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Utah / kirklin > Congenital > Flashcards

Congenital Flashcards

1
Q

Murmur of a patient with a VSD

A

Murmur: precordial – pansystolic / hyperactive

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2
Q

Anatomy of a PFO

A

septum primum lies to the left of the PFO (ostium secundum) - fails to fuse with the sides.

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3
Q

surgical correction of PDA in preterm infants

phrenic nerve injury

A

More common than full-term infants (4%)

opposite side injury common

(not surgical damage)

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4
Q

Perimembranous

Frequency: ?

A

Perimembranous

Frequency: most common (80%)

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5
Q

Repair of coarctiaion of the aorta - current mortality for isolated lesions

A

2-10%

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6
Q

Neonatal Presentation of aortic coarc ?

A

Heart failure after a variable time of being well

Once PDA closure –> Heart failure; this is related to the amount of collaterals which have developed

Symptoms:

  1. tachypnea
  2. feeding difficulties
  3. acidosis
  4. bp miss match with decreased or absent femoral pulses
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7
Q

HLHS - frequency of concominant coarctation ?

A

80%

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8
Q

Outcomes (In hospital mortality) for type B interrupted aortic arch ?

A

Outcomes (In hospital mortality) for type B interrupted aortic arch

Type B: 11%

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9
Q

The timing of the repair of an aortic coarctation?

A
  1. Neonate with severe failure: operation at the time of diagnosis
  2. If NO cardiac failure or FTT: 3-6 months

Issues with timing of operation:

a) Increased re-stenosis if operated before 3 months
b) Persistent HTN if operated on after infancy

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10
Q

PDA in preterm infants

indications for surgical intervention

A

Indications for surgical correction of a PDA in a preterm infant

  1. Respiratory Distress
  2. Large PDA
  3. Failure of two courses of inomethacin
  4. NEC (cant give NSAID)
  5. Intracrainial hemorrhage
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11
Q

Interrupted aortic arch is ?% of all CHD

A

1%

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12
Q

Hybrid approach for HLHS

A

the hybrid procedure for hypoplastic left heart physiology was first developed in 1993 in response to poor outcomes following the Norwood procedure.

The hybrid procedure (avoids CPB) :

  1. surgical placement of bilateral branch pulmonary artery bands
  2. placement of a stent in the ductus arteriosus
  3. catheter-based atrial septostomy,
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13
Q

Conal septal VSD

  1. AKA?
  2. Morphology?
  3. Conduction system?
A

Conal septal VSD

  1. AKA: conalseptal, supracristal, infundibular, subpulmonary, doubly committed
  2. Morphology: conjoined leaflets of the aortic and pulmonary valves form a rim
    • may have AI in up to 50% of the cases
  3. Conduction system: remote to the defect
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14
Q

Factors which promote the closure of a VSD

A

those VSD that are muscular or juxtatricuspid are most likely to close spontaneously

Adherence to the tricuspid leaflet and chordal tissue is an important mechanism for perimembranous VSD

  • Can also result in a LV-RA shunting – Gerbode Defect

.

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15
Q

Outcomes (In hospital mortality) for Type C interrupted aortic arch

A

Outcomes (In hospital mortality) for type C interrupted aortic arch

Type C: highly lethal

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16
Q

Unrestricted VSD

A

No resistance to flow across the VSD

the LV and RV pressures become equal

the Qp/Qs become Equal

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17
Q

Type B interrupted aortic arch - % of cases and anatomic description?

A

A. Classification:

  1. Type A: (40%) – distal to the left subclavian
  2. Type B: (55%) – between L common carotid and L subclavian
  3. Type C: (5%) – between the innominate and L common carotid
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18
Q

In what type of patient is the common atrium usually found

A

Heterotaxy

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19
Q

Cardiac Anomalies associated with AVSD ?

A

Associated Cardiac Anomalies

  1. PDA: 10%
  2. TET : 10%
  3. 1% of TET have AVSD
  4. DORV: 2%
  5. TGA – Rare
  6. Unroofed coronary siunus – 3%
  7. Downs Syndrome
    • 45% of Down’s patients have CHD
    • 45% of the Down’s CHD cases are AVSD
    • 75% complete AVSD
    • Partial VSD is rare
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20
Q

Anatomic definition of the PDA

A

Connection between the upper descending aorta and the left PA

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21
Q

Partial AV septal defect

A
  • Formerly known as an Ostium primum defect
  • Usually associated with a cleft in the anterior leaflet of the mitral valve
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22
Q

PDA in preterm infants

What is the frequency of PDA in babies < 1000g?

A

83%

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23
Q

PDA in preterm infants

What is the frequency of a PDA in infants born at < 30 weeks?

A

75%

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24
Q

HLHS - most common

A

Most common is combined aortic and mitral atresia (2/3)

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25
Q

Perimembranous

Conduction system location: ?

A

Perimembranous

Conduction system location: is on the posterior rim

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26
Q

Septum primum

A

Thin flap

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27
Q

Percutaneous closure of PDA in infants

Indications and contraindications

A

Percutaneous closure

  • Contraindicated in patients < 6kg
  • best in a PDA 1.5mm or smaller
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28
Q

Malalignment Type type of VSD - 3 varities ?

A

Malalignment Type

a) Anterior: tetralogy of Fallot
b) Posterior: interrupted arch or coarct-VSD
c) Rotational: Taussig-Bing

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29
Q

Problems with the Pott’s shunt

A
  1. L PA aneurysm formation
  2. Risk of stroke with shunt take down
  3. Excessive _pulmonary flo_w
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30
Q

Embryologic and anatomic significance of the ductus in in the devellopment of coarc of the aorta ?

A
  1. Ductus –> highly muscular
    1. In the normal aorta < 30% of normal circumference
    2. 95% of pathology specimines is comprises the circumference
    3. Aorta proximal to the coarc is abnormal
  2. Normal fetus has decreased blood flow across the aortic isthmus
    1. Increased blood flow across in coarctatioin
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31
Q

Rate of early, non-facial shunt closure

A

7%

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32
Q

Type C interrupted aortic arch - % of cases, anatomic description ?

A

Classification:

  1. Type A: (40%) – distal to the left subclavian
  2. Type B: (55%) – between L common carotid and L subclavian
  3. Type C: (5%) – between the innominate and L common carotid
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33
Q

Most common ASD

A

Most common: ostium secundum (fossa ovalis)

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34
Q

surgical correcton of PDA in preterminfant

long term survival

A

1-5 year survival is ~50%

typically due to unrelated conditions

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35
Q

what % of Coarctation are Isolated lesion ?

A

what % of Coarctation are Isolated lesion: 82%

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36
Q

Coarctation of the aorta - associated abnormalities

A
  • Isolated lesion: 82%
  • VSD: 11%
  • other (HLHS): 8%
  • distal aortic arch narrowing: 50-60%
  • bicuspid aortic valve: 27-46%
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37
Q

Downs Syndrome

what % of Down’s patients have CHD?

what % of the Down’s CHD cases are AVSD?

A

Downs Syndrome

45% of Down’s patients have CHD

45% of the Down’s CHD cases are AVSD

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38
Q

How much of the CO is delivered through a PDA

A

55%

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39
Q

Clinical presentation of children with a VSD

A

Clinical Symptoms:

(1) Heart failure: poor feeding, growth failure, tachypnea
(2) If PVR > SVR à Cyanosis
(3) Murmur: precordial – pansystolic / hyperactive

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40
Q

Medical Support for HLHS

A
  1. PGE-1 - to maintain ductal patency
  2. Keep HCT 45-50%
  3. Keep O2 sat between 70-75%
    • FiO2 only should be 0.18 to 0.21
  4. Maintain PCO2 40-50
  5. Do not perform atrial septostomy preop
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41
Q

what % of Coarctation are associated with VSD ?

A

what % of Coarctation are associated with VSD : 11%

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42
Q

?% of TET have AVSD?

A

1% of TET have AVSD

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43
Q

Sinus Venosus Syndrome in general terms.

A

Sinus Venosus Syndrome: Right upper and middle pulmonary veins attach to the low SVC or the SVC/RA junction

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44
Q

Overall classification of interrupted aortic arch

A

Classification:

  • Type A: (40%) – distal to the left subclavian
  • Type B: (55%) – between L common carotid and L subclavian
  • Type C: (5%) – between the innominate and L common carotid
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45
Q

1st and 2nd most common types of VSD

A

Perimembranous – most common (80%)

Doubly committed subarterial (10% -second most common)

46
Q

Frequency of first degree AV block after repair of AVSD

A

Up to 50% of patients will have first degree AV block after repair

47
Q

Perimembranous VSD

  • Frequency?
  • Borders ?
  • Conduction System Location?
A

Perimembranous VSD

  • Frequency: most common (80%)
  • Borders: the tricuspid valve
  • Conduction system: is on the posterior rim
48
Q

Interrupted Aortic Arch

Initial medical management?

A

Interrupted Aortic Arch

Initial medical management?

  • PGE-1 (keep duct open)
  • Oxygenation
  • Pressors
49
Q

what % of Coarctation are associated with bicuspid aortic valve?

A

27-46%

50
Q

Repair of Sinus Venosus Syndrome

A

Sinsus venosus

  • Path*: Right upper pulmonary vein is shunted to the Right atrium
  • Repair* : Patch shunting pulmonary vein flow to the left atrium
51
Q

Aortic valve morphology associated with interrupted aortic arch

A

Aortic valve: bicuspid in: 30-59%

52
Q

Indictions For palliative AP shunt

A

Decreased pulmonary Q and cyanosis SaO2.

53
Q

Factors which increase the risk of mortality with the repair of aortic coarctation ?

A

Repair of coarctation of the aorta –> Increased mortality if

  • distal arch hypoplasia
  • increased mortality and recurrence if < 3months
54
Q

Coarctatioin of the aorta

Epidemiology - gender association ?

A

Coarctatioin of the aorta

  1. Gender:
    1. Isolated: Male / Female: 2:1
    2. Coexisting: Male/Female: 1:1
55
Q

Embryologic derrivation of the PDA

A

6th aortic arch

56
Q

Classic BT shunt - technic

A

Direct anastomosis of the subclavian artery to the PA

57
Q

What is the problem with a waterson shunt

A

uncontrolled, may lead to pulmonary overflow and pulmonary HTN

58
Q

what % of Coarctation are associated with (HLHS) ?

A

what % of Coarctation are associated with HLHS: 8%

59
Q

Stages of PDA closure

A

First stage

  1. complete 10-15 horus
  2. smooth muscle contraction
  3. intimal cushion apposition

Second stage

  1. Complete at 2-3 weeks
  2. Fiberous poliferation of the intima
  3. Necrosis of the inner layer of the media
  4. Hemorrhage into the wall

*** ocurs from PA to aorta –> residual PDA on the aorta –> Kommerell’s Diverticulum

60
Q

PDA is associated with __?__ prenatal infection?

A

Rubella in the first trimester

61
Q

what % of AVSD have an Unroofed coronary siunus?

A

Unroofed coronary siunus – 3%

62
Q

surgical correction of PDA in preterm infants

What is the in-hospital mortality?

A

10-20%

63
Q

Interrupted Aortic Arch

typical anatomy of the ascending aorta and subsequent branches

A

Interrupted aortic arch

Ascending aorta: about ½ normal diameter and divides into two –> Branches nl size

but …. the Brachiocephalic vessels: Commonly anomalous origins

64
Q

Effect of aneurysm of the membranous septum on a VSD

A

Aneurysm of the membranous septum functional reduces the size of the VSD but promotes tricuspid regurgitation and is a nidus for endocarditis

65
Q

% of AVSD have DORV?

A

DORV: 2%

66
Q

Sequale of Eisenmenger Syndrome

A

in untreated patients:

increased PVR –> high right side pressure –> shunt reversal & Cyanosis

tends to occur in the 3rd and 4th decades of life

Sequale:

  1. Fatal hemotypsis
  2. Polycythemia
  3. Cerebral abscess or infarction
  4. RV failure
67
Q

Most common cause of death < 1 month of age

A

HLHS

68
Q

Potts Shunt

A

Descending aorta to the Left PA

Basically a Potts is a PFO

69
Q

ASD /TAPVR Contraindications for Surgery

A

ASD /TAPVR Contraindications for Surgery

  1. PVR > 7 wu
70
Q

Types of Palliative Operations - general categories

A

Palliative Operations:

  1. Increase pulmonary blood flow (eg: AP shunt)
  2. Decrease pulmonary Q (eg: PA band)
  3. Improve mixing
  4. Reduce ventricular work
71
Q

% AVSD asspciated with a PDA?

A

PDA: 10%

72
Q

Indication for surgical repair of a VSD

A
  1. Large VSD
    • < 3 months - chf / Qp/Qs > 2.1
    • Elective repair at 6 months if PVR < 8.0 wu
  2. Small VSD (QP/Qs<2.1)
    • Endocarditis
    • Cardiac enlargement
    • Any AI
  3. Subarterial VSD - any size
73
Q

Surgery for Interrupted Aortic Arch ?

  • Timing
  • Surgical Technic
A
  • Surgery* for Interrupted Aortic Arch ?
  • Timing
  1. If in good condition operate at the first convenient time
  2. If Unsuitable for surgery – pressors, treat acidosis, and increase the CO2
  • Surgical Technic:
    1. Palliation vs 1 stage correction
74
Q

% of AVSD associated with TET?

A

TET : 10%

75
Q

Most important risk factor for death after shunting procedure

A

pulmonary arterial problems

76
Q

Conduction system in AVSD

A
  • Coronary sinus is displaced inferior
  • AV node displaced inferior
    • Located between the coronary ostium and the ventricular crest
  • Bundle of His courses along the top of the ventricular septum
  • Becomes the right bundle at the midpoint of the crest
    • RBB common after complete repair
    1. Up to 50% of patients will have first degree AV block after repair
77
Q

Disadvantage of the BT shunt

A

sacrifices a sublcaivan artery

78
Q

Unroofed Coronary Sinus Syndrome

4 forms:

A

Unroofed Coronary Sinus Syndrome

4 forms:

  1. Completely unroofed with persistent LSVC
  2. Completely unroofed without persistent LSVC
  3. Partially unroofed mid portion
  4. Partially unroofed terminal portion
79
Q

Epidemiology of Ventricular septal defects

A

Epidemiology:

Most common congenital heart defect

1/100 Births –

25% of all CHD

80
Q

Risks if a VSD does not spontaneously close and is left untreated:

A

Infancy:

Small or moderate VSD: Endocarditis is a rare, but more common with the small or moderate

Large: 9% mortality at 1 year if left untreated

PVR increases with age:

  • AI in the first decade
  • Eisenmenger’s syndrome > 20years old (typically)
  • Infundibular pulmonary stenosis (5%)
81
Q

Indications of the classic BT shunt

A

Increases pulmonary artery blood flow in a controlled fashion - most common:

  1. TOF
  2. Pulmonary atresia
  3. Tricuspid atresia
82
Q

Type A interrrupted aortic arch - % of cases, and anatomical description

A
  1. Type A: (40%) – distal to the left subclavian
  2. Type B: (55%) – between L common carotid and L subclavian
  3. Type C: (5%) – between the innominate and L common carotid
83
Q

what % of all CHD have a PDA?

A

5-10%

of all patients with CHD have a PDA

84
Q

Valve regurgitation and the presentation of an AVSD

A

A. AV Valve regurgitation – worsens the course and severity

a) Moderate – 20%
b) Severe – 15%

85
Q

PDA epidemiology -

M:F ratio

A

PDA is 2x as common in females

(think “p” aka concha- DA)

86
Q

Coarcation of the aorta

% narrowing needed for hemodynamic significant narrowing

A

50%

87
Q

Determinants of the clinical presentation of a PDA

A

The Magnitude of the shunt, which is determined by:

  1. size of the PDA
  2. PVR
88
Q

ASD /TAPVR optimal age for Surgery ?

A

Age – optimal at 1-2 years

89
Q

Pulmonary trunk in patients with an interrupted aortic arch

A

Pulmonary trunk : HUGE

90
Q

Trussler’s Rule

A

Trusler’s rule for the circumference of a PA Band

  • 20mm + 1mm/kg body weight (non mix VSD)
  • 22mm + 1mm/kg bw (pre-fontan)
  • 24mm + 1mm/kg bw (TGA w/ VSD)
91
Q

what % of Coarctation are associated with distal aortic arch narrowing?

A

50-60%

92
Q

Timing of the repair of a coarc aorta in infant with failure?

A
  1. Neonate with severe failure – operation at the time of diagnosis
  2. If NO cardiac failure or FTT à 3-6 months

Issues with timing of operation:

a) Increased re-stenosis if operated before 3 months
b) Persistent HTN if operated on after infancy

93
Q

Anomalies associated with scimitar syndrome

A
  • Lung hypoplasia
  • Anomalous arterial supply via the inferior pulmonary ligament
  • Pulmonary hypertension
  • 20% are associated with cardiac and diaphram abnormalities
94
Q

Effect of prostaglandins and indomethacin on PDA

A

PGE1 / PGE2 –> promote opening

Indomethacin —>encourage closure

95
Q

Perimembranous

Borders: ?

A

Perimembranous

Borders: the tricuspid valve

96
Q

PVR where VSD repair is prohibitive

A

> 8

97
Q

Outcomes (In hospital mortality) for type A interrupted aortic arch

  1. Type A:
A

Outcomes (In hospital mortality) for type A interrupted aortic arch

Type A: 4%

98
Q

Interrupted Aortic Arch

Clinical Presentation?

A

Interrupted Aortic Arch

Presentation - keep in mind the distal perfusion is highly duct dependent

  1. Critically ill patient in severe heart failure
  2. A left to right shunt, with high afterload following closure of the ductus
  3. Decreased pulses, acidosis, and anurea
99
Q

Symptoms of Coarctaion of the Aorta in Childhood (1-14yo)?

A

Childhood (1-14)

  1. HTN – 90% “Hypertension in a 5 year old is never normal “
  2. Cardiomegaly (33%)
  3. Rib notching 15% (>3 years)
  4. LVH on EKG
100
Q

Posterior Malalignment Type VSD

A

Posterior: interrupted arch or coarct-VSD

101
Q

what % of AVSD have TGA?

A

TGA – Rare

102
Q

Interrupted Aortic Arch - In hospital Mortality for:

Type A:

Type B:

Type C:

A

Outcomes (mortality)

  1. Type A: 4%
  2. Type B: 11%
  3. Type C: highly lethal
103
Q

coarctation of the aorta

  1. Epidemiology
    1. % of all CHD?
A
  1. Coarctation is 6.5% of all CHD
104
Q

Rate of closure of a VSD

A

a) 80% at 1 month
b) 60% at 3 months
c) 25% at one year

105
Q

% of PDA that are typically closed by 8 weeks

A

88% are closed

106
Q

ASD /TAPVR Indications for Surgery

A

ASD /TAPVR Indications for Surgery

  • Qp/Qs >= 2
  • Scimitar
  • PAPVR
    • Part of one lung < 1.8 No
    • Whole lung – yes
  • Age – optimal at 1-2 years
  • TVD/MVD
107
Q

Anterior Malalignment Type VSD

A

Malalignment Type

a) Anterior: tetralogy of Fallot

108
Q

Waterson shunt

A

Ascending aorta to right PA

109
Q

Rotational malalignment VSD

A

Rotational: Taussig-Bing

110
Q
A
111
Q

Septum secundum

A

Thick round superior rim of in folded atrial roof

112
Q

Salient features of the morphology of an interrupted aortic arch

A
  1. Aortic valve: bicuspid in 30-59%
  2. Ascending aorta: about ½ normal diameter and divides into two à Branches nl size
  3. Brachiocephalic vessels: Commonly anomalous origins
  4. Descending Aorta: is a continuation of the ductus
  5. Pulmonary trunk : HUGE
  6. Large VSD is always present

Utah / kirklin (12 decks)

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