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Peds Cardiology > 2. Malformations > Flashcards

2. Malformations Flashcards

1
Q

Inheritance of Alagille?

A

AD

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2
Q
  • Liver disease (bile duct paucity)
  • Cholestasis
  • CHD
  • Skeletal abnormalities
  • Ocular abnormalities
  • Broad forehead, pointed chin
A

Alagille

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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

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4
Q

Notch ligand

JAG1

A

Alagille

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5
Q

NOTCH1

A

AoV pathology

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6
Q

PTPN11

A

Noonan

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7
Q

Hypertelorism, ptosis, short stature, webbed neck, skeletal anomalies, bleeding diathesis, lymphatic disorders, mental retardation, cryptorchidism, CHD

A

Noonan

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8
Q

Types of CHD in Noonan

A 
HCM
PV stenosis
ASD (primum/secundum)
VSD
ToF
PA stenosis
CoA
Partial AVSD
Polyvalvulopathy
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9
Q

TBX1

A

DiGeorge

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10
Q

Hypocalcemia, immunodeficiency, CHD

A

DiGeorge

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11
Q

Types of CHD seen in DiGeorge?

A

IAA-Type B
Truncus
ToF

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12
Q

GATA4

A

Septation defects

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13
Q

Rubella in 1st trimester increases fetal risk for what CHD?

A
  • PS (valvar, supravalvar, peripheral)
  • PDA

*ToF also reported

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14
Q

True or False: Excessive caffeine during pregnancy increases risk for CHD

A

False

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15
Q

CHD associated with maternal PKU?

A
  1. Left-sided defects
  2. Septal defects
  3. ToF
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16
Q
  • Microcephaly
  • Mental retardation
  • High phenylalanine
A

PKU

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17
Q

7q11.23 deletion

A

Williams

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18
Q

Hypercalcemia, skeletal and renal anomalies, cognitive deficits, social, “elfin” facies

A

Williams

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19
Q

Types of CHD seen in Williams?

A
  1. Supravalvar AS

2. Supravalvar PS

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20
Q

3/6 SEM, friendly, stellate irises, long philtrum, depressed nasal bridge, prominent lower lip, enamel hypoplasia

A

Williams

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21
Q

How is the deletion associated with Williams detected?

A

FISH (not by routine karyotype)

*7q11.23 deletion

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22
Q

In Williams, does the supravalvar PS or AS tend to improve with time?

A

PS improves with time

AS usually progresses

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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)

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24
Q

Why are patients with William’s syndrome prone to develop HTN?

A

Renal artery stenosis

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25
Q

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

A

T18

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26
Q

What is the % incidence of CHD in T18?

A

> 90%

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27
Q

Most common CHD in T18?

A
  1. Perimembranous VSD
  2. ToF
  3. DORV
  4. Polyvalvular dysplasia
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28
Q

What % of children with T18 die within 1st year of life?

A

90%

*Usually not from heart disease

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29
Q

Most common CHD associated with 22q11 deletion?

A
  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

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30
Q

What can develop is a large ASD is left unrepaired?

A

Severe irreversible pulmonary HTN

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31
Q

In which gender is there a higher preponderance for the development of pulmonary HTN from an unrepaired ASD?

A

Female

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32
Q

Above what diameter ASD is spontaneous closure rare?

A

> 8mm

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33
Q

What may need to be done prior to repair of any type of ASD that is not discovered until adolescence or adulthood?

A

Cath to assess for pulmonary HTN and vasoreactivity

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34
Q

Sinus venosus ASDs

A
  • 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
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35
Q

What is seen on ECG for a sinus venosus ASD?

A

Frontal plane P-wave axis <30 degrees (up to 1/2 patients)

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36
Q

Which types of ASDs have a normal P-wave axis?

A
  1. Secundum
  2. Primum
  3. PFO
  4. Unroofed CS

*Sinus venosus ASDs can have

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37
Q

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?

A

Complete AV canal defect

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38
Q

How many leaflets does a common AV valve have?

A

5

*Beneath 5 commissures are 5 papillary muscles

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39
Q

In a complete AVCD are the left sided papillary muscles closer together or further apart as compared to a normal heart?

A

Closer

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40
Q

Which AV valve leaflet is smaller than normal in a complete AVCD?

A

The left lateral

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41
Q

How are the left sided papillary muscles in a complete AVCD rotated and what effect does this have on their position?

A

Counterclockwise

  • Posterior further from septum
  • Anterior closer to septum
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42
Q

What is the potential result of the change in left sided papillary muscle arrangement in complete AVCD?

A

Progressive LVOT obstruction

also the large anterolateral muscle bundle

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43
Q

Components of a partial AVSD?

A
  1. Primum ASD

2. Cleft anterior MV leaflet

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44
Q

True or False: A primum ASD results in earlier and more severe symptoms as compared to a secundum ASD

A

True

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45
Q

What is the most common reason for reoperation in partial AVSD?

A

Repair of recurrent or residual MR/MS

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46
Q

What do you need to think about in a patient with AVSD and a SEM?

A

LVOTO

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47
Q

Progressive LVOT obstruction is more common in patients with what type of AVSD?

A

Partial- 2 AV valve orifices

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48
Q

What is different about the aortic valve in AVSD?

A

Displaced anteriorly (versus being wedged between mitral/tricuspid annuli)

  • Creates elongated/gooseneck deformity of LVOT
  • LVOT obstruction can happen in any form of AVSD
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49
Q

Is LVOTO more common in AVSD with a common AV valve or 2 AV valve orifices?

A

Common

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50
Q

What % of patients with AVSD require reoperation for LVOTO?

A

10%

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51
Q

List mechanisms for progressive LVOTO in AVSD?

A
  • 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
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52
Q

Post-op from ASD/VSD repair hearing widely split S2 and no murmur… what is the cause?

A

RBBB

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53
Q

True or False: Prominent splitting of S2 can be heard with a small VSD

A

True

-Usually normal, but can have this sometimes

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54
Q

What should you consider in a VSD when you have systolic murmur that transmits to the LUSB or Apex?

A

LUSB: PS
Apex: MR

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55
Q

What limits flow across a large/unrestrictive VSD?

A

Relative resistances of systemic/pulmonary circulations

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56
Q

What anomalies of the tricuspid valve can be seen in a perimembranous VSD with L-R shunting?

A

Redundant septal leaflet tissue (this can partially or completely occlude the defect)

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57
Q

What happens to LV mass and volume after a VSD repair?

A

Decrease

Volume decreases at a greater rate than mass

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58
Q

Patients who develop Eisenmenger physiology begin manifesting cyanosis before what age?

A

2

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59
Q

What can happen to the aortic valve with an unrepaired outlet or perimembranous VSD?

A

Prolapse of one of the aortic cusps

-Leaflet partially closes the VSD and eliminates shunting, but the AI is progressive

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60
Q

What can be seen in an unrepaired outlet VSD?

A
  • 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
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61
Q

What can be seen in an unrepaired perimembranous VSD?

A
  • 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
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62
Q

Relationship of the bundle of His to an inlet VSD?

A

Bundle passes anterior-superior to defect

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63
Q

Relationship of perimembranous VSD to bundle of His?

A

Bundle of His is subendocarial and courses along the posterior-inferior margin of the defect

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64
Q

Where is the conduction tissue in relation to a muscular or outlet VSD?

A

Usually far removed unless the defect extends into the perimembranous area.

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65
Q

NEC + PDA

A

Surgical closure of PDA

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66
Q

Potential complication of unrepaired PDA?

A

Endarteritis or endocarditis

*PDA can account for up to 15% of all endocarditis cases

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67
Q

Most common organisms for endocarditis with PDA?

A

S. Viridans

S. Aureus

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68
Q

Where are vegetations most commonly seen in a PDA?

A

PA end of the duct

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69
Q

Which event is most responsible for early, functional closure of ductus arteriosus?

A

Medial smooth muscle cell migration in the wall of the ductus

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70
Q

When do the 2 phases of post-natal closure of the PDA occur?

A

1st: Within 12 hours after birth
2nd: 2-3 weeks

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71
Q

Describe the 2 phases of post-natal closure of the PDA

A
  • 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
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72
Q

Theoretical benefits of ibuprofen over indomethacin for closure of PDA in a premature infant?

A
  • Similar rates of ductal closure

- Fewer negative effects on renal function, cerebral vasculature and cerebral blood flow

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73
Q

Which has higher risk of intraventricular hemorrhage in PDA closure… indomethacin or ibuprofen?

A

Equivocal

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74
Q

What is the use of ibuprofen as prophylaxis for PDA closure associated with an increased risk of?

A

Pulmonary HTN

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75
Q

What is the standard surgical approach for ALCAPA?

A

Direct reimplantation of the origin of the LCA into the aorta

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76
Q

What is an alternative approach for repair of ALCAPA?

A

Takeuchi

-AP window created and a tunnel created that directs blood from aorta to left coronary ostium

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77
Q

What is a risk factor for mortality and late reoperation in ALCAPA?

A

Mitral insufficiency

*Due to papillary muscle infarction and dysfunction

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78
Q

What determines specific features in a sinus of Valsalva fistula from the aorta to the RA?

A

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

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79
Q

What type of shunting results from a sinus of Valsalva fistula emptying into the left heart v. right?

A

Right: Right to left shunt
Left: No shunt (O2 blood back to LV)

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80
Q

Describe the murmur in a small sinus of Valsalva fistula

A
  • 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
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81
Q

Describe exam findings in a large sinus of Valsalva fistula

A
  • 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)
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82
Q

Which sinus does the coronary in ALCAPA usually arise from?

A

Left posterior facing sinus on PA

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83
Q

Describe pathophysiology in ALCAPA

A
  • 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
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84
Q

True or False: Anomalous coronary artery origins from the wrong sinus of Valsalva are generally asymptomatic in infancy?

A

True

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85
Q

What is the most common coronary artery anomaly?

A

Origin of left circumflex from right main (1/3 of all major coronary arterial anomalies)

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86
Q

True or False: A left circumflex from the right coronary artery has no general clinical significance in the absence of intracardiac surgery

A

True

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87
Q

Why is an origin of LCA from the right sinus of Valsalva important clinically?

A
  • 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
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88
Q

What is often seen with aneurysms in the sinus of Valsalva?

A

VSD (50% of the time)

*Especially right sinus aneurysms associated with defects of the outlet septum

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89
Q

What causes an aneurysm of the sinus of Valsalva?

A
  • 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
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90
Q

Which gender is more affected by aneurysm of the sinus of Valsalva?

A

Male (75%)

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91
Q

Where are sinus of Valsalva aneurysms more frequently located?

A
  • 65% located in right aortic sinus
  • 25% in non-coronary sinus
  • 10% in left aortic sinus
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92
Q

What circumstance is most common for rupture of sinus of Valsalva aneurysm?

A

-Rupture of right sinus into RV in setting of an outlet VSD

  • Can rupture into any chamber
  • Rupture into pericardium is rare
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93
Q

What is risk of mortality in 1st week of like with cerebral AVM causing high-output cardiac failure?

A

90%

*From intractable CHF or neurologic complications (seizures, intracranial hemorrhage)

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94
Q

Central nervous system AVMs manifest symptoms according to what?

A

Hemodynamic effect

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95
Q

What are the most common cerebral AVMs to present with CHF?

A
  1. Deep (vein of Galen)
  2. Superficial (pial)
  3. Dural
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96
Q

What is seen in infants with significant cerebral AVMs?

A
  1. High-output CHF

2. Dilation of all cardiac chambers, feeding arteries and draining veins

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97
Q

What happens if there is venous obstruction in a cerebral AVM?

A

Flow can be restricted… patient may present with venous HTN or cerebral ischemia

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98
Q

If an infant with a significant cerebral AVM survives, what are long-term problems?

A

Neurologic morbidity- Hydrocephalus, mental retardation, hemorrhage

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99
Q

If you have multiple pulmonary AVMs, there is a >80% of having what?

A

HHT

  • Most pulmonary AVMs are congenital or associated with HHT
  • Overall 30-50% of patients with pulmonary AVMs have HHT
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100
Q

What happens to pulmonary AVMs as a child grows?

A

Enlarge

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101
Q

What type of AVMs have increased CO?

A

Systemic

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102
Q

What happens to pulmonary blood flow and pressures with a pulmonary AVM?

A

Unchanged

*PVR normal, resistance within AVM is low, resistance in other lung segments may be elevated

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103
Q

What is the treatment of choice for a pulmonary AVM?

A

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
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104
Q

Embolization of pulmonary AVMs prevents what?

A
  1. Stroke
  2. Tia

*DOES NOT reduce risk of brain abscesses

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105
Q

What is done to avoid device embolization when closing a pulmonary AVM?

A

-Using a coil or umbrella versus liquid adhesive or beads

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106
Q

What measurement has best potential to distinguish a large AVM from a large PDA in a young infant?

A

Systemic vein oxygen saturation during cath

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107
Q

In what ways are large AVMs and large PDAs similar?

A
  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

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108
Q

What does a cath for a large AVM show?

A
  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)
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109
Q

Barium esophagram with anterior indentation?

A
  • Pulmonary artery sling

- Tumor

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110
Q

Describe the anatomy in a pulmonary sling

A
  • 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)
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111
Q

What tracheal anomaly is a pulmonary sling frequently associated with?

A

-Complete cartilaginous rings in the distal trachea resulting in tracheal stenosis

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112
Q

Describe a right aortic arch with mirror image branching

A
  • 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
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113
Q

What should be done if a right aortic arch is noted CT imaging?

A
  • Echo to assess for CHD

- Commonly associated with ToF, but also other conotruncal abnormalities (DORV, etc.)

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114
Q

True or False: A right aortic arch with retroesophageal innominate artery, left PDA results in a vascular ring?

A

True

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115
Q

What is a vascular ring?

A

Aortic arch anomaly in which trachea and esophagus are completely surrounded by vascular structures

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116
Q

Symptoms of a vascular ring?

A
  • Respiratory: Stridor, PNA, bronchitis, cough, neck hyperextension
  • Swallowing difficulty (less common, usually toddlers or older children)
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117
Q

Absent limb pulses in all extremities, but strong carotid pulses bilaterally?

A

IAA type B + Anomalous subclavian

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118
Q

Describe the anatomy of type A, B, C IAA

A 
IAA = Complete separation of ascending and descending aorta
A = Interruption distal to left subclavian
B = Interruption between carotid and subclavian
C = Between carotid arteries
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119
Q

How to differentiate critical AS v. IAA type B + Anomalous subclavian?

A
  • IAA type B + Anomalous subclavian will have strong carotid pulses
  • Critical AS will have all weak pulses
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120
Q

What is the most common vascular ring?

A

Double aortic arch

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121
Q

What is the second most common vascular ring?

A

Right arch with diverticulum of Kommerell

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122
Q

True or False: A right aortic arch with mirror image branching usually causes no ring

A

True

*Unless there is a left duct

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123
Q

What is the most common aortic arch abnormality?

A

Left arch with retroesophageal right subclavian

*Doesn’t form a ring and usually asymptomatic

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124
Q

True or False: Right aortic arch with retroesophageal innominate artery doesn’t create a vascular ring

A

False

  • Very rare
  • Ductus completes vascular ring (connects LPA with base of innominate)
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125
Q

Describe cor triatriatum

A
  • 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
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126
Q

What 2 things is prognosis in TAPVC influenced by?

A
  • Size of interatrial communication

- Degree of obstruction in anomalous venous pathways

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127
Q

What is the long-term prognosis in TAPVC dependent on?

A
  • State of pulmonary vascular bed at time of surgery
  • Patency of pulmonary venous-LA anastomosis

*Overall mortality for unrepaired 80% + at one year

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128
Q

What arrythmias are most common to develop in repaired TAPVC?

A

Atrial: Sinus bradycardia, atrial flutter, SVT

*Ventricular arrhythmias unusual

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129
Q

Describe Scimitar syndrome

A
  • 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)
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130
Q

What are frequent coexistent anomalies in Scimitar?

A
  • Hypoplasia of right lung/chest
  • Mesocardia or dextrocardia
  • Lung parenchymal abnormalities
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131
Q

What causes a superior sinus venosus ASD?

A

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)
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132
Q

What should be done for infradiaphragmatic TAPVC?

A

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
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133
Q

What is the most common site of obstruction in infradiaphragmatic TAPVC?

A

-Anomalous vessels’ connection with portal vein or hepatic veins

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134
Q

If unobstructed, what does flow look like in the anomalous vessel in infradiaphragmatic TAPVC?

A
  • 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
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135
Q

When do symptoms from cor triatriatum present?

A

Usually in 1st few years of life, but some can present in 2-3rd decade of life

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136
Q

What are common late presenting symptoms of cor triatriatum?

A
  • Dyspnea
  • Frequent respiratory issues (asthma)
  • PNA

*Usually thought to have primary pulmonary disease

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137
Q

Untreated cor triatiatum results in what?

A

Pulmonary HTN

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138
Q

Exam findings of cor triatriatum?

A
  • 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
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139
Q

True or False: Patients with pulmonary edema or right heart failure due to cor triatiatum often have progressive disease despite maximal medical management

A

True

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140
Q

What should be done for someone with cor triatriatum and pulmonary edema or right heart failure?

A

Surgery ASAP

*Resection of membrane under bypass

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141
Q

How long is survival with cor triatriatum with pulmonary edema and right heart failure if untreated?

A

Months

*If survive operative correction, severe PA changes which cause pulmonary HTN can regress… for these patients, prognosis is excellent

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142
Q

What is an interrupted IVC?

A

Absence of the hepatic segment of the IVC with azygous continuation to the right or left SVC

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143
Q

What is a factor that could cause hypoxia in a patient with interrupted IVC with azygous continuation and bilateral Glenn shunts years following surgrey?

A

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)

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144
Q

What does a vertical vein represent?

A

A persistent embryologic connection between the splanchnic plexus of the lung buds and the cardinal veins

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145
Q

Besides PAPVR to the right SVC (sinus venosus defect) and RA (malposition of the septum primum) what is the most common type of PAPVC?

A

Left pulmonary veins to the left innominate vein

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146
Q

What is the name of the vein that connects the left sided pulmonary veins to the left innominate vein in PAPVR?

A

Vertical vein

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147
Q

Where anatomically is a vertical vein compared to a L-SVC?

A

Vertical vein usually more posterior than L-SVC (which is immediately behind the LAA)

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148
Q

If the left pulmonary veins drain to the L-SVC, what should the L-SVC drain to?

A

Still CS or LA

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149
Q

What type of ASD is common with PAPVC to the left innominate vein?

A

Secundum

*Primum uncommon and rarely is septum intact

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150
Q

Congenital malformations of the CS are frequently associated with what?

A

Arrhythmias

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151
Q

What is reported in a significant % of patients with diverticular of the CS?

A

SVT and SCD

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152
Q

What causes SVT in a patient with diverticulum of the CS?

A

Accessory pathway that traverses the diverticulum to form an AV connection

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153
Q

2 week old with tricuspid atresia, d-TGA, moderately restrictive VSD, moderately hypoplastic arch, acidotic… best surgical approach?

A

DKS with arch augmentation and BTT shunt

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154
Q

Are ventricular endocardial potentials recorded past the expected anatomic tricuspid valve annulus during EP assessment more specific for Uhl or Ebstein?

A

Uhl

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155
Q

What is a congenital cardiac malformation that consists of an almost total absence of the RV myocardium?

A

Uhl

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156
Q

Physical exam consistent with Uhl?

A
  • Cyanosis
  • Hepatosplenomegaly
  • JVD
  • Quiet precordium
  • Diminished peripheral pulses
  • Decreased heart tones
  • Pansystolic TR murmur (also can be no murmur or non-specific murmur)
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157
Q

What is seen on ECG in Uhl’s?

A
  • Prominent P waves

- Diminished QRS amplitude (especially in the right precordial leads)

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158
Q

What is seen on CXR in Uhl’s?

A
  • Cardiomegaly
  • Normal to diminished pulmonary vascularity

*Similar to Ebstein

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159
Q

What is seen on Echo in Uhl’s?

A

Marked right sided dilation

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160
Q

Describe the PA, RA, RV pressure waveforms in Uhl’s

A

Similar to each other

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161
Q

What is dominant in the RA waveform in Uhl’s?

A

A-wave

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162
Q

What is seen on endocardial potentials in Uhl’s?

A

Normal transition between the ventricular and atrial complexes

*This helps rule out Ebsteins

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163
Q

True or False: Many patients with Uhl’s anomaly die in infancy or childhood?

A

True

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164
Q

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)

A

Uhl’s Anomaly

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165
Q

True or False: With normal CO, the severity of PS is based on measurements of RV pressure and valve gradient?

A

True

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166
Q

What constitutes mild PS?

A

RV pressure < LV pressure or

Peak valve gradient <35-40mmHg

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167
Q

What constitutes moderate PS?

A

RV pressure 50-75% of LV pressure or

Peak valve gradient 40-60mmHg

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168
Q

What constitutes severe PS?

A

RV pressure >75% of LV pressure or

Peak valve gradient 60-70mmHg

169
Q

What is the modified Bernoulli equation?

A

[4(V2^2 - V1^2)]

170
Q

What is expected in a neonate after balloon valvuloplasty for critical PS?

A
  • 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
Q

What often causes desaturation following balloon valvuloplasty for critical PS?

A

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
Q

True or False: With unilateral branch PS without significant L-R shunting, the resting RVSP will remain normal?

A

True

*Contralateral PA accommodates CO w/o increase in pressure

173
Q

What could be underestimated in unilateral branch PS?

A

Severity of obstruction by systolic pressure difference estimation

*Because flow to stenotic side is lower than normal

174
Q

If doing a balloon angioplasty for unilateral branch PS, what size should the balloon be?

A

3-4 times the narrowest PA segment

*Position the balloon dilation catheter across the stenotic segment of the PA

175
Q

True or False: Percutaneous balloon angioplasty of peripheral PA stenosis has a lower success rate than pulmonary valvuloplasty

A

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
Q

What has to be done for a neonate with PA + IVS prior to any surgical intervention?

A

Cath + Angiography

*Need to confirm coronary circulation

177
Q

Describe RV dependent coronary circulation as seen in PA + IVS

A
  • 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
Q

In normal circulation, what drives coronary blood flow?

A

Aortic diastolic pressure

*Anything that reduces this will compromise coronary blood flow

179
Q

What can happen to the coronary arteries if there are ventriculocoronary artery connections?

A
  • 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
Q

What factor predicts a successful decompression of the RV using radiofrequency ablation and balloon pulmonary valvotomy in patients with PA + IVS?

A

Tricuspid valve Z-score < -2

181
Q

What are the 2 options for PA + IVS if able to decompress?

A
  • Transcatheter perforation of the atretic PV with subsequent balloon dilation
  • Surgical valvotomy
182
Q

What are good features for success of of RV decompression/bi-ventricular repair in PA + IVS?

A
  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
Q

In PA + IVS what are the smallest RVs associated with?

A

Ventriculocoronary connections

*Unipartite or Bipartite ventricles much more likely to have ventriculocoronary communications

184
Q

In PA-IVS, what is a more negative tricuspid Z-score correlated with?

A

Presence of ventriculocoronary connections

185
Q

What cannot be done for a PA + IVS patient with RV-dependent coronary circulation?

A

Decompression of RV (RV-PA conduit or pulmonary valvotomy)

186
Q

If you decompress a PA + IVS patient with RV-dependent coronary circulation, what will you see on ECG?

A

Myocardial ischemia (ST segment elevation) where the RV-dependent coronary circulation is located (regional distribution)

187
Q

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

A

True

188
Q

True or False: In PA + IVS, ventriculocoronary connections are seen in <45% of patients, but only <10% have wholly RV-dependent coronary circulation

A

True

189
Q

True or False: In PA + IVS, there are often MAPCAs

A

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
Q

What are the 3 types of RV seen in PA + IVS?

A
  1. Tripartite
  2. Bipartite
  3. Unipartite
191
Q

True or False: PA + IVS is more common in males?

A

False- No gender predilection

192
Q

What is the ideal goal for PA/VSD + MAPCAs?

A

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
Q

After attempted complete repair for PA/VSD + MAPCAs, what would necessitate VSD to be re-opened?

A
  • If the RVSP >70% systemic

- If central PA size is <50% normal

194
Q

In PA-VSD, the blood supply to lungs is from what?

A

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
Q

In PA-VSD, what is the caliber of the central PAs directly related to?

A

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
Q

In a patients with PA + VSD, where is proximal His bundle relative to the VSD?

A

Along the posterior-inferior rim of the VSD on the LV side

197
Q

Describe the EP anatomy in PA + VSD

A
  • 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
Q

What is the main goal of management in a hypercyanotic spell?

A
  1. Lower PVR

2. Increase SVR

199
Q

List some options for management of a hypercyanotic spell

A
  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
Q

What is a Waterston shunt?

A

Anastomosis of ascending aorta to RPA

201
Q

What is a Potts shunt?

A

Anastomosis of descending aorta to LPA

202
Q

What can a Waterston shunt or Potts shunt result in?

A
  • PA distortion

- Inconsistent transmission of flow/pressure to PA bed

203
Q

What precludes the use of a Waterston shunt or Potts shunt?

A
  • PA stenosis

- Pulmonary vascular disease

204
Q

What type of shunt should you consider a history of in a patient with a significant MPA to RPA gradient?

A

Waterston

205
Q

In a neonate with ToF absent PV, what can be done to help with respiratory failure that isn’t improving despite mechanical ventilation?

A

Prone- Suspend PAs off airway

206
Q

What causes respiratory failure in neonates with ToF absent PV?

A

Central bronchial compression from massively dilated PAs

207
Q

What physiology is a milder care of ToF absent PV similar to?

A

VSD (once PVR drops and L-R shunting increases)

208
Q

What should be done for a baby with ToF who becomes cyanotic with no murmur at 3-4 days of life?

A

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
Q

True or False: A restrictive VSD is common in ToF?

A

False- Very uncommon

210
Q

How is the outlet septum deviated in all patients with ToF?

A

Anterior and cephalad

211
Q

The degree of what determines the severity of subpulmonary obstruction, size of VSD and degree of aortic override in patients with ToF?

A

Anterior/cephalad deviation of the outlet septum

212
Q

What is present in virtually all patients with severe infundibular obstruction and ToF?

A

Large, non-restrictive VSD and prominent overriding aorta

213
Q

A large conal branch or accessory LAD is seen in what % of ToF patients?

A

<15%

214
Q

A L-SVC is found in what % of ToF patients?

A

<10%

215
Q

A continuous murmur on exam is more suggestive of PA + VSD or Truncus?

A

PDA + VSD

216
Q

What causes a continuous murmur in PA + VSD?

A

PDA or systemic collaterals to PAs

*Common

217
Q

True or False: A continuous murmur is uncommon in truncus

A

True

218
Q

What is the usual location of the coronary arteries in truncus?

A
  • Left coronary from left posterolateral truncal surface

- Right from right anterolateral surface

219
Q

Describe coronary findings common in Truncus

A
  • 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
Q

Why is early surgical correction needed in truncus patients?

A

They are at risk for developing pulmonary vascular obstructive disease early

221
Q

What needs to be assessed in an older truncus patient before repair is considered?

A

PVR

222
Q

Above what PVR would a truncus patient likely not be offered surgery?

A

> 8U/m2 with 2PAs

*Some might consider if the patient was under 2 years and the PVR decreases with vasoreactivity testing

223
Q

In a truncus patients with irreversible obstructive pulmonary vascular disease, is transplant an option?

A

No

224
Q

In type 1 Truncus, where do the PAs most commonly arise from?

A

Left posterolateral aspect of truncus (small distance above the truncal valve)

225
Q

What % of patients have Type 1, Type 2 and Type 3 Truncus?

A

1: <50-65%
2: 30-45%
3: 5-10%

226
Q

What is the most common truncal valve morphology?

A

Tricuspid (70%)

227
Q

What is the most common abnormal truncal valve morphology?

A

Quadricuspid (20%)

*Then bicuspid (<10%)

228
Q

True or False: Right arch with mirror-image brachiocephalic branching is commonly associated with truncus

A

True- 35%

229
Q

What arch abnormality can be seen in patients with Truncus and DiGeorge?

A

IAA (10-20% of patients)

230
Q

What % of truncus patients have a secundum ASD?

A

10-20%

231
Q

True or False: Aberrant subclavian, persistent L-SVC to CS and mild tricuspid stenosis are less common abnormalities seen in Truncus?

A

True:

  • Aberrant subclavian: 5-10%
  • Persistent L-SVC to CS: <5% to 10%
  • Mild tricuspid stenosis: <6%
232
Q

IAA in the setting of truncus is frequently associated with what?

A

DiGeorge (22q11 deletion)

233
Q

When could you consider surgical correction for a non-neoate with truncus?

A

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
Q

Describe the 2nd heart sound in truncus

A

Single and loud

235
Q

What might cause a split S2 in truncus?

A

Delayed closure of some of the cusps of the abnormal truncal valve (usually S2 single/loud)

236
Q

When is corrective surgery for truncus preferred?

A

First weeks of life (neonatal period)

237
Q

Why should truncus repair be done early?

A
  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
Q

What type of RV-PA conduit is preferred in Truncus?

A

Valved- Due to pulmonary HTN

239
Q

True or False: Presence of a regurgitant truncal valve is almost always amenable to valve repair?

A

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
Q

What is the most common arrangement of of papillary muscles in the setting of asymmetric congenital mitral stenosis with unbalanced chordal attachment?

A

2 separate papillary muscles

241
Q

What is asymmetric congenital mitral stenosis with unbalanced cord attachment often called?

A

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
Q

What is a classic parachute mitral valve?

A

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
Q

What is it called when mitral valve leaflets are thickened and the chordae are markedly shortened or absent?

A

Mitral arcade or “hammock valve”

244
Q

Describe the leaflet insertion in mitral arcade or “hammock valve”

A
  • 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
Q

True or False: It is common after surgical relief of MS to have pulmonary HTN persist

A

True

246
Q

Where is an isolated cleft of the MV directed?

A

Anteriorly towards LVOT

247
Q

Describe an isolated cleft in the MV

A
  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)
  6. MV annulus commonly dilated
  7. 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
Q

Where is the cleft in the MV associated with AVSD directed?

A

More posterior towards inlet septum

249
Q

What is initial management for patients with moderate MR?

A

Medical- Diuretics and afterload reducing agents (ACEi)

*If severe MR/HF unresponsive to medical management, then surgeryC

250
Q

What do you need to be mindful of in a patient with moderate MR?

A

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
Q

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.

A

MVP

252
Q

What is a small subset of MVP patients at risk for?

A

SCD

253
Q

When do you have to restrict MVP patients to low-intensity 1A sports?

A

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
Q

In neonates with critical AS and a small LV, what factors help decide about Norwood palliation v. 2V repair?

A

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
Q

What are the Rhodes criteria parameters for success of 2V repair in critical AS?

A
  1. Aortic root dimension indexed to BSA
  2. Ratio of long axis of LV to long axis of heart
  3. Indexed MV area
256
Q

What are the CHSC study parameters for success of 2V repair in critical AS?

A
  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
Q

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?

A

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
Q

Asymptomatic children and young adults with Doppler mean gradient of what across AoV should be considered for cardiac cath and possible balloon valvuloplasty?

A

> 40mmHg

259
Q

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?

A

> 30mmHg

260
Q

Balloon valvuloplasty is indicated if a catheter peak to peak gradient across the AoV is what?

A

> 60mmHg

261
Q

Balloon valvuloplasty is indicated of a catheter peak to peak gradient across the AoV is what?

A

> 50mmHg

262
Q

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?

A

> 50mmHg

263
Q

True or False: Valvuloplasty isn’t recommended for asymptomatic patients with peak-to-peak gradients <40mmHg unless cardiac output is impaired

A

True

264
Q

What echo modality is helpful to assess systolic and diastolic dysfunction in patients with AS?

A

TDI

265
Q

Doppler parameters from what have been used for estimation of LV filling pressure?

A

Mitral or pulmonary vein flow

266
Q

What are mitral or pulmonary vein flow Doppler parameters for estimation of LV filling pressure limited by?

A

Measurements are dependent on loading conditions and HR

267
Q

How does TDI work?

A

Directly measures myocardial velocities (typically systolic and diastolic mitral annular velocity)

*Allows quantification of systolic long-axis function and diastolic function

268
Q

What ratio on echo correlates with LVEDP?

A

Early mitral inflow velocity (E) to early diastolic mitral annular velocity (E’)

269
Q

What can measurements of the mitral annular systolic velocity (S’) by TDI show in patients with AS who otherwise have normal EF?

A

Systolic long-axis dysfunction

270
Q

Why might long axis dysfunction precede transverse axis dysfunction in patients with AS?

A
  • 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
Q

What cath peak to peak, mean Doppler and PIG represent mild AS?

A

Peak to Peak: <30mmHg
Mean Doppler: <25mmHg
PIG: <40mmHg

272
Q

What cath peak to peak, mean Doppler and PIG represent moderate AS?

A

Peak to Peak: 30-50mmHg
Mean Doppler: 25-40mmHg
PIG: 40-70mmHg

273
Q

What cath peak to peak, mean Doppler and PIG represent severe AS?

A

Peak to Peak: >50mmHg
Mean Doppler: >40mmHg
PIG: >70mmHg

274
Q

Which AS patients can do all competitive sports?

A
  • Mild stenosis
  • Asymptomatic
  • Normal exercise tolerance
275
Q

Which AS patients can do no sports?

A

Severe stenosis

276
Q

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?

A
  • 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
Q

What do you do to assess activity restrictions in someone with AS/AI?

A

Combine both sets of recommendations

278
Q

Significant retrograde diastolic flow reversal in the distal arch from PDA is consistent with what?

A

Severe stenosis (AS/LVOTO)

279
Q

What factors are associated with more rapid progression of subaortic stenosis?

A
  • Higher initial pressure gradient
  • Short distance between obstructive lesion and AoV
  • Anterior MV leaflet involvement
280
Q

At what stage of AS in infants can EFE/papillary muscle infarction be seen?

A

Severe

*Usually not present in mild forms of disease

281
Q

What can be present in asymptomatic childre with hemodynamically moderate congenital AS?

A

Myocardial fibrosis

282
Q

What are the Doppler estimates of pressure gradients across stenotic valves used for estimation of severity of obstruction dependent on?

A
  • Loading conditions
  • HR
  • Other factors
283
Q

What are the 2 options for infants with severe/critical AS who are candidates for 2V repair?

A
  • Balloon valvuloplasty

- Surgical valvotomy

284
Q

Which has a higher risk for AI, balloon valvuloplasty or surgical valvotomy?

A

Balloon valvuloplasty

285
Q

Which has a higher risk for residual/recurrent stenosis, balloon valvuloplasty or surgical valvotomy?

A

Surgical valvotomy

286
Q

What group of patients are more likely to have complex coarctation with associated lesions?

A

Children who present in infancy (versus older children)

287
Q

What % of patients who require surgical correction before 12 months have a simple CoA?

A

50%

*Remainder with complex CoA

288
Q

In complex CoA, what is the most common associated lesion?

A

VSD

*Perimembranous, muscular or malalignment VSD most common

289
Q

What is common in a critically ill infant with CoA + VSD?

A

Subvalvar AS

-Malalignment VSD may occur with posterior deviation of the conal septum and LVOTO

290
Q

A bicuspid AoV is present in what % of patients with CoA?

A

85%

-Valve may be stenotic or the annulus hypoplastic

291
Q

Besides a bicuspid AoV, what other left sided lesion can occur in patients with CoA?

A

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
Q

What is the association of multiple left sided obstructive lesions with CoA called?

A

Shones

293
Q

How does CoA present in an asymptomatic child?

A

Upper extremity HTN

Heart murmur

294
Q

When is repair recommended for CoA in asymptomatic child without severe upper extremity HTN?

A

2-3 years

295
Q

What is increased if CoA repair is done on a patient <1 year?

A

Risk for late recurrence of CoA

296
Q

The normal descending aorta has attained what % of its final adult diameter by 3 years?

A

<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
Q

Why shouldn’t elective CoA repair be delayed into later childhood or adolesence?

A

There is increased risk for residual HTN and early atherosclerotic CV disease

298
Q

What should you consider with a LA SBP that is >20mmHg higher than the RA or legs?

A

CoA with anomalous aortic arch branching pattern (anomalous right subclavian distal to CoA)

299
Q

True or False: Normal abdominal aortic Doppler profile doesn’t rule out CoA

A

True- If the PDA is patent, pulse wave Doppler can be normal

300
Q

Widely spaced nipples, webbed neck, CoA

A

Turners

301
Q

Genotype in Turner’s

A

45 XO

302
Q

What causes exercise induced UE HTN in repaired CoA?

A

Increase in CoA pressure gradient due to increased Ao blood flow across non-distensible CoA repair site

303
Q

What can be beneficial for patients with exercise HTN, but no significant anatomic stenosis following CoA repair?

A

B-blocker- Will decrease exercise HTN and CoA gradient

304
Q

Long-term prognosis following CoA repair can be adversely affected by what 2 things?

A
  1. Systemic arterial HTN

2. Increase in late premature atherosclerotic disease

305
Q

Resting systolic and diastolic HTN at rest occurs most commonly in what type of CoA repair?

A

Delayed (beyond later childhood)

306
Q

What is the risk for late HTN (even if CoA repaired in infancy)?

A

10-20%

307
Q

What might eh cause of late post-op HTN in patients w/o residual resting CoA relate to?

A

Anatomic/functional changes in arterial vasculature proximal to CoA

308
Q

Acidosis, increasing LFTs and elevated creatine are signs of what in a post-op patient?

A

Inadequate tissue delivery to end organs

309
Q

What is the more effective way to match O2 delivery to changes in O2 consumption in a post-op stage 1 single ventricle?

A

Interventions in total CO or Hgb concentration

-Versus precise manipulation of Qp/Qs

310
Q

How is milrinone used in the setting of an elevated Qp:Qs and compromised systemic perfusion in a stage 1 single ventricle?

A
  • Reduces PVR and can help to increase Qp/Qs

* Also can cause significant hypotension so have to be cautious

311
Q

What would happen if you increased FiO2 or started iNO on a stage 1 single ventricle?

A

Increased PBF at the expense of systemic perfusion

312
Q

What happens if you increase respiratory rate in a stage 1 single ventricle?

A

Drive down pCO2, increase PBR a expense of systemic perfusion

313
Q

In D-TGA, what are anterior malalignment VSDs associated with?

A

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
Q

In D-TGA, what is subaortic stenosis caused by anterior malalignment of the infundibular septum frequently associated with?

A

Aortic arch hypoplasia, CoA or IAA

315
Q

True or False: In d-TGA the sinus node and AV nodes are typically in their normal location

A

True

*Normal atrial anatomy, so sinus an AV nodes are in the usual locations

316
Q

Where is a normal conus located?

A

Subpulmonary, left-sided and anterior

*This prevents fibrous continuity between pulmonary and tricuspid valve rings

317
Q

In D-TGA, where is the conus (or infundibulum) located?

A

Subaortic, right sided and anterior

*This prevents fibrous continuity between aortic and tricuspid valve ring

318
Q

What is D-TGA hypothesized to result from?

A

Abnormal growth and development of the subaortic infundibulum and concurrent absence of growth of the subpulmonary infundibulum

319
Q

Describe the shape of the IVS in D-TGA?

A

Entire septum usually straight (doesn’t have sigmoid curvature of normal heart)

320
Q

What are 2 approaches for a neonate with d-TGA/large VSD and severe LVOTO?

A
  1. Palliate BTT shunt

2. Corrective surgery (Rastelli)

321
Q

How would a neonate with d-TGA/large VSD and severe LVOTO present?

A

Markedly restricted PBF and severe hypoxemia

322
Q

What surgery is most appropriate for d-TGA with a large VSD and extensive LVOTO?

A

Rastelli (intraventricular repair and placement of an extracardiac RV-PA conduit)

*Complete bypass of the LVOTO and anatomic correction of transposition pathology

323
Q

What is an alternative to a Rastelli?

A

REV- Reparation a l’etage ventriculaire

324
Q

What are benefits to a REV?

A
  1. Can be used in younger patients
  2. Avoids prosthetic extracardiac conduit
  3. Avoids intracardiac tunnel obstruction
325
Q

What is a REV?

A
  • 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
Q

What is the technique described by Nikaidoh in relation to D-TGA with VSD and LVOTO?

A

Posterior translocation of the aortic root and coronary arteries with enlargement of the LVOT and conduit placement from RV-PAs anteriorly

327
Q

In cc-TGA, how is the IVS positioned?

A

Sagittal

328
Q

Describe the conduction system in cc-TGA

A
  • 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
Q

What are the expected ECG changes in cc-TGA?

A
  • 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
Q

Where is the AV node in patients with cc-TGA?

A

Along the anterior aspect of the AV ring near the atrial septum

331
Q

What does subaortic stenosis in DORV result from?

A

Extensive hypertrophy of the aortic conus and conal septum or marked malalignment of the conal septum

332
Q

What is there an association with in DORV and subpulmonary VSD?

A

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
Q

True or False: CoA has been described in instances of subaortic, double committed and remote VSD

A

True

334
Q

What does complete correction of DORV depend on?

A

Complexity of the cardiac anatomy

*May have to palliate some infants and small children who are symptomatic in the 1st year of life

335
Q

What AV valve issue makes 2V repair impossible in the setting of DORV?

A

Mitral valve straddling

336
Q

DORV with side-by-side great arteries, large inlet VSD with MV straddle, moderate subaortic stenosis, severe CoA… most appropriate initial surgery?

A

Norwood-Sano

337
Q

Surgical procedure of choice for a patient with DORV and subpulmonary VSD w/o PS?

A

ASO + Patch closure of VSD

338
Q

What is the physiology of DORV + Subpulmonary VSD?

A

TGA

339
Q

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?

A

DORV with subaortic VSD and PS

340
Q

True or False: Patients with subaortic VSDs and PS have varying degrees of cyanosis similar to ToF?

A

True

341
Q

In ToF like DORV, if the PS is severe, what is the clinical presentation?

A
  • 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
Q

DORV + Subpulmonary VSD and no PS presents similarly to what?

A

d-TGA with VSD

343
Q

How does DORV + Subpulmonary VSD and no PS present?

A
  • 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
Q

How does a DORV + Subpulmonary VSD, no PS and associated CoA present?

A

In infancy with heart failure, cyanosis and diminished/absent femoral pulses

345
Q

DORV + Subpulmonary VSD and PS murmur?

A
  • Systolic thrill
  • Loud murmur (grade 3-4/6)
  • 2nd heart sound loud/single (due to proximity of aorta to chest wall)
346
Q

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?

A

BAS

-Physiology like d-TGA and patient needs increased mixing at atrial level

347
Q

Why is systemic outflow tract obstruction in a single ventricle a risk factor for poor outcome at Fontan?

A

Promotes myocardial hypertrophy

348
Q

Why can PA banding cause increased subaortic gradient in a patients with DORV, normally related great arteries and multiple muscular VSDs?

A

Hypertrophy of subaortic conud

349
Q

Why does banding help in the setting of DORV with multiple muscular VSDs or a remote VSD?

A

Reduces pulmonary flow and protects the pulmonary arterioles from obstructive pulmonary arteriopathy

350
Q

What type of DILV is at risk for failing a 2V repair?

A

DILV with a right-sided hypoplastic subaortic RV

351
Q

What should be done for a neonate with DILV with a hypoplastic subaortic RV and restrictive bulboventricular foramen and severe suboartic stenosis?

A

DKS with BTT

*Systemic perfusion at risk and baby needs stable systemic blood supply

352
Q

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?

A

Can cause ventricular hypertrophy and obstruction in patients who have naturally occurring mild restriction at the VSD

353
Q

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?

A

MRI (with concern for partial pericardial defect)

354
Q

What is the best way image with clinical suspicion for a partial pericardial defect?

A

MRI

355
Q

Where are most defects in partial absence of the pericardium?

A

Left side (80%)

356
Q

What causes partial absence of the pericardium?

A

Premature atrophy of the left duct of Cuvier during embryologic development

357
Q

True or False: Most cases of pericardial defect are identified incidentally and symptomatic cases are rare?

A

True

358
Q

What are possible symptoms of a partial defect of the pericardium?

A

Syncope, chest pain, arrhythmia, death

359
Q

What are severe symptoms from a partial defect of the pericardium secondary to?

A

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
Q

What testing modality is ideal for evaluating the pericardium and the procedure of choice for partial absence of the pericardium?

A

MRI

*May see a tongue of pulmonary tissue between the aorta and MPA

361
Q

What could be seen on CXR in complete absence of the pericardium?

A

Leftward displacement of the cardiac border with posterior bulging of the heart

362
Q

True or False: Herniation of the LAA due to absence of the pericardium can be seen on CXR?

A

True- Herniated appendage resembles an enlarged MPA

363
Q

Describe the ECG in absence of the pericardium

A

Often normal

*Can have RBBB or other conduction abnormalities

364
Q

What can be seen on echo for absence of the pericardium?

A
  • RV may be enlarged
  • Excessive cardiac motion
  • LAA may be prominent

*Can’t see actual defect

365
Q

True or False: Cath is helpful to diagnose absence of the pericardium?

A

False- Cath is of little diagnostic value (other than to document coexisting heart disease)

366
Q

True or False: Thorascopy may be necessary to confirm a diagnosis of absence of the pericardium

A

True

367
Q

What factors are associated with increased risk for sudden death in kids with HCM?

A
  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
Q

What is recommended for screening of family members with HCM?

A
  • ECG/Echo every 12-18 months starting no later than 12

- If no LVH by 21, then echo every 5 years

369
Q

What is the known inheritance pattern of HCM?

A

AD

370
Q

Any HCM patient with a history of aborted cardiac death needs what?

A

ICD as primary prevention

371
Q

When do you consider a myectomy in HCM?

A

Severe hypertrophy of septum, significant gradient, symptoms

372
Q

The subaortic gradient and SEM in HCM is what?

A

Dynamic

373
Q

What things reduce the SEM in HCM?

A
  • Decrease myocardial contractility: B-blockers

- Increase ventricular volume or arterial pressure: Squatting, isometric handgrip, phenylephrine

374
Q

What things increase the SEM in HCM?

A
  • Decrease arterial pressure or ventricular volume (Valsalva, nitro)
  • Increase contractility: Standing, amyl nitrate inhalation, isoproterenol, exercise
375
Q

When does sudden death in individuals with HCM most commonly occur?

A

During adolescence and young adulthood (12-35)

*Rarely before 10

376
Q

What are sudden cardiac death events in HCM often due to?

A

Primary ventricular tachycardia and/or ventricular fibrillation

377
Q

What is the relationships between SCD in HCM and exercise?

A
  • 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
Q

What is the most common cause of sudden cardiac death in the young (including athletes)?

A

HCM

379
Q

True or False: Septal myectomy decreases SCD risk in HCM?

A

False: Septal myectomy can improve symptoms, but doesn’t change SCD risk

*To prevent SCD, an ICD is needed

380
Q

True or False: A drop in BP during exercise is associated with an increased risk of sudden death in HCM

A

True

381
Q

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?

A

None… no medical therapy or activity restriction needed

*Low risk patient with no symptoms, negative family history and normal echo

382
Q

How does milrinone work?

A

Phosphodiesterase inhibitor

383
Q

What are the hemodynamic effects of milrinone?

A
  • 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
Q

How do beta blockers effect milrinone?

A

Milrinone retains its full hemodynamic effects in the presence of beta-blocker therapy

385
Q

Skeletal myopathy, congenital dilated cardiomyopathy, short stature, neutropenia

A

Barth syndrome

386
Q

How is Barth syndrome inherited?

A

X-linked

387
Q

What is the overall prognosis in Barth syndrome?

A

Affected individuals die in early childhood

388
Q

How is Barth syndrome diagnosed?

A

UA with elevated 3-mthylglutaconic acid

389
Q

How does Barth syndrome present?

A
  • 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
Q

What is the gene affected in Barth syndrome?

A

Tafazzin (TAZ) on chromosome Xq28

391
Q

What is the problem with the mutation in the TAZ gene leading to Barth?

A

Decreased production of an enzyme needed for synthesis of cardiolipin

392
Q

What is the treatment for Barth?

A

None specifically

-Each problem can individually be controlled

393
Q

What is needed to help differentiate between RCM and CP?

A

Cath

394
Q

What cath sign do RCM and CP have in common?

A

Square root- Early diastolic dip and plateau pattern

395
Q

What are the cath findings in classic RCM?

A

LVEDP, LA pressure and PCWP are all markedly elevated and >4-5mmHg (ideally >10mmHg) greater than the RAP and RVEDP

396
Q

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?

A

Volume loading

397
Q

What is Loeffler endocarditis?

A

Hypereosinophilic syndrome (HES)

398
Q

What type of patients get hypereosinophilic snydrome (HES)?

A

Adult males who live in temperate climates

399
Q

What are the lab seen in HES?

A

Persistent hypereosinophilia: 1500 eosinophils/mm3 for >5 months or until death

400
Q

What organs can be involved in HES?

A

Heart, lungs, bone marrow, brain

401
Q

What is the cause of eosinophilia in HES?

A

Unknown

402
Q

What is seen on cardiac histology in HES?

A

Eosinophilic myocarditis- Inflammatory reaction in the small intramural coronary vessels with thrombosis and fibrinoid change and endocardial mural thrombosis and fibrotic thickening

403
Q

How does HES present?

A

Weight loss, fever, cough, rash, heart failure, systemic embolism

404
Q

What typically causes death in HES?

A

Cardiac manifestations of disease

405
Q

How is HES treated?

A
  • Steroids, hydroxyurea, vincristine

- Digoxin, diuretics, afterload reduction, anticoagulation

406
Q

What are surgical options for HES?

A
  • Mitral/tricuspid valve repair or replacement

- Excision of fibrotic endocardium

407
Q

What happens to the ventricular size and function in RCM?

A
  • Small ventricular volumes
  • Ventricular wall thickness usually normal
  • Normal or near-normal systolic function

*Atria are enlarged

408
Q

True or False: Patients with Duchenne/Becker muscular dystrophy can have severe anesthesia complications including cardiac arrest?

A

True

409
Q

What agent used for anesthesia/induction causes the most complications in patients with Duchenne/Becker muscular dystrophy?

A

Succinylcholine (muscle relaxant that may trigger hyperkalemia)

*Others have been attributed to the use of volatile anesthetic agents

410
Q

What could happen to a Duchenne or Becker muscular dystrophy patients who receives succinylcholine?

A

Reaction similar to malignant hyperthermia

*Develop rhabdo with things like masseter muscle spasm

411
Q

What is the characteristic ECG finding in Duchenne muscular dystrophy?

A

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
Q

What is often seen on Holter in patients with Duchenne muscular dystrophy?

A
  • Automaticity is affected
  • Resting sinus tachycardia
  • Loss of circadian rhythm
  • Reduced heart rate variability
413
Q

What types of dysrhythmias are common in older patients with Duchenne muscular dystrophy?

A

EAT
A-fib
Transient 2/3 degree heart block
Ventricular tachycarias

414
Q

What is concerning for increased risk of sudden death due to v-fib in Duchenne muscular dystrophy?

A

Presence of multiform PVCs and ventricular tachycardia on Holter monitoring

415
Q

Waddling gait, calf pseudohypertrophy?

A

Duchenne

416
Q

What is the typical cardiac exam in Duchenne muscular dystrophy?

A

Normal (even with cardiomyopathy)

  • Occasionally a 3rd/4th heart sound
  • May have neck vein distention of hepatomegaly
417
Q

What can distort the cardiac exam in older patients with Duchenne?

A

Chest wall deformities (scoliosis)

418
Q

What is the most commonly reported arrhythmia in kids with RCM?

A

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
Q

What % of patients with RCM have arrhythmias or conduction disturbances?

A

<15%

Peds Cardiology (20 decks)

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