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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
**Perimembranous** _Conduction system location:_ ?
**Perimembranous** _Conduction system location:_ is on the posterior rim
26
Septum primum
Thin flap
27
Percutaneous closure of PDA in infants Indications and contraindications
Percutaneous closure * Contraindicated in patients \< 6kg * best in a PDA 1.5mm or smaller
28
**Malalignment** Type type of **VSD** - 3 varities ?
**Malalignment Type** a) _Anterior_: tetralogy of Fallot b) _Posterior_: interrupted arch or coarct-VSD c) _Rotational_: Taussig-Bing
29
*Problems* with the **Pott's shunt**
1. L PA _aneurysm_ formation 2. Risk of _stroke_ with shunt take down 3. *Excessive* _pulmonary flo_w
30
*Embryologic* and *anatomic* significance of the _ductus_ in in the devellopment of **coarc of the aorta ?**
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
31
Rate of early, non-facial shunt closure
7%
32
Type C interrupted aortic arch - % of cases, anatomic description ?
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
33
Most common ASD
_Most common_: ostium secundum (fossa ovalis)
34
surgical correcton of PDA in preterminfant long term survival
1-5 year survival is ~50% typically due to unrelated conditions
35
what % of Coarctation are Isolated lesion ?
what % of Coarctation are Isolated lesion: **82%**
36
Coarctation of the aorta - associated abnormalities
* Isolated lesion: 82% * VSD: 11% * other (HLHS): 8% * distal aortic arch narrowing: 50-60% * bicuspid aortic valve: 27-46%
37
**Downs Syndrome** what % of Down’s patients have CHD? what % of the Down’s CHD cases are AVSD?
_Downs Syndrome_ 45% of Down’s patients have CHD 45% of the Down’s CHD cases are AVSD
38
How much of the CO is delivered through a **PDA**
55%
39
Clinical presentation of children with a VSD
Clinical Symptoms: (1) Heart failure: poor feeding, growth failure, tachypnea (2) If PVR \> SVR à Cyanosis (3) Murmur: precordial – pansystolic / hyperactive
40
Medical Support for HLHS
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_
41
what % of Coarctation are associated with VSD ?
what % of Coarctation are associated with VSD : **11%**
42
?% of TET have AVSD?
1% of TET have AVSD
43
**Sinus Venosus Syndrome** in general terms.
**_Sinus Venosus Syndrome:_** _Right upper_ and _middle pulmonary veins_ attach to the *low* _SVC or the SVC/RA junction_
44
Overall classification of **interrupted aortic arch**
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
45
**1st and 2nd** _most common_ types of **VSD**
**Perimembranous** – most common (80%) **Doubly committed subarterial** (10% -second most common)
46
Frequency of **first degree AV block** after repair of **AVSD**
Up to 50% of patients will have first degree AV block after repair
47
**Perimembranous VSD** * Frequency? * Borders ? * Conduction System Location?
**Perimembranous VSD** * _Frequency_: most common (80%) * _Borders_: the tricuspid valve * _Conduction system:_ is on the posterior rim
48
**Interrupted Aortic Arch** Initial _medical_ management?
**Interrupted Aortic Arch** Initial _medical_ management? * PGE-1 (keep duct open) * Oxygenation * Pressors
49
what % of Coarctation are associated with bicuspid aortic valve?
27-46%
50
Repair of Sinus Venosus Syndrome
Sinsus venosus * Path*: Right upper pulmonary vein is shunted to the Right atrium * Repair* : Patch shunting pulmonary vein flow to the left atrium
51
_Aortic valve morphology_ associated with **interrupted aortic arch**
Aortic valve: bicuspid in: 30-59%
52
Indictions For palliative AP shunt
Decreased pulmonary Q and cyanosis SaO2.
53
Factors which increase the risk of mortality with the repair of aortic coarctation ?
Repair of coarctation of the aorta --\> Increased mortality if * distal arch hypoplasia * increased mortality and recurrence if \< 3months
54
Coarctatioin of the aorta Epidemiology - gender association ?
**Coarctatioin of the aorta** 1. Gender: 1. _Isolated_: Male / Female: **2:1** 2. _Coexisting_: Male/Female: **1:1**
55
Embryologic derrivation of the PDA
6th aortic arch
56
Classic BT shunt - technic
Direct anastomosis of the subclavian artery to the PA
57
What is the problem with a waterson shunt
uncontrolled, may lead to pulmonary overflow and pulmonary HTN
58
what % of Coarctation are associated with (HLHS) ?
what % of Coarctation are associated with HLHS: **8%**
59
Stages of PDA closure
**_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
**PDA** is associated with \_\_?\_\_ **prenatal infection**?
*_Rubella_* in the **first trimester**
61
what % of AVSD have an Unroofed coronary siunus?
Unroofed coronary siunus – 3%
62
_surgical correction of **PDA** in preterm infants_ What is the in-hospital mortality?
10-20%
63
**Interrupted Aortic Arch** typical anatomy of the ascending aorta and subsequent branches
Interrupted aortic arch Ascending aorta: about ½ normal diameter and divides into two --\> Branches nl size but .... the Brachiocephalic vessels: Commonly anomalous origins
64
Effect of aneurysm of the membranous septum on a VSD
Aneurysm of the membranous septum functional _reduces the size of the VSD_ but _promotes tricuspid regurgitation_ and is a _nidus for endocarditis_
65
% of AVSD have DORV?
DORV: 2%
66
Sequale of **Eisenmenger Syndrome**
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
Most common cause of death \< 1 month of age
HLHS
68
Potts Shunt
_*Descending* aorta_ to the _Left PA_ Basically a **_P_**otts is a **_P_**FO
69
ASD /TAPVR Contraindications for Surgery
ASD /TAPVR Contraindications for Surgery 1. PVR \> 7 wu
70
Types of **Palliative Operations** - general categories
**_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
% AVSD asspciated with a PDA?
PDA: 10%
72
Indication for surgical repair of a VSD
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
*Surgery* for **Interrupted Aortic Arch ?** * Timing * Surgical Technic
* 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
% of AVSD associated with TET?
TET : 10%
75
Most important risk factor for death after shunting procedure
pulmonary arterial problems
76
Conduction system in AVSD
* **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
Disadvantage of the BT shunt
sacrifices a sublcaivan artery
78
Unroofed Coronary Sinus Syndrome 4 forms:
_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
Epidemiology of Ventricular septal defects
Epidemiology: Most common congenital heart defect 1/100 Births – 25% of all CHD
80
Risks if a VSD does not spontaneously close and is left untreated:
*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
*_Indications_* of the *classic* **BT shunt**
Increases pulmonary artery blood flow in a controlled fashion - most common: 1. TOF 2. Pulmonary atresia 3. Tricuspid atresia
82
**Type A** _interrrupted aortic arch_ - % of cases, and anatomical description
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
what % of all CHD have a PDA?
5-10% of all patients with CHD have a PDA
84
Valve regurgitation and the presentation of an AVSD
A. AV Valve regurgitation – worsens the course and severity a) Moderate – 20% b) Severe – 15%
85
PDA epidemiology - M:F ratio
PDA is 2x as common in females | (think "p" aka concha- DA)
86
Coarcation of the aorta % narrowing needed for hemodynamic significant narrowing
50%
87
Determinants of the clinical presentation of a PDA
The Magnitude of the shunt, which is determined by: 1. size of the PDA 2. PVR
88
ASD /TAPVR **optimal age** for Surgery ?
Age – optimal at 1-2 years
89
Pulmonary trunk in patients with an interrupted aortic arch
Pulmonary trunk : HUGE
90
**Trussler's Rule**
**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
what % of Coarctation are associated with distal aortic arch narrowing?
50-60%
92
Timing of the repair of a coarc aorta in infant with failure?
1. **Neonate with severe failure** – operation a*t 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
Anomalies associated with scimitar syndrome
* Lung hypoplasia * Anomalous arterial supply via the inferior pulmonary ligament * Pulmonary hypertension * **20%** are associated with cardiac and diaphram abnormalities
94
Effect of _prostaglandins_ and _indomethacin_ on **PDA**
PGE1 / PGE2 --\> promote opening Indomethacin ---\>encourage closure
95
**Perimembranous** _Borders_: ?
**Perimembranous** _Borders_: the tricuspid valve
96
PVR where VSD repair is prohibitive
\> 8
97
Outcomes (In hospital mortality) **for type A interrupted aortic arch** 1. Type A:
Outcomes (In hospital mortality) **for type A interrupted aortic arch** Type A: 4%
98
**Interrupted Aortic Arch** Clinical Presentation?
**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
_Symptoms_ of **Coarctaion of the Aorta** in *Childhood* (1-14yo)?
_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
Posterior Malalignment Type VSD
Posterior: interrupted arch or coarct-VSD
101
what % of AVSD have TGA?
TGA – Rare
102
**Interrupted Aortic Arch** - In hospital _Mortality_ for: Type A: Type B: Type C:
Outcomes (mortality) 1. Type A: 4% 2. Type B: 11% 3. Type C: highly lethal
103
coarctation of the aorta 1. Epidemiology 1. % of all CHD?
1. Coarctation is 6.5% of all CHD
104
Rate of closure of a VSD
a) 80% at 1 month b) 60% at 3 months c) 25% at one year
105
% of PDA that are typically closed by 8 weeks
88% are closed
106
ASD /TAPVR Indications for Surgery
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
Anterior Malalignment Type VSD
Malalignment Type a) Anterior: tetralogy of Fallot
108
Waterson shunt
Ascending aorta to right PA
109
Rotational malalignment VSD
Rotational: Taussig-Bing
110
111
Septum secundum
Thick round superior rim of in folded atrial roof
112
Salient features of the morphology of an **interrupted aortic arch**
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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