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Utah / kirklin > Congenital 3 > Flashcards

Congenital 3 Flashcards

1
Q

Surgical plan for correction of subvalvular aortic stenosis

A
  • Diffuse form:
  • (basically a septal myectomy or Ross kono
  • or a Nicks procedure)
    • Aortoseptal approach
      • Preserve the aortic valve if normal
      • Septal resection or patch
      • Ross-Kono procedure vs a mechanical prosthesis
    • Valve-preserving technique
      • Incision through the left and non coronary commissure into the roof of the LA and across the mitral (Nicks procedure?)
      • Replacethe mitral and patch the annulus/roof of the LA
      • Reclose the aorta preserving the aortic root
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2
Q

Ebstein’s Anomaly

Features of the tricupid valve

A
  • Septal leaflet is always affected
  • Posterior leaflet: almost always
  • Anterior leaflet involvement is unusual, but is usually large and sail-like
  • The leaflets are typically adherent to the RV wall
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3
Q

Chamber morphology in Ebstein Anomaly

A
  • RA is dilated, with a PFO or ASD
  • RV is atrerialized: thin and dilated
    • Extreme form is Uhl’s Disease, with a large parachute RV
  • Papillary: Shortened chordae to a small papillary muscle (which can be obstructive)
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4
Q

Ebstein Anomaly

Indications for early repair

A
  1. Anatomic pulmonary atresia
  2. Congestive heart failure
  3. Ventilator dependent
  4. Failed medical therapy
  5. Persistent cyanosis

Usually is two of these at once

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5
Q

GOS Ratio

A

GOS Score: Great Ormond Street ratio

Calculated from 4 chamber echo view

Area of the RA + arterilized RV / area of RV+LA+LV

Radio > 1 correlates with a poor px

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6
Q

General surgical strategy in the treatment of single ventricle syndromes

A
  • First stage:
    • palliation as a neonate
  • Second stage:
    • Palliation during the first year
  • Third stage:
    • Palliation (Fontan) between ages 1-5
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7
Q

Morphologic subsets of single left ventricle

A

Morphologic Subsets of Single ventricle physiology:

  1. Tricuspid atresia
  2. Double inlet left ventricle
  3. Mitral atresia
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8
Q

Tricuspid Atresia - standard anatomic classification

A

Types – Relate to the relationship of the great vessels

Type I (70%): Normally related great vessels

Type II (30%): D-Transposition

Subsets: status of the pulmonary valve

A: Pulmonary atresia

B: Pulmonary Stenosis

C: Normal pulmonary valve

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9
Q

Tricuspid atresia

Subset A

A

Subsets: status of the pulmonary valve

A: Pulmonary atresia

B: Pulmonary Stenosis

C: Normal pulmonary valve

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10
Q

Tricuspid atresia - subset B

A

Subsets: status of the pulmonary valve

A: Pulmonary atresia

B: Pulmonary Stenosis

C: Normal pulmonary valve

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11
Q

Morphogy of the tricuspid atresia physiology

A
  1. Muscular (70%)
    1. AV connection is absent.
  2. Membranous (three variants)
    1. Fiberous diaphragm blocks the AV orifice (imperforate valve membrane )
    2. Classic Ebstein anomaly which is imperforate
    3. AV septal type with imperforate Right side valve
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12
Q

Tricuspid atresia - what is the more comon form of AV valve discontinuity ?

A
  1. Muscular (70%)
    • AV connection is absent.
  2. Membranous (three variants)
    1. Fiberous diaphragm blocks the AV orifice (imperforate valve membrane )
    2. Classic Ebstein anomaly which is imperforate
    3. AV septal type with imperforate Right side valve
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13
Q

Tricuspid atresia - what are the anatomic varriants of the less common type of discontiuity betwen the RA and RV ?

A
  1. Muscular (70%)
    • AV connection is absent.
  2. Membranous (three variants)
    1. Fiberous diaphragm blocks the AV orifice (imperforate valve membrane )
    2. Classic Ebstein anomaly which is imperforate
    3. AV septal type with imperforate Right side valve
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14
Q

How does tricuspid atresia first present?

A

The presentation depends mainly on the subtype

Cyanosis is mainly type 1-

  • Type 1A, 1B, tricuspid atresia
  • DILV with reduced PVF (PS or pulmonary atresia)

Congestive heart failure:

  • Type IIc – tricuspid atresia
  • DILV with TGA from the rudimentary chamber
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15
Q

Tricuspid atresia - which patients typically present with cyanosis ?

A

Cyanosis is mainly type 1-

Type 1A, 1B, tricuspid atresia

DILV with reduced PVF (PS or pulmonary atresia)

Congestive heart failure:

Type IIc – tricuspid atresia

DILV with TGA from the rudimentary chamber

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16
Q

Tricuspid atresia

which variants present with Heart failure?

A

Cyanosis is mainly type 1-

Type 1A, 1B, tricuspid atresia

DILV with reduced PVF (PS or pulmonary atresia)

Congestive heart failure:

Type IIc – tricuspid atresia

DILV with TGA from the rudimentary chamber

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17
Q

Tricuspid Atresia

What is the second procedure?

Timing?

A
  1. Bidirectional caval pulmonary anastomosis
  2. Secondstage paliation is at 3-6 months
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18
Q

What patient is the ideal fontan candidate

A
  1. Age > 2 years
  2. LV morphology
  3. Mean PAP < 15mmHg
  4. PVR < 2WU
  5. Large un-obstruve PA’s
  6. No ventricular hypertrophy
  7. Normal EF (>50%)
  8. LVADP < 12mmHg
  9. No AV valve regurgitation
  10. No subaortic stenosis
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19
Q

Forntan operative mortality

A

< 5%

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20
Q

Fontan - 15 year survival

A

60-70%

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21
Q

Typical presentation of a HLHS

A

Clinical features/Diagnosis

Male (70% are male) presenting as a newborn with cyanosis and tachypnea

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22
Q

Gender predominance with HLHS

A

70% are male

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23
Q

Medical support of HLHS

A
  • PGE-1 for ductal patency
  • Maintain HCT 45-50%
  • Balance pulmonary blood flow: proper respiratory maintenance allows for significant support
    • FiO2 0.18 – 0.21 (not hi fio2) to give a systemic O2 sat of 70-75%
    • Maintain higher than regular PCO2 40-50
  • No atrial septostomy
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24
Q

Role of the ventilator in pre-op HLHS

A

Balance pulmonary blood flow: proper respiratory maintenance allows for significant support

FiO2 0.18 – 0.21 (not hi fio2) to give a systemic O2 sat of 70-75%

Maintain higher than regular PCO2 40-50

25
Q

Diagnosis

A

TGA

26
Q

Surgery for simple TGA

A

Really, the first two

  1. Atrial switch
    • Senning
    • Mustard
  2. Arterial Switch
  3. Intraventricular Tunnel
    • Rastelli
    • Nikaidoh root transoocation
    • Lecompte REV
27
Q

Eponyms for the atrial switch procedure

A
  1. Atrial switch
    • Senning
    • Mustard
28
Q

Senning Procedure

A
  • Senning Atrial Switch:
    • Uses autologous atrial tissue
    • Atrial septal flap and right atrial flap to baffle flow to the correct ventricle
29
Q
  • Mustard Procedure:
A
  • to address TGA
  • Mustard Procedure:
    • Uses pericardial / prosthetic material to baffle flow to the correct ventricle from the IVC/SVC
30
Q

What is the major issues with atrial swtich procedure

A
  • Significant flaw with atrial switch procedures is leaving the RV as the systemic ventricle à and are prone to dysrhythmias
31
Q

congenital corrected TGA

Co-existing abnormalities

A
  • None – 1%
  • VSD – subpulmonary 85%
  • Tricuspid abnormal 90%
    • Significant Tricuspid regurg – 30%
    • Left AV valve (mitral)
    • Ebstein’s anomaly
  • Complete heart block -15%
    • At birth: 5-15%
    • By adulthood: 30%
32
Q

c-TGA

How common is it to have no accompanying abnormality

A

1%​

33
Q

cTGA

Frequency of a VSD ?

A

VSD – subpulmonary 85%

34
Q

cTGA

frequency of tricuspid issues

A

Tricuspid abnormal 90%

Significant Tricuspid regurg – 30%

Left AV valve (mitral)

Ebstein’s anomaly

35
Q

cTGA

frequency of rhythm issues

A

Complete heart block -15%

At birth: 5-15%

By adulthood: 30%

36
Q

cTGA

course of the conduction sytem

A

Conduction system

Runs anterior to the pulmonary valve

Through the RA

Aortic valve, rarely the LV

Anterior around an FO

37
Q

cTGA

General types of Repair

A
  1. Physiologic repair
  2. Anatomic repair
38
Q

cTGA

Steps to the physiologic repair

A

VSD Closure

Relief of pulmonary stenosis

  • LV to PA conduit
  • Posterior spiral-transannular patch

AV valve repair or replacement

Tricuspid annuloplasty in Ebstein’s

39
Q

cTGA

Physiologic repair - operative mortality

A

Operative mortality 10-20%

40
Q

cTGA

Physiologic repair - frequency of iatrogenic heart block

A

Iatrogenic heart block 10-30

41
Q

cTGA

Physiologic repair - 5 year survival

5 year reoperation rate

A

5 year survival: 50-70%

5 year Reoperation: 20-30%

42
Q

cTGA

Anatomic repair

A

Double Switch Procedure

Atrial switch (Senning or Mustard)

Arterial switch or rastelli procedure

+/- Bidirectional cavalpulmonary shunt

43
Q

TGA - arterial switch procedure outcomes

operative mortality

A
  • Operative mortality 2-5%
44
Q

TGA - arterial switch

5 year survival

A

> 90%

45
Q

TGA - arterial switch operation

significant complications

A
  • Neor aortic regurgitaion: 5%
  • Supravalvular PS 10-15%
    *
46
Q

Requirements of a Fontan to work

” original 10 commandments of the Fontan”

A
  1. Age > 4 (2-3 in Utah)
  2. Sinus rhythm
  3. Normal Caval Drainage
  4. Normal volume in the RA
  5. Mean PA pressure =< 15mmHg
  6. PVR < 4units/m2
  7. PA diameter : aorta >= 0.75
  8. LVEF >= 0.6
  9. Competent AV Valve
  10. No shunts
47
Q

Tricuspid atresia

% of all CHD

Relative to other cyanotic defects

A

1-3% of all CHD

thrid most common cardiac dfect

48
Q

Tricuspid atresia

Murmur

A

present in 80% of patients

harsh ejection murmur through the VSD - p

49
Q

Most common single ventricle pathology

A

Tricuspid atresia

50
Q

how does tye 1A tricuspid atresia present ?

A

Cyanosis -

Most tricuspid atresia type 1 presents with cyanosis

51
Q

How does Type 1B, tricuspid atresia present?

A

The presentation depends mainly on the subtype

Cyanosis is mainly type 1-

Type 1A, 1B, tricuspid atresia

DILV with reduced PVF (PS or pulmonary atresia)

Congestive heart failure:

Type IIc – tricuspid atresia

DILV with TGA from the rudimentary chamber

52
Q

how does tricuspid atresia Type IIc present?

A

The presentation depends mainly on the subtype

Cyanosis is mainly type 1-

Type 1A, 1B, tricuspid atresia

DILV with reduced PVF (PS or pulmonary atresia)

Congestive heart failure:

Type IIc – tricuspid atresia

DILV with TGA from the rudimentary chamber

53
Q

Most common cause of delayed paralysis after TAAA repair

A

The most frequent inciting factor for delayed-onset spinal cord injury is postoperative hypotension, which should be considered relative to the patient’s preoperative baseline blood pressure.

Etz CD .. Griep “Paraplegia after extensive thoracic and thoracoabdominal aortic aneurysm repair: does critical spinal cord ischemia occur postoperatively?” JCTVS 2008

54
Q

The frequency of hemodynamically PDA in infants with birth weight < 1750?

A

45%

55
Q

The frequency of PDA in patients with a birthweight < 1200g ?

A

85%

56
Q

Rx for the medical closure of a PDA

A

Indomethacin 0.2mg/kg infused over 20 minutes at 12 and 24 hours.

57
Q

How to address a calcified PDA

A
  1. Sternotomy
  2. CPB
  3. Ductal flow can initially be achieved by inverting LPA while cooling
  4. Cool to 28-32
  5. Patch the PDA
  6. Need DHCA
58
Q

Should a PDA be closed on an asymptomatic adult

A

Yes - Risk of having endocarditis

If feasible, a transcatheter closure should be performed

59
Q

Closing a PDA through a left thoracotomy a tear occurs in the LPA

A

Control bleeding with manual pressure.

Bypass: LA to the Descending aorta

Utah / kirklin (12 decks)

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