Can rupture into any chamber
- Rupture into pericardium is rare
93
What is risk of mortality in 1st week of like with cerebral AVM causing high-output cardiac failure?
90%
*From intractable CHF or neurologic complications (seizures, intracranial hemorrhage)
94
Central nervous system AVMs manifest symptoms according to what?
Hemodynamic effect
95
What are the most common cerebral AVMs to present with CHF?
1. Deep (vein of Galen)
2. Superficial (pial)
3. Dural
96
What is seen in infants with significant cerebral AVMs?
1. High-output CHF
| 2. Dilation of all cardiac chambers, feeding arteries and draining veins
97
What happens if there is venous obstruction in a cerebral AVM?
Flow can be restricted... patient may present with venous HTN or cerebral ischemia
98
If an infant with a significant cerebral AVM survives, what are long-term problems?
Neurologic morbidity- Hydrocephalus, mental retardation, hemorrhage
99
If you have multiple pulmonary AVMs, there is a >80% of having what?
HHT
* Most pulmonary AVMs are congenital or associated with HHT
* Overall 30-50% of patients with pulmonary AVMs have HHT
100
What happens to pulmonary AVMs as a child grows?
Enlarge
101
What type of AVMs have increased CO?
Systemic
102
What happens to pulmonary blood flow and pressures with a pulmonary AVM?
Unchanged
*PVR normal, resistance within AVM is low, resistance in other lung segments may be elevated
103
What is the treatment of choice for a pulmonary AVM?
Transcatheter embolization
* Goal is to raise systemic arterial oxygen tension by occluding most significant afferent arteries (>3mm diameter)
* Improves hypoxia, resolves orthodexia
* Minimal growth of small remaining AVMs
104
Embolization of pulmonary AVMs prevents what?
1. Stroke
2. Tia
*DOES NOT reduce risk of brain abscesses
105
What is done to avoid device embolization when closing a pulmonary AVM?
-Using a coil or umbrella versus liquid adhesive or beads
106
What measurement has best potential to distinguish a large AVM from a large PDA in a young infant?
Systemic vein oxygen saturation during cath
107
In what ways are large AVMs and large PDAs similar?
1. Hemodynamic effects (large extracardica L-R shunts)
| 2. Same changes in pulse pressure, liver span, CT ratio on CXR and QRS axis on ECG
108
What does a cath for a large AVM show?
1. High CO
2. Elevated atrial and ventricular end-diastolic pressures
3. Widened systemic arterial pulse pressure
4. Large difference in the oxygen saturation between the SVC and IVC (higher sat from involved area)
109
Barium esophagram with anterior indentation?
- Pulmonary artery sling
| - Tumor
110
Describe the anatomy in a pulmonary sling
- Origin of LPA from RPA
- LPA arises as a very proximal branch of the RPA and loops around the trachea
- Only situation in which a major vascular structure passes between the tracheal and esophagus
- Rare
- Usually isolated abnormality, but can be associated with other CHD (ToF)
111
What tracheal anomaly is a pulmonary sling frequently associated with?
-Complete cartilaginous rings in the distal trachea resulting in tracheal stenosis
112
Describe a right aortic arch with mirror image branching
- Traverses right mainstem bronchus
- 1st branch left innominate (divides into left carotid and left subclavian
- 2nd branch is right carotid
- 3rd branch is right subclavian
- Duct usually on left and arises from base of innominate artery
- Doesn't typically form a vascular ring
113
What should be done if a right aortic arch is noted CT imaging?
- Echo to assess for CHD
| - Commonly associated with ToF, but also other conotruncal abnormalities (DORV, etc.)
114
True or False: A right aortic arch with retroesophageal innominate artery, left PDA results in a vascular ring?
True
115
What is a vascular ring?
Aortic arch anomaly in which trachea and esophagus are completely surrounded by vascular structures
116
Symptoms of a vascular ring?
- Respiratory: Stridor, PNA, bronchitis, cough, neck hyperextension
- Swallowing difficulty (less common, usually toddlers or older children)
117
Absent limb pulses in all extremities, but strong carotid pulses bilaterally?
IAA type B + Anomalous subclavian
118
Describe the anatomy of type A, B, C IAA
```
IAA = Complete separation of ascending and descending aorta
A = Interruption distal to left subclavian
B = Interruption between carotid and subclavian
C = Between carotid arteries
```
119
How to differentiate critical AS v. IAA type B + Anomalous subclavian?
- IAA type B + Anomalous subclavian will have strong carotid pulses
- Critical AS will have all weak pulses
120
What is the most common vascular ring?
Double aortic arch
121
What is the second most common vascular ring?
Right arch with diverticulum of Kommerell
122
True or False: A right aortic arch with mirror image branching usually causes no ring
True
*Unless there is a left duct
123
What is the most common aortic arch abnormality?
Left arch with retroesophageal right subclavian
*Doesn't form a ring and usually asymptomatic
124
True or False: Right aortic arch with retroesophageal innominate artery doesn't create a vascular ring
False
* Very rare
* Ductus completes vascular ring (connects LPA with base of innominate)
125
Describe cor triatriatum
- Membrane separates more proximal chamber which receives the pulmonary veins from the more distal LA which communicates with the mitral valve
- Typically a hole in the membrane ranging from <3mm to 1cm
- Distal (true) LA is in continuity with the LAA
- Fossa ovalis is usually between the distal LA and RA
- Occasionally, a PFO/ASD in present in this area
- RVH and RV dilation are almost invariably found
- RAH and dilation are present in < 25% of cases
- Hypertrophy and dilation of RA results in tall, broad and oftentimes peaked P waves on ECG
126
What 2 things is prognosis in TAPVC influenced by?
- Size of interatrial communication
| - Degree of obstruction in anomalous venous pathways
127
What is the long-term prognosis in TAPVC dependent on?
- State of pulmonary vascular bed at time of surgery
- Patency of pulmonary venous-LA anastomosis
*Overall mortality for unrepaired 80% + at one year
128
What arrythmias are most common to develop in repaired TAPVC?
Atrial: Sinus bradycardia, atrial flutter, SVT
*Ventricular arrhythmias unusual
129
Describe Scimitar syndrome
- Anomalous connection of the right pulmonary veins to the IVC
- Creates a crescent-like shadow in the right lower lung field which resembles a Turkish sword (Scimitar)
130
What are frequent coexistent anomalies in Scimitar?
- Hypoplasia of right lung/chest
- Mesocardia or dextrocardia
- Lung parenchymal abnormalities
131
What causes a superior sinus venosus ASD?
Deficiency of the common wall of the SVC and pulmonary vein (RUPV)
* Defct "unroofs" the RUPV
* Unroofed pulmonary vein drains into the SVC and LA orifice becomes an interatrial communication (isn't actually a defect of atrial septum)
132
What should be done for infradiaphragmatic TAPVC?
Surgery ASAP
* If sick, optimize as able
* In past BAS was used as palliation, but just delays definitive procedure and not recommended
* Balloon dilation of obstructed venous channel not typically successful
133
What is the most common site of obstruction in infradiaphragmatic TAPVC?
-Anomalous vessels' connection with portal vein or hepatic veins
134
If unobstructed, what does flow look like in the anomalous vessel in infradiaphragmatic TAPVC?
- Low-velocity, phasic laminar flow pattern with brief flow reversal during atrial systole
- Luminal narrowing is associated with flow acceleration and turbulence by color Doppler
135
When do symptoms from cor triatriatum present?
Usually in 1st few years of life, but some can present in 2-3rd decade of life
136
What are common late presenting symptoms of cor triatriatum?
- Dyspnea
- Frequent respiratory issues (asthma)
- PNA
*Usually thought to have primary pulmonary disease
137
Untreated cor triatiatum results in what?
Pulmonary HTN
138
Exam findings of cor triatriatum?
- Loud pulmonary component of 2nd heart sound
- RV heave
- Pulmonary systolic ejection click
- TR murmur can be present
- Less often, a diastolic murmur is detected at the mitral area or a continuous murmur can be heard
- Right heart failure common
- Rales heard if pulmonary edema present
139
True or False: Patients with pulmonary edema or right heart failure due to cor triatiatum often have progressive disease despite maximal medical management
True
140
What should be done for someone with cor triatriatum and pulmonary edema or right heart failure?
Surgery ASAP
*Resection of membrane under bypass
141
How long is survival with cor triatriatum with pulmonary edema and right heart failure if untreated?
Months
*If survive operative correction, severe PA changes which cause pulmonary HTN can regress... for these patients, prognosis is excellent
142
What is an interrupted IVC?
Absence of the hepatic segment of the IVC with azygous continuation to the right or left SVC
143
What is a factor that could cause hypoxia in a patient with interrupted IVC with azygous continuation and bilateral Glenn shunts years following surgrey?
Pulmonary AVMs
*Can develop after Glenn due to exclusion of hepatic factor to the lungs (may be 1 or both lungs pending on preferential blood flow)
144
What does a vertical vein represent?
A persistent embryologic connection between the splanchnic plexus of the lung buds and the cardinal veins
145
Besides PAPVR to the right SVC (sinus venosus defect) and RA (malposition of the septum primum) what is the most common type of PAPVC?
Left pulmonary veins to the left innominate vein
146
What is the name of the vein that connects the left sided pulmonary veins to the left innominate vein in PAPVR?
Vertical vein
147
Where anatomically is a vertical vein compared to a L-SVC?
Vertical vein usually more posterior than L-SVC (which is immediately behind the LAA)
148
If the left pulmonary veins drain to the L-SVC, what should the L-SVC drain to?
Still CS or LA
149
What type of ASD is common with PAPVC to the left innominate vein?
Secundum
*Primum uncommon and rarely is septum intact
150
Congenital malformations of the CS are frequently associated with what?
Arrhythmias
151
What is reported in a significant % of patients with diverticular of the CS?
SVT and SCD
152
What causes SVT in a patient with diverticulum of the CS?
Accessory pathway that traverses the diverticulum to form an AV connection
153
2 week old with tricuspid atresia, d-TGA, moderately restrictive VSD, moderately hypoplastic arch, acidotic... best surgical approach?
DKS with arch augmentation and BTT shunt
154
Are ventricular endocardial potentials recorded past the expected anatomic tricuspid valve annulus during EP assessment more specific for Uhl or Ebstein?
Uhl
155
What is a congenital cardiac malformation that consists of an almost total absence of the RV myocardium?
Uhl
156
Physical exam consistent with Uhl?
- Cyanosis
- Hepatosplenomegaly
- JVD
- Quiet precordium
- Diminished peripheral pulses
- Decreased heart tones
- Pansystolic TR murmur (also can be no murmur or non-specific murmur)
157
What is seen on ECG in Uhl's?
- Prominent P waves
| - Diminished QRS amplitude (especially in the right precordial leads)
158
What is seen on CXR in Uhl's?
- Cardiomegaly
- Normal to diminished pulmonary vascularity
*Similar to Ebstein
159
What is seen on Echo in Uhl's?
Marked right sided dilation
160
Describe the PA, RA, RV pressure waveforms in Uhl's
Similar to each other
161
What is dominant in the RA waveform in Uhl's?
A-wave
162
What is seen on endocardial potentials in Uhl's?
Normal transition between the ventricular and atrial complexes
*This helps rule out Ebsteins
163
True or False: Many patients with Uhl's anomaly die in infancy or childhood?
True
164
Markedly dilated, "parchment-like" RV with thickened endocardium, few if any true myocardial cells in the RV free wall, tricuspid valve arises normally from dilated valve annulus and might be dysplastic (but not displaced into RV cavity)
Uhl's Anomaly
165
True or False: With normal CO, the severity of PS is based on measurements of RV pressure and valve gradient?
True
166
What constitutes mild PS?
RV pressure < LV pressure or
| Peak valve gradient <35-40mmHg
167
What constitutes moderate PS?
RV pressure 50-75% of LV pressure or
| Peak valve gradient 40-60mmHg
168
What constitutes severe PS?
RV pressure >75% of LV pressure or
| Peak valve gradient 60-70mmHg
169
What is the modified Bernoulli equation?
[4(V2^2 - V1^2)]
170
What is expected in a neonate after balloon valvuloplasty for critical PS?
- May need PGE for a few weeks to allow for RV compliance to improve and atrial R-L shunting to decrease (most neonates demonstrate improvement over weeks to months)
- Intermittently assess if ductal closure is tolerated with SpO2 >70%
*If they ultimately need a shunt, can either get surgical shunt or ductal stent
171
What often causes desaturation following balloon valvuloplasty for critical PS?
Infundibular RVOTO
- With PS, get secondary changes in RV and PAs
- Infundibulum becomes hypertrophied and get dynamic subvalvular obstruction
- Hypertrophy persists immediately post-valvuloplasty which causes limited pulmonary outflow
- Over time, hypertrophy improves once fixed pulmonary obstruction removed
172
True or False: With unilateral branch PS without significant L-R shunting, the resting RVSP will remain normal?
True
*Contralateral PA accommodates CO w/o increase in pressure
173
What could be underestimated in unilateral branch PS?
Severity of obstruction by systolic pressure difference estimation
*Because flow to stenotic side is lower than normal
174
If doing a balloon angioplasty for unilateral branch PS, what size should the balloon be?
3-4 times the narrowest PA segment
*Position the balloon dilation catheter across the stenotic segment of the PA
175
True or False: Percutaneous balloon angioplasty of peripheral PA stenosis has a lower success rate than pulmonary valvuloplasty
True
*Acute success rate for branch PA angioplasty is <50-60% and rate of recurrent stenosis has been 15-20% in short to mid-term follow-up
176
What has to be done for a neonate with PA + IVS prior to any surgical intervention?
Cath + Angiography
*Need to confirm coronary circulation
177
Describe RV dependent coronary circulation as seen in PA + IVS
- Myocardium is supplied by blood that originates in RV at systemic or suprasystemic systolic pressure and perfuses myocardium in a retrograde fashion
- Myocardial ischemia, infarction and death can happen if significant ventriculocoronary connections are present and RV pressure is reduced following an intervention
178
In normal circulation, what drives coronary blood flow?
Aortic diastolic pressure
*Anything that reduces this will compromise coronary blood flow
179
What can happen to the coronary arteries if there are ventriculocoronary artery connections?
- Coronary artery stenosis
- Coronary interruption
*Aortic diastolic pressure may not be enough to drive coronary blood flow in this case and elevated RV pressures are needed to perfuse coronaries... In this case, interference with blood flow into the RV or other reduction of RVSP can be dangerous
180
What factor predicts a successful decompression of the RV using radiofrequency ablation and balloon pulmonary valvotomy in patients with PA + IVS?
Tricuspid valve Z-score < -2
181
What are the 2 options for PA + IVS if able to decompress?
- Transcatheter perforation of the atretic PV with subsequent balloon dilation
- Surgical valvotomy
182
What are good features for success of of RV decompression/bi-ventricular repair in PA + IVS?
1. Tripartite RV near normal in size
2. Valvar PA
3. Well-developed pulmonary arterial circulation
4. No RV-dependent coronary circulation
5. Tricuspid valve Z-score > -2
183
In PA + IVS what are the smallest RVs associated with?
Ventriculocoronary connections
*Unipartite or Bipartite ventricles much more likely to have ventriculocoronary communications
184
In PA-IVS, what is a more negative tricuspid Z-score correlated with?
Presence of ventriculocoronary connections
185
What cannot be done for a PA + IVS patient with RV-dependent coronary circulation?
Decompression of RV (RV-PA conduit or pulmonary valvotomy)
186
If you decompress a PA + IVS patient with RV-dependent coronary circulation, what will you see on ECG?
Myocardial ischemia (ST segment elevation) where the RV-dependent coronary circulation is located (regional distribution)
187
True or False: In PA + IVS, even if coronaries look okay by echo, do you still need to confirm no RV-dependent coronary circulation with cath/angiography
True
188
True or False: In PA + IVS, ventriculocoronary connections are seen in <45% of patients, but only <10% have wholly RV-dependent coronary circulation
True
189
True or False: In PA + IVS, there are often MAPCAs
False: Confluent PAs usually supplied by a left-sided PDA and MPA segment almost always present
*Rarely, non-confluent PAs are supported by bilateral PDA or AP collaterals
190
What are the 3 types of RV seen in PA + IVS?
1. Tripartite
2. Bipartite
3. Unipartite
191
True or False: PA + IVS is more common in males?
False- No gender predilection
192
What is the ideal goal for PA/VSD + MAPCAs?
Complete repair (close all septal defects, interrupt all extracardiac sources of PA blood flow, incorporate at least 14 PA segments in connection to RV) if PA anatomy can be reconstructed
* Connect RV to central PA with conduit
* Unifocalize to get max # of PA segments to eventual RV outflow reconstruction
* Close VSD
193
After attempted complete repair for PA/VSD + MAPCAs, what would necessitate VSD to be re-opened?
- If the RVSP >70% systemic
| - If central PA size is <50% normal
194
In PA-VSD, the blood supply to lungs is from what?
Arterial circulation
* May be a PDA, MAPCAs, coronary artery, plexuses of bronchial or pleural arteries
* Ductal and collateral sources can be present in the same patient, but rarely same lung
195
In PA-VSD, what is the caliber of the central PAs directly related to?
Amount of blood flow through that segment
* When PDA/collaterals connect proximally to the central PAs, they may be mildly hypoplastic or normal
* When multiple collateral arteries are present more distally, the central PAs are usually hypoplastic
196
In a patients with PA + VSD, where is proximal His bundle relative to the VSD?
Along the posterior-inferior rim of the VSD on the LV side
197
Describe the EP anatomy in PA + VSD
- Normal sinus node
- Normal AV node (in triangle of Koch)
- Non-branching proximal portion of His bundle penetrates the central fibrous body and lines along the LV aspect of the posterior-inferior rim of VSD
198
What is the main goal of management in a hypercyanotic spell?
1. Lower PVR
| 2. Increase SVR
199
List some options for management of a hypercyanotic spell
1. Supplemental O2
2. Volume (avoid dehydration)
3. B-blocker
4. Sedation (morphine or ketamine)
5. Vasopressors (phenylephrine)
6. Avoidance of anesthesia that decreases SVR more than PVR
* All of these help to decrease the ratio between pulmonary and systemic resistance
* Occasionally, emergent surgical palliation may be needed
200
What is a Waterston shunt?
Anastomosis of ascending aorta to RPA
201
What is a Potts shunt?
Anastomosis of descending aorta to LPA
202
What can a Waterston shunt or Potts shunt result in?
- PA distortion
| - Inconsistent transmission of flow/pressure to PA bed
203
What precludes the use of a Waterston shunt or Potts shunt?
- PA stenosis
| - Pulmonary vascular disease
204
What type of shunt should you consider a history of in a patient with a significant MPA to RPA gradient?
Waterston
205
In a neonate with ToF absent PV, what can be done to help with respiratory failure that isn't improving despite mechanical ventilation?
Prone- Suspend PAs off airway
206
What causes respiratory failure in neonates with ToF absent PV?
Central bronchial compression from massively dilated PAs
207
What physiology is a milder care of ToF absent PV similar to?
VSD (once PVR drops and L-R shunting increases)
208
What should be done for a baby with ToF who becomes cyanotic with no murmur at 3-4 days of life?
PGE- Baby is ductal dependent and you need to try to re-open PDA for pulmonary blood flow
*Once stabilized, consider complete repair v. shunt
209
True or False: A restrictive VSD is common in ToF?
False- Very uncommon
210
How is the outlet septum deviated in all patients with ToF?
Anterior and cephalad
211
The degree of what determines the severity of subpulmonary obstruction, size of VSD and degree of aortic override in patients with ToF?
Anterior/cephalad deviation of the outlet septum
212
What is present in virtually all patients with severe infundibular obstruction and ToF?
Large, non-restrictive VSD and prominent overriding aorta
213
A large conal branch or accessory LAD is seen in what % of ToF patients?
<15%
214
A L-SVC is found in what % of ToF patients?
<10%
215
A continuous murmur on exam is more suggestive of PA + VSD or Truncus?
PDA + VSD
216
What causes a continuous murmur in PA + VSD?
PDA or systemic collaterals to PAs
*Common
217
True or False: A continuous murmur is uncommon in truncus
True
218
What is the usual location of the coronary arteries in truncus?
- Left coronary from left posterolateral truncal surface
| - Right from right anterolateral surface
219
Describe coronary findings common in Truncus
- Usual coronaries: Left coronary from left posterolateral truncal surface, Right from right anterolateral surface
- LAD frequently relatively small and displaced leftward
- Conal branch of RCA usually prominent and supplies several large branches to the RVOT
- Posterior descending coronary artery arises from left circumflex (left-dominance) in 25-30%
- Coronary anomalies common (37-49%)
220
Why is early surgical correction needed in truncus patients?
They are at risk for developing pulmonary vascular obstructive disease early
221
What needs to be assessed in an older truncus patient before repair is considered?
PVR
222
Above what PVR would a truncus patient likely not be offered surgery?
>8U/m2 with 2PAs
*Some might consider if the patient was under 2 years and the PVR decreases with vasoreactivity testing
223
In a truncus patients with irreversible obstructive pulmonary vascular disease, is transplant an option?
No
224
In type 1 Truncus, where do the PAs most commonly arise from?
Left posterolateral aspect of truncus (small distance above the truncal valve)
225
What % of patients have Type 1, Type 2 and Type 3 Truncus?
1: <50-65%
2: 30-45%
3: 5-10%
226
What is the most common truncal valve morphology?
Tricuspid (70%)
227
What is the most common abnormal truncal valve morphology?
Quadricuspid (20%)
*Then bicuspid (<10%)
228
True or False: Right arch with mirror-image brachiocephalic branching is commonly associated with truncus
True- 35%
229
What arch abnormality can be seen in patients with Truncus and DiGeorge?
IAA (10-20% of patients)
230
What % of truncus patients have a secundum ASD?
10-20%
231
True or False: Aberrant subclavian, persistent L-SVC to CS and mild tricuspid stenosis are less common abnormalities seen in Truncus?
True:
- Aberrant subclavian: 5-10%
- Persistent L-SVC to CS: <5% to 10%
- Mild tricuspid stenosis: <6%
232
IAA in the setting of truncus is frequently associated with what?
DiGeorge (22q11 deletion)
233
When could you consider surgical correction for a non-neoate with truncus?
If child is <2 years and PVR decreases to <8Um2 with vasoreactivity testing (100% O2 or iNO)
*Still higher risk, but increased resistance may result from arteriolar or medial smooth muscle hypertrophy/vasoconstriction rather than advanced intimal occlusive disease... these changes may be reversible
234
Describe the 2nd heart sound in truncus
Single and loud
235
What might cause a split S2 in truncus?
Delayed closure of some of the cusps of the abnormal truncal valve (usually S2 single/loud)
236
When is corrective surgery for truncus preferred?
First weeks of life (neonatal period)
237
Why should truncus repair be done early?
1. Delay increases risk of ischemia to hypertrophied ventricle (desaturated blood in coronaries with low diastolic perfusion pressure from runoff through PAs and "aortic " insufficiency if present)
2. PVOD can develop early
3. Repair at 6-12 months carries mortality rate 2x that for repair between 6 weeks to 6 months
238
What type of RV-PA conduit is preferred in Truncus?
Valved- Due to pulmonary HTN
239
True or False: Presence of a regurgitant truncal valve is almost always amenable to valve repair?
True- replacement is rarely needed in neonatal period
*If recurrent truncal valve incompetence occurs, repair or replacement can happen at time of re-operation for conduit replacement
240
What is the most common arrangement of of papillary muscles in the setting of asymmetric congenital mitral stenosis with unbalanced chordal attachment?
2 separate papillary muscles
241
What is asymmetric congenital mitral stenosis with unbalanced cord attachment often called?
Parachute (like) mitral valve
*With 2 papillary muscles (most common type), the valve is parachute-like with unbalanced chordae that predominantly attach to 1 papillary muscle... this mimics the appearance of a classic parachute mitral valve
242
What is a classic parachute mitral valve?
Valve with hypoplastic, fused or single papillary muscles and focalized chordal attachments
*Less common than a MV with 2 papillary muscles and unbalanced chordal attachments
243
What is it called when mitral valve leaflets are thickened and the chordae are markedly shortened or absent?
Mitral arcade or "hammock valve"
244
Describe the leaflet insertion in mitral arcade or "hammock valve"
- Leaflets insert directly to papillary muscles or posterior ventricular wall
- Abnormal band of fibrous tissue often extends along the free margin of one or both valve leaflets which tethers the leaflets and papillary muscles
- Results in limited MV excursion, stenosis and insufficiency
245
True or False: It is common after surgical relief of MS to have pulmonary HTN persist
True
246
Where is an isolated cleft of the MV directed?
Anteriorly towards LVOT
247
Describe an isolated cleft in the MV
1. Rare
2. Associated with significant MR
3. Valve is somewhat dysplastic and cleft edges are usually thick and rolled
4. Cleft directed anteriorly towards outflow septum
5. Papillary muscles usually normal, but may have accessory chords which attach to the membranous and muscular septum (this could cause LVOTO)
5. MV annulus commonly dilated
6. Most commonly associated CHD: ASD, VSD, TGA
*Some cases of complete cleft, accessory chords are absent and anterior leaflet flails or is grossly insufficient
248
Where is the cleft in the MV associated with AVSD directed?
More posterior towards inlet septum
249
What is initial management for patients with moderate MR?
Medical- Diuretics and afterload reducing agents (ACEi)
*If severe MR/HF unresponsive to medical management, then surgeryC
250
What do you need to be mindful of in a patient with moderate MR?
Atrial dilation leading to atrial arrhythmias (may need anti-arrhythmics)
*If severe LAE +/- A fib, may even develop atrial thrombosis and require platelet antagonists or anticoagulation
251
Click when supine shortly after S1 at apex. When sitting, click moves towards S1 followed by 1/6 systolic murmur at apex which ends before systole concludes.
MVP
252
What is a small subset of MVP patients at risk for?
SCD
253
When do you have to restrict MVP patients to low-intensity 1A sports?
If they have...
1. Arrhythmogenic-mediated syncope
2. Repetitive non-sustained or sustained SVT or frequent/complex ventricular tachyarrhythmias on ambulatory Holter monitoring
3. Color Doppler evidence of severe MR
4. LVEF <50%
*Otherwise MVP can do all competitive sports
254
In neonates with critical AS and a small LV, what factors help decide about Norwood palliation v. 2V repair?
Factors that may have improved survival with Norwood-type palliation or transplant...
1. Non-apex forming LV
2. Small aortic annulus (<5mm)
3. Small MV annulus (<9mm)
4. Significant retrograde flow in the distal aortic arch through PDA
*Rhodes criteria and CHSC study
255
What are the Rhodes criteria parameters for success of 2V repair in critical AS?
1. Aortic root dimension indexed to BSA
2. Ratio of long axis of LV to long axis of heart
3. Indexed MV area
256
What are the CHSC study parameters for success of 2V repair in critical AS?
1. Age
2. Aortic valve Z-score
3. Grade of EFE
4. Diameter of ascending aorta
5. Presence of significant TR
6. LV length Z-score
257
3/6 harsh systolic murmur along left mid-sternal border radiating to neck, no diastolic murmur or click, few PVCs with intensity of murmur louder after PVC... most likely etiology?
Subaortic stenosis
* Systolic click helps differentiate subvalvular v. valvular AS
* LV impulse may be hyperdynamic and associated findings of AR or MR may be present
258
Asymptomatic children and young adults with Doppler mean gradient of what across AoV should be considered for cardiac cath and possible balloon valvuloplasty?
>40mmHg
259
Patients who desire to participate in competitive sports or are contemplating pregnancy with a mean Doppler gradient of what across the AoV should be considered for cath and possible valvuloplasty?
>30mmHg
260
Balloon valvuloplasty is indicated if a catheter peak to peak gradient across the AoV is what?
>60mmHg
261
Balloon valvuloplasty is indicated of a catheter peak to peak gradient across the AoV is what?
>50mmHg
262
Patients with symptoms (angina, syncope, DOE) or ischemic or repolarization changes on rest to exercise ECG should have valvuloplasty if the cath peak-to-peak gradient across the AoV is what?
>50mmHg
263
True or False: Valvuloplasty isn't recommended for asymptomatic patients with peak-to-peak gradients <40mmHg unless cardiac output is impaired
True
264
What echo modality is helpful to assess systolic and diastolic dysfunction in patients with AS?
TDI
265
Doppler parameters from what have been used for estimation of LV filling pressure?
Mitral or pulmonary vein flow
266
What are mitral or pulmonary vein flow Doppler parameters for estimation of LV filling pressure limited by?
Measurements are dependent on loading conditions and HR
267
How does TDI work?
Directly measures myocardial velocities (typically systolic and diastolic mitral annular velocity)
*Allows quantification of systolic long-axis function and diastolic function
268
What ratio on echo correlates with LVEDP?
Early mitral inflow velocity (E) to early diastolic mitral annular velocity (E')
269
What can measurements of the mitral annular systolic velocity (S') by TDI show in patients with AS who otherwise have normal EF?
Systolic long-axis dysfunction
270
Why might long axis dysfunction precede transverse axis dysfunction in patients with AS?
- Longitudinally oriented fibers are present in the subendocardial region
- The subendocardium is most susceptible to ischemia in patients with AS so these fibers are at greater risk than circumferentially oriented fibers
271
What cath peak to peak, mean Doppler and PIG represent mild AS?
Peak to Peak: <30mmHg
Mean Doppler: <25mmHg
PIG: <40mmHg
272
What cath peak to peak, mean Doppler and PIG represent moderate AS?
Peak to Peak: 30-50mmHg
Mean Doppler: 25-40mmHg
PIG: 40-70mmHg
273
What cath peak to peak, mean Doppler and PIG represent severe AS?
Peak to Peak: >50mmHg
Mean Doppler: >40mmHg
PIG: >70mmHg
274
Which AS patients can do all competitive sports?
- Mild stenosis
- Asymptomatic
- Normal exercise tolerance
275
Which AS patients can do no sports?
Severe stenosis
276
Patients with moderate AS who are asymptomatic with absent or mild LVH, no repolarization abnormality on ECG and normal exercise testing can do what activities?
- Low static component
- Low-moderate dynamic component
* Golf, bowling, baseball/softball, volleyball
*No history of SVT or ventricular tachyarrhythmia at rest or with exercise can do moderate static component and low dynamic component (diving, archery, equestrian, motorcycle)
277
What do you do to assess activity restrictions in someone with AS/AI?
Combine both sets of recommendations
278
Significant retrograde diastolic flow reversal in the distal arch from PDA is consistent with what?
Severe stenosis (AS/LVOTO)
279
What factors are associated with more rapid progression of subaortic stenosis?
- Higher initial pressure gradient
- Short distance between obstructive lesion and AoV
- Anterior MV leaflet involvement
280
At what stage of AS in infants can EFE/papillary muscle infarction be seen?
Severe
*Usually not present in mild forms of disease
281
What can be present in asymptomatic childre with hemodynamically moderate congenital AS?
Myocardial fibrosis
282
What are the Doppler estimates of pressure gradients across stenotic valves used for estimation of severity of obstruction dependent on?
- Loading conditions
- HR
- Other factors
283
What are the 2 options for infants with severe/critical AS who are candidates for 2V repair?
- Balloon valvuloplasty
| - Surgical valvotomy
284
Which has a higher risk for AI, balloon valvuloplasty or surgical valvotomy?
Balloon valvuloplasty
285
Which has a higher risk for residual/recurrent stenosis, balloon valvuloplasty or surgical valvotomy?
Surgical valvotomy
286
What group of patients are more likely to have complex coarctation with associated lesions?
Children who present in infancy (versus older children)
287
What % of patients who require surgical correction before 12 months have a simple CoA?
50%
*Remainder with complex CoA
288
In complex CoA, what is the most common associated lesion?
VSD
*Perimembranous, muscular or malalignment VSD most common
289
What is common in a critically ill infant with CoA + VSD?
Subvalvar AS
-Malalignment VSD may occur with posterior deviation of the conal septum and LVOTO
290
A bicuspid AoV is present in what % of patients with CoA?
85%
-Valve may be stenotic or the annulus hypoplastic
291
Besides a bicuspid AoV, what other left sided lesion can occur in patients with CoA?
MS
-From a supravalvar mitral ring, thickening and dysplasia of mitral leaflets, short dysplastic chordae tendineae or presence of a single "parachute" papillary muscle
292
What is the association of multiple left sided obstructive lesions with CoA called?
Shones
293
How does CoA present in an asymptomatic child?
Upper extremity HTN
| Heart murmur
294
When is repair recommended for CoA in asymptomatic child without severe upper extremity HTN?
2-3 years
295
What is increased if CoA repair is done on a patient <1 year?
Risk for late recurrence of CoA
296
The normal descending aorta has attained what % of its final adult diameter by 3 years?
<50%
*Significant hemodynamic obstruction at rest occurs only if the Ao diameter is reduced by >50%, so restenosis following CoA repair after 3 years is uncommon
297
Why shouldn't elective CoA repair be delayed into later childhood or adolesence?
There is increased risk for residual HTN and early atherosclerotic CV disease
298
What should you consider with a LA SBP that is >20mmHg higher than the RA or legs?
CoA with anomalous aortic arch branching pattern (anomalous right subclavian distal to CoA)
299
True or False: Normal abdominal aortic Doppler profile doesn't rule out CoA
True- If the PDA is patent, pulse wave Doppler can be normal
300
Widely spaced nipples, webbed neck, CoA
Turners
301
Genotype in Turner's
45 XO
302
What causes exercise induced UE HTN in repaired CoA?
Increase in CoA pressure gradient due to increased Ao blood flow across non-distensible CoA repair site
303
What can be beneficial for patients with exercise HTN, but no significant anatomic stenosis following CoA repair?
B-blocker- Will decrease exercise HTN and CoA gradient
304
Long-term prognosis following CoA repair can be adversely affected by what 2 things?
1. Systemic arterial HTN
| 2. Increase in late premature atherosclerotic disease
305
Resting systolic and diastolic HTN at rest occurs most commonly in what type of CoA repair?
Delayed (beyond later childhood)
306
What is the risk for late HTN (even if CoA repaired in infancy)?
10-20%
307
What might eh cause of late post-op HTN in patients w/o residual resting CoA relate to?
Anatomic/functional changes in arterial vasculature proximal to CoA
308
Acidosis, increasing LFTs and elevated creatine are signs of what in a post-op patient?
Inadequate tissue delivery to end organs
309
What is the more effective way to match O2 delivery to changes in O2 consumption in a post-op stage 1 single ventricle?
Interventions in total CO or Hgb concentration
-Versus precise manipulation of Qp/Qs
310
How is milrinone used in the setting of an elevated Qp:Qs and compromised systemic perfusion in a stage 1 single ventricle?
- Reduces PVR and can help to increase Qp/Qs
| * Also can cause significant hypotension so have to be cautious
311
What would happen if you increased FiO2 or started iNO on a stage 1 single ventricle?
Increased PBF at the expense of systemic perfusion
312
What happens if you increase respiratory rate in a stage 1 single ventricle?
Drive down pCO2, increase PBR a expense of systemic perfusion
313
In D-TGA, what are anterior malalignment VSDs associated with?
Varying degrees of override of the pulmonary annulus into the RV
*With increasing degrees of override, the anatomy is more like DORV with a subpulmonary defect (Taussig-Bing)
314
In D-TGA, what is subaortic stenosis caused by anterior malalignment of the infundibular septum frequently associated with?
Aortic arch hypoplasia, CoA or IAA
315
True or False: In d-TGA the sinus node and AV nodes are typically in their normal location
True
*Normal atrial anatomy, so sinus an AV nodes are in the usual locations
316
Where is a normal conus located?
Subpulmonary, left-sided and anterior
*This prevents fibrous continuity between pulmonary and tricuspid valve rings
317
In D-TGA, where is the conus (or infundibulum) located?
Subaortic, right sided and anterior
*This prevents fibrous continuity between aortic and tricuspid valve ring
318
What is D-TGA hypothesized to result from?
Abnormal growth and development of the subaortic infundibulum and concurrent absence of growth of the subpulmonary infundibulum
319
Describe the shape of the IVS in D-TGA?
Entire septum usually straight (doesn't have sigmoid curvature of normal heart)
320
What are 2 approaches for a neonate with d-TGA/large VSD and severe LVOTO?
1. Palliate BTT shunt
| 2. Corrective surgery (Rastelli)
321
How would a neonate with d-TGA/large VSD and severe LVOTO present?
Markedly restricted PBF and severe hypoxemia
322
What surgery is most appropriate for d-TGA with a large VSD and extensive LVOTO?
Rastelli (intraventricular repair and placement of an extracardiac RV-PA conduit)
*Complete bypass of the LVOTO and anatomic correction of transposition pathology
323
What is an alternative to a Rastelli?
REV- Reparation a l'etage ventriculaire
324
What are benefits to a REV?
1. Can be used in younger patients
2. Avoids prosthetic extracardiac conduit
3. Avoids intracardiac tunnel obstruction
325
What is a REV?
- High/anterior RV incision and radial excision of the outlet septum to create an unobstructed anterior RV cavity
- Establishes a short/direct intraventricular tunnel from LV to aorta
- Closure of PA orifice
- Reimplantation of transected PA directly onto RVOT without prosthetic conduit
326
What is the technique described by Nikaidoh in relation to D-TGA with VSD and LVOTO?
Posterior translocation of the aortic root and coronary arteries with enlargement of the LVOT and conduit placement from RV-PAs anteriorly
327
In cc-TGA, how is the IVS positioned?
Sagittal
328
Describe the conduction system in cc-TGA
- Both surfaces and ventricular bundle branches are inverted
- Sequence of initial activation is oriented from R-L and usually more superior/anterior direction
- This results in reversal of normal Q-wave pattern in precordial leads (Q waves present in right precordial leads, but absent in left precordial leads)
329
What are the expected ECG changes in cc-TGA?
- Reversal of Q-wave distribution in the precordial leads with QS complexes in the right precordial leads and large Q waves in leads III and avF
* Q-waves in V1, no Q waves in V6, Complete AV block
330
Where is the AV node in patients with cc-TGA?
Along the anterior aspect of the AV ring near the atrial septum
331
What does subaortic stenosis in DORV result from?
Extensive hypertrophy of the aortic conus and conal septum or marked malalignment of the conal septum
332
What is there an association with in DORV and subpulmonary VSD?
IAA or CoA
*Compression of the aortic outflow tract and reduction of aortic flow leading to secondary hypoplasia of the aortic annulus and aorta
333
True or False: CoA has been described in instances of subaortic, double committed and remote VSD
True
334
What does complete correction of DORV depend on?
Complexity of the cardiac anatomy
*May have to palliate some infants and small children who are symptomatic in the 1st year of life
335
What AV valve issue makes 2V repair impossible in the setting of DORV?
Mitral valve straddling
336
DORV with side-by-side great arteries, large inlet VSD with MV straddle, moderate subaortic stenosis, severe CoA... most appropriate initial surgery?
Norwood-Sano
337
Surgical procedure of choice for a patient with DORV and subpulmonary VSD w/o PS?
ASO + Patch closure of VSD
338
What is the physiology of DORV + Subpulmonary VSD?
TGA
339
6 m/o with SpO2 75-80% that doesn't change with O2, gradually bluer over last months, loud/harsh SEM... what type of DORV most likely?
DORV with subaortic VSD and PS
340
True or False: Patients with subaortic VSDs and PS have varying degrees of cyanosis similar to ToF?
True
341
In ToF like DORV, if the PS is severe, what is the clinical presentation?
- Early cyanosis
- FTT
- Exertional dyspnea
- Polycythemia
- RV impulse at LSB
- Prominent systolic thrill over LUSB
- Grade 4-5/6 SEM radiating to lung fields
- 1st heart sound normal
- 2nd heart sound single
- 3rd heart sound at apex
- Possible clubbing
342
DORV + Subpulmonary VSD and no PS presents similarly to what?
d-TGA with VSD
343
How does DORV + Subpulmonary VSD and no PS present?
- Cyanosis and HF in early infancy
- Severe FTT
- Frequent respiratory tract infections
- Precordial bulge and RV impulse at LSB
- Grade 2-3/6 high-pitched systolic murmur at LUSB
- Increased pulmonary flow, apical diastolic rumble
-Like a d-TGA with VSD
344
How does a DORV + Subpulmonary VSD, no PS and associated CoA present?
In infancy with heart failure, cyanosis and diminished/absent femoral pulses
345
DORV + Subpulmonary VSD and PS murmur?
- Systolic thrill
- Loud murmur (grade 3-4/6)
- 2nd heart sound loud/single (due to proximity of aorta to chest wall)
346
What would a Taussig-Bing DORV with a non-restrictive VSD, large PDA with low-velocity bidirectional shunt, tiny PFO, mildly hypoplastic, but unobstructed aortic arch need who is persistently hypoxic despite PGE?
BAS
-Physiology like d-TGA and patient needs increased mixing at atrial level
347
Why is systemic outflow tract obstruction in a single ventricle a risk factor for poor outcome at Fontan?
Promotes myocardial hypertrophy
348
Why can PA banding cause increased subaortic gradient in a patients with DORV, normally related great arteries and multiple muscular VSDs?
Hypertrophy of subaortic conud
349
Why does banding help in the setting of DORV with multiple muscular VSDs or a remote VSD?
Reduces pulmonary flow and protects the pulmonary arterioles from obstructive pulmonary arteriopathy
350
What type of DILV is at risk for failing a 2V repair?
DILV with a right-sided hypoplastic subaortic RV
351
What should be done for a neonate with DILV with a hypoplastic subaortic RV and restrictive bulboventricular foramen and severe suboartic stenosis?
DKS with BTT
*Systemic perfusion at risk and baby needs stable systemic blood supply
352
Why isn't PA banding the best option for a neonate with DILV with a hypoplastic subaortic RV and restrictive bulboventricular foramen and severe suboartic stenosis?
Can cause ventricular hypertrophy and obstruction in patients who have naturally occurring mild restriction at the VSD
353
Chest pain, syncope, hyperactive impulse slightly displaced to left, CXR with slightly displaced cardiac silhouette to left, prominent bulges of aortic knob and PA... what test to do?
MRI (with concern for partial pericardial defect)
354
What is the best way image with clinical suspicion for a partial pericardial defect?
MRI
355
Where are most defects in partial absence of the pericardium?
Left side (80%)
356
What causes partial absence of the pericardium?
Premature atrophy of the left duct of Cuvier during embryologic development
357
True or False: Most cases of pericardial defect are identified incidentally and symptomatic cases are rare?
True
358
What are possible symptoms of a partial defect of the pericardium?
Syncope, chest pain, arrhythmia, death
359
What are severe symptoms from a partial defect of the pericardium secondary to?
Herniation or incarceration of LAA through the defect, torsion of the great arteries or constriction of a coronary artery at the rim of the defect
360
What testing modality is ideal for evaluating the pericardium and the procedure of choice for partial absence of the pericardium?
MRI
*May see a tongue of pulmonary tissue between the aorta and MPA
361
What could be seen on CXR in complete absence of the pericardium?
Leftward displacement of the cardiac border with posterior bulging of the heart
362
True or False: Herniation of the LAA due to absence of the pericardium can be seen on CXR?
True- Herniated appendage resembles an enlarged MPA
363
Describe the ECG in absence of the pericardium
Often normal
*Can have RBBB or other conduction abnormalities
364
What can be seen on echo for absence of the pericardium?
- RV may be enlarged
- Excessive cardiac motion
- LAA may be prominent
*Can't see actual defect
365
True or False: Cath is helpful to diagnose absence of the pericardium?
False- Cath is of little diagnostic value (other than to document coexisting heart disease)
366
True or False: Thorascopy may be necessary to confirm a diagnosis of absence of the pericardium
True
367
What factors are associated with increased risk for sudden death in kids with HCM?
1. Prior cardiac arrest or sustained v-tach
2. FHx of one or more premature HCM related deaths (particularly is sudden/multiple)
3. Syncope
4. Massive LVH (wall thickness >30mm, but risk of SCD increases when septal thickness is >20mm)
368
What is recommended for screening of family members with HCM?
- ECG/Echo every 12-18 months starting no later than 12
| - If no LVH by 21, then echo every 5 years
369
What is the known inheritance pattern of HCM?
AD
370
Any HCM patient with a history of aborted cardiac death needs what?
ICD as primary prevention
371
When do you consider a myectomy in HCM?
Severe hypertrophy of septum, significant gradient, symptoms
372
The subaortic gradient and SEM in HCM is what?
Dynamic
373
What things reduce the SEM in HCM?
- Decrease myocardial contractility: B-blockers
| - Increase ventricular volume or arterial pressure: Squatting, isometric handgrip, phenylephrine
374
What things increase the SEM in HCM?
- Decrease arterial pressure or ventricular volume (Valsalva, nitro)
- Increase contractility: Standing, amyl nitrate inhalation, isoproterenol, exercise
375
When does sudden death in individuals with HCM most commonly occur?
During adolescence and young adulthood (12-35)
*Rarely before 10
376
What are sudden cardiac death events in HCM often due to?
Primary ventricular tachycardia and/or ventricular fibrillation
377
What is the relationships between SCD in HCM and exercise?
- Most die while sedentary or during normal/modest physical exertion, but important proportion die suddenly during or just after vigorous activity
* HCM patient's can't do intense competitive sports
378
What is the most common cause of sudden cardiac death in the young (including athletes)?
HCM
379
True or False: Septal myectomy decreases SCD risk in HCM?
False: Septal myectomy can improve symptoms, but doesn't change SCD risk
*To prevent SCD, an ICD is needed
380
True or False: A drop in BP during exercise is associated with an increased risk of sudden death in HCM
True
381
What activity restrictions are needed for a teenager with a gene mutation for HCM, but no symptoms, normal echo and no family history of sudden death?
None... no medical therapy or activity restriction needed
*Low risk patient with no symptoms, negative family history and normal echo
382
How does milrinone work?
Phosphodiesterase inhibitor
383
What are the hemodynamic effects of milrinone?
- Increased stroke work and CO
- SVR/PVR decreased
- Lusitropic properties that affect relaxation and ventricular compliance
- Increases intracellular Ca concentration (by inhibition of phosphodiesterase III)
384
How do beta blockers effect milrinone?
Milrinone retains its full hemodynamic effects in the presence of beta-blocker therapy
385
Skeletal myopathy, congenital dilated cardiomyopathy, short stature, neutropenia
Barth syndrome
386
How is Barth syndrome inherited?
X-linked
387
What is the overall prognosis in Barth syndrome?
Affected individuals die in early childhood
388
How is Barth syndrome diagnosed?
UA with elevated 3-mthylglutaconic acid
389
How does Barth syndrome present?
- Boys with hypotonia and dilated cardiomyopathy
- Labored breathing, poor appetite, slow weight gain in first few months after birth
- Bacterial infections (because of neutropenia)
390
What is the gene affected in Barth syndrome?
Tafazzin (TAZ) on chromosome Xq28
391
What is the problem with the mutation in the TAZ gene leading to Barth?
Decreased production of an enzyme needed for synthesis of cardiolipin
392
What is the treatment for Barth?
None specifically
-Each problem can individually be controlled
393
What is needed to help differentiate between RCM and CP?
Cath
394
What cath sign do RCM and CP have in common?
Square root- Early diastolic dip and plateau pattern
395
What are the cath findings in classic RCM?
LVEDP, LA pressure and PCWP are all markedly elevated and >4-5mmHg (ideally >10mmHg) greater than the RAP and RVEDP
396
When trying to distinguish between RCM and CP, what can bring out differences in pressure between the right and left side in cases where the pressures are essentially equal?
Volume loading
397
What is Loeffler endocarditis?
Hypereosinophilic syndrome (HES)
398
What type of patients get hypereosinophilic snydrome (HES)?
Adult males who live in temperate climates
399
What are the lab seen in HES?
Persistent hypereosinophilia: 1500 eosinophils/mm3 for >5 months or until death
400
What organs can be involved in HES?
Heart, lungs, bone marrow, brain
401
What is the cause of eosinophilia in HES?
Unknown
402
What is seen on cardiac histology in HES?
Eosinophilic myocarditis- Inflammatory reaction in the small intramural coronary vessels with thrombosis and fibrinoid change and endocardial mural thrombosis and fibrotic thickening
403
How does HES present?
Weight loss, fever, cough, rash, heart failure, systemic embolism
404
What typically causes death in HES?
Cardiac manifestations of disease
405
How is HES treated?
- Steroids, hydroxyurea, vincristine
| - Digoxin, diuretics, afterload reduction, anticoagulation
406
What are surgical options for HES?
- Mitral/tricuspid valve repair or replacement
| - Excision of fibrotic endocardium
407
What happens to the ventricular size and function in RCM?
- Small ventricular volumes
- Ventricular wall thickness usually normal
- Normal or near-normal systolic function
*Atria are enlarged
408
True or False: Patients with Duchenne/Becker muscular dystrophy can have severe anesthesia complications including cardiac arrest?
True
409
What agent used for anesthesia/induction causes the most complications in patients with Duchenne/Becker muscular dystrophy?
Succinylcholine (muscle relaxant that may trigger hyperkalemia)
*Others have been attributed to the use of volatile anesthetic agents
410
What could happen to a Duchenne or Becker muscular dystrophy patients who receives succinylcholine?
Reaction similar to malignant hyperthermia
*Develop rhabdo with things like masseter muscle spasm
411
What is the characteristic ECG finding in Duchenne muscular dystrophy?
Deep Q waves in I, aVL, V5, V6 and sometimes II, III, aVF
* Often tall right precordial R waves and increased R/S ratio
* PR shortened in many patients
* Some with QR prolongation and QT dispersion abnormalities
412
What is often seen on Holter in patients with Duchenne muscular dystrophy?
- Automaticity is affected
- Resting sinus tachycardia
- Loss of circadian rhythm
- Reduced heart rate variability
413
What types of dysrhythmias are common in older patients with Duchenne muscular dystrophy?
EAT
A-fib
Transient 2/3 degree heart block
Ventricular tachycarias
414
What is concerning for increased risk of sudden death due to v-fib in Duchenne muscular dystrophy?
Presence of multiform PVCs and ventricular tachycardia on Holter monitoring
415
Waddling gait, calf pseudohypertrophy?
Duchenne
416
What is the typical cardiac exam in Duchenne muscular dystrophy?
Normal (even with cardiomyopathy)
* Occasionally a 3rd/4th heart sound
* May have neck vein distention of hepatomegaly
417
What can distort the cardiac exam in older patients with Duchenne?
Chest wall deformities (scoliosis)
418
What is the most commonly reported arrhythmia in kids with RCM?
Atrial flutter
* After this was high grade 2nd and 3rd degree heart block
* Other possibilities: A-fib, atrial tachycardia, WPW, SVT, symptomatic sinus bradycardia (needing pacing), v-tach, torsades
419
What % of patients with RCM have arrhythmias or conduction disturbances?
<15%
