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Congenital Defects & Pediatrics IA > Multiple Cardiac Anomalies- Topic 12 > Flashcards

Multiple Cardiac Anomalies- Topic 12 Flashcards

1
Q

Cor Triatriatum

A

Heart with 3 apparent atria (tri-atrial heart); left atrium or right atrium is divided into 2 parts by a fold of tissue, a membrane, or a fibromuscular band.

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2
Q

Left Atrium AKA

A

Cor triatriatum sinistrum

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3
Q

Right Atrium AKA

A

Cor triatriaum dextrum

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4
Q

Right Atrium AKA

A

Cor triatriatum dextrum

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5
Q

Right Atrium AKA

A

Cor triatriatum dextrum

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6
Q

Describe the membrane that separates the atrium into 2 parts in cor triatriatum.

A

Varies significantly in size and shape

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7
Q

Describe the membrane that separates the atrium into 2 parts in cor triatriatum.

A

Varies significantly in size and shape
May be:
a diaphragm
Funnel-shaped, bandlike, entirely intact (imperforate)
Contains 1 or more openings (fenestrations) ranging from small, restrictive-type to large and widely open

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8
Q

Describe the membrane that separates the atrium into 2 parts in cor triatriatum.

A

Varies significantly in size and shape
May be:
a diaphragm
Funnel-shaped, bandlike, entirely intact (imperforate)
Contains 1 or more openings (fenestrations) ranging from small, restrictive-type to large and widely open

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9
Q

Cor Triatriatum (Dextrum)

A

Right atrial

Extremely rare

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10
Q

Cor Triatriatum (Sinistrum)

A

Left atrial

Misdiagnosed frequently as asthma, mitral stenosis or obstructed pulmonary venous return

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11
Q

Cor Triatriatum (Sinistrum)

A

Left atrial

Misdiagnosed frequently as asthma, mitral stenosis or obstructed pulmonary venous return

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12
Q

Cor Triatriatum (Sinistrum): Pathophysiology

A

Occurs when pulmonary vein fails to incorporate the pulmonary circulation into the left atrium; the result is a septum-like structure that divides the left atrium into 2 compartments

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13
Q

Cor Triatriatum (Sinistrum): Pathophysiology

A

Occurs when pulmonary vein fails to incorporate the pulmonary circulation into the left atrium; the result is a septum-like structure that divides the left atrium into 2 compartments

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14
Q

What do others believe that the membrane dividing the left atrium in Cor triatriatum is?

A

Abnormal growth of the septum primum

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15
Q

What do others believe that the membrane dividing the left atrium in Cor triatriatum is?

A

Abnormal growth of the septum primum

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16
Q

What unique cases have been reported in cor triatriatum?

A

Cases have been reported in which 1 or 2 pulmonary veins drain into the proximal (accessory) chamber and the others drain directly into the true LA

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17
Q

What unique cases have been reported in cor triatriatum?

A

Cases have been reported in which 1 or 2 pulmonary veins drain into the proximal (accessory) chamber and the others drain directly into the true LA

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18
Q

Cor Triatriatum (Dextrum): Pathophysiology

A

During embryogenesis, the original embryologic RA forms the trabeculated anterior portion of the RA; complete persistence of the right sinus valve of embryonic life results in separation of the smooth and trabeculated portions of the right atrium and constitutes cor triatriatum dextrum

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19
Q

Cor Triatriatum (Dextrum): Pathophysiology

A

During embryogenesis, the original embryologic RA forms the trabeculated anterior portion of the RA; complete persistence of the right sinus valve of embryonic life results in separation of the smooth and trabeculated portions of the right atrium and constitutes cor triatriatum dextrum

This forms a sheet that serves to direct the oxygenated venous return from the IVC across the foramen ovale to the left side of the heart. If this membrane is fenestrated and weblike, then it is referred to as the chiari network

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20
Q

Cor Triatriatum (Dextrum): Pathophysiology

A

During embryogenesis, the original embryologic RA forms the trabeculated anterior portion of the RA; complete persistence of the right sinus valve of embryonic life results in separation of the smooth and trabeculated portions of the right atrium and constitutes cor triatriatum dextrum

This forms a sheet that serves to direct the oxygenated venous return from the IVC across the foramen ovale to the left side of the heart. If this membrane is fenestrated and weblike, then it is referred to as the chiari network

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21
Q

Why is the morbidity and mortality of cor triatriatum sinistrum high in those who are symptomatic in infancy?

A

Severely restrictive opening in the accessory membrane and the association with major cyanotic or acyanotic congeital heart lesions

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22
Q

What is the mortality in cor triatriatum sinistrum?

A

May exceed 75% in untreated symptomatic infants

Severe obstruction = poor prognosis

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23
Q

How soon is surgery performed after diagnosis of cor triatriatum?

A

Performed soon after diagnosis

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24
Q

What type of surgical entry is performed in correction of cor triatriatum?

A

Median sternotomy

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25
Q

What are some surgery notes for correcting cor triatriatum?

A

CPB + XC

XC time is short

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26
Q

What are some surgery notes for correcting cor triatriatum?

A

CPB + XC
XC time is short
Surgical correction can be done through the foramen ovalve
Procedure will be quick if pulmonary veins are not involved
Mild to “drift” cooling
Circ arrest if a small child or pulmonary veins involved

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27
Q

Cor Triatriatum: Cannulation

A 
Aortic Arterial
Bicaval venous (open procedure)
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28
Q

Cor Triatriatum: Cannulation

A 
Aortic Arterial
Bicaval venous (open procedure)
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29
Q

Patent Ductus Arteriosus (PDA)

A

the ductus arteriosus fails to close normally in an infant soon after birth

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30
Q

PDA leads to what?

A

Abnormal blood flow between the aorta and pulmonary artery (A-P shunt)

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31
Q

PDA Flow

A

Allows antegrade flow from the RV to aorta prior to birth
If closes: all flow out the aorta
If open: shunt Ao-PA - PDA size determines flow and Qp/Qs
(L –> R due to decreased PVR)

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32
Q

What would cause organ hypoperfusion in PDA?

A

Extensive aortic runoff w/ low aortic diastolic pressure

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33
Q

What would cause organ hypoperfusion in PDA?

A

Extensive aortic runoff w/ low aortic diastolic pressure

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34
Q

PDA: Gender

A

Affects girls more often than boys

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35
Q

PDA: Who is it common in?

A

In premature infants and those with neonatal respiratory distress syndome
Seen in Down’s Syndrome
Common in babies with congenital heart problems, such as hypoplastic left heart syndrome, TGV/TGA, PS

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36
Q

HLHS

A

hypoplastic left heart syndrome

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37
Q

TGV/TGA

A

transposition of the great vessels

38
Q

PS

A

pulmonary stenosis

39
Q

TGV/TGA

A

transposition of the great vessels

40
Q

PS

A

pulmonary stenosis

41
Q

PDA: Pathophysiology

A

If a large PDA is not corrected, then the pressures in the pulmonary arteries may become very high due to volume from the aorta

  • shunt reversal can occur
  • This situation is called Eisenmenger’s Syndrome
42
Q

Eisenmenger’s Syndrome

A

reversal of shunt, may result from several similar abnormalities

43
Q

Eisenmenger’s Syndrome

A

reversal of shunt, may result from several similar abnormalities

44
Q

Goal of Surgical Tx of PDA (Normal otherwise)

A

(if the rest of circulation is normal or close to normal) is to close the PDA

45
Q

Goal of Surgical Tx of PDA (other heart problems)

A

(with problems such as HLHS) the PDA may actually be lifesaving and medicine may be used to prevent it from closing

46
Q

Can a PDA close on its own?

A

Sometimes. Premature babies have a high rate of closure within the first 2 years of life. In full-term infants, a PDA rarely closes on its own after the first few weeks

47
Q

Cath Lab (PDA)

A

transcatheter device
Minimally invasive procedure using a thin, hollow tube. the doctor passes a small metal coil or other blocking device through the catheter to the site of hte PDA. this blocks blood flow through the vessel. such endovascular coils have been used successfully as an alternative to surgery

48
Q

OR (PDA)

A

Surgery may be needed if the catheter procedure does not work or cannot be used. Surgery involves making a small cut between the ribs (thoracotomy) to tie off the PDA

49
Q

What can be used to close the PDA?

A

Coil
Surgical ligation
occluder closure

50
Q

What can be used to close the PDA?

A

Coil
Surgical ligation
occluder closure

51
Q

How to keep the PDA open?

A

Exogenous prostaglandins extends patency of pDA in neonates where bypassing the defective vessel or continued mixing of oxygnated and unoxygenated blood is neeed to provide adequate systemic circulation

*Prostaglandin E1 (PGE1) is known pharmaceutically as alprostadil

52
Q

How to keep the PDA open?

A

Exogenous prostaglandins extends patency of pDA in neonates where bypassing the defective vessel or continued mixing of oxygnated and unoxygenated blood is neeed to provide adequate systemic circulation

*Prostaglandin E1 (PGE1) is known pharmaceutically as alprostadil

53
Q

What is routinely used in infants with ductus-dependent cardiac lesions to improve circulation prior to balloon atrial septostomy or surgery?

A

PGE1

54
Q

How long is therapy with PGE1 continued?

A

Until balloon atrial septostomy or cardiac surgery is done

55
Q

When will ductus reopen after starting PGE1?

A

In most infants, within 30 min to 2 hours after starting PGE1

56
Q

Will surgical correction of PDA be a pump case when existing alone?

A

No, done in NICU or Peds ICU

57
Q

PDA is frequently seen with what?

A

Other anomalies in surgery

58
Q

What happens if the balloon procedure fails, when correcting PDA?

A

An atrial septectomy may have to be done

Done immediately with TAPVR, HLHS

59
Q

What are the 4 goals of palliative shunts?

A

Increased pulmonary blood flow
Decrease pulmonary artery blood flow
Improve mixing
Reduce ventricular work

60
Q

What are the 6 shunts to increase pulmonary blood flow?

A 
Classic Blalock-Taussig shunt
Modified Blalock-Taussig shunt
Central
Waterston
Pott's
Brock
61
Q

Classic Black-Taussig Shunt

A

Subclavian to PA

62
Q

Modified Blalock-Taussig Shunt

A

Gore-tex graft

Subclavian to PA

63
Q

Central

A

Ascending aorta to main PA (gore-text graft)

64
Q

Waterston

A

Ascending aorta to RPA

65
Q

Pott’s

A

Descending aorta to LPA

66
Q

Brock

A

Pulmonary valvotomy, closed

67
Q

What shunts are used to decrease pulmonary artery blood flow?

A

PA banding

68
Q

Shunts to increase mixing (3)

A
  1. Blalock Hanlon
  2. Rashkind
  3. Open atrial septectomy
69
Q

Blalock-Hanlon

A

Blade septectomy- cath lab procedure

70
Q

Rashkind

A

Balloon septostomy

71
Q

Open Atrial Septectomy

A

usually a concomitant procedure

72
Q

Balloon Septostomy (Palliation)

A

Another palliative procedure
Widening of a foramen ovale (PFO) or ASD via cardiac cath (or bedside) using a balloon
This procedure allows a greater amount of oxygenated blood to enter the circulation (improves mixing)

73
Q

Shunts that Decrease Ventricular Work

A

Bi-Directional Glenn Shunt

74
Q

Glenn Shunt

A

ventricular unloading

75
Q

ALCAPA

A

Anomalous Left Coronary Artery to PUlmonary Artery

76
Q

ALCAPA: WHat is it?

A

Rare malformation in which the left coronary artery originates from the pulmonary artery

77
Q

ALCAPA: What does it lead to?

A

Leads to severe coronary hypoperfusion and left ventricular dysfunction when PVR falls in the postnatal period

78
Q

ALCAPA: What does it lead to?

A

Leads to severe coronary hypoperfusion and left ventricular dysfunction when PVR falls in the postnatal period

79
Q

ALCAPA: Pathophysiology

A

In fetal/early neonatal life, the LCA from the PA is well tolerated

80
Q

Why is the LCA from the PA well tolerated in fetal/early neonatal life?

A
  1. PAP= systemic pressure (leading to antegrade flow in both the anomalous LCA and the normal RCA)
  2. PAP
81
Q

ALCAPA: If LCA is dominant…

A

and if intercoronary collaterals are inadequate, severe left ventricular dysfunction with ischemic mitral regurgitation develops; the prognosis is poor

82
Q

ALCAPA: If RCA is dominant….

A

and if collaterals develop efficiently, normal LCA perfusion may be maintained while left-to-right shunt from the right coronary artery to the LCA and the pulmonary artery progressively increases

83
Q

What is considered to be the standard treatment of ALCAPA?

A

Surgical correction performed on making a diagnosis of ALCAPA syndrome

84
Q

WHat is the aim of surgery when correcting ALCAPA syndrome?

A

To restore a two-coronary-artery circulation system

2 techniques utilized

85
Q

What are the two techniques of surgically correcting ALCAPA?

A
  1. Coronary reimplantation

2. Takeuchi procedure ( creating AP window)

86
Q

Takeuchi Procedure

A

Creating an A-P window
a transpulmonaryb affle between the coronary ostium in the PA and the Ao is created.
Baffles (tunnels) blood across aorta –> PA

87
Q

CPB Considerations: Cannulation ALCAPA

A 
Aortic Arterial Cannulation
Bicaval cannulation (open procedure)
88
Q

ALCAPA Surgery: Temp

A

Hypothermia: normothermic CPB

Although moderate hypothermia may be necessary to allow low flow bypass if needed

89
Q

ALCAPA Surgery: Vent

A

a left ventricular vent is inserted through the superior right pulmonary vein (RSPV) and both pulmonary arteries are snared to avoid runoff of coronary perfusion into the pulmonary circulation

90
Q

ALCAPA Surgery: Cardioplegia

A

Aortic root + ostial:
The first cardioplegic administration is performed in the aortic room (and thus right coronary artery) and it is completely by direct administration into hte anomalous LCA (Ostial)

Both RCA and LCA ostial are given for maintenance doses

91
Q

ALCAPA Surgery: Cardioplegia

A

Aortic root + ostial:
The first cardioplegic administration is performed in the aortic room (and thus right coronary artery) and it is completely by direct administration into hte anomalous LCA (Ostial)

Both RCA and LCA ostial are given for maintenance doses

Congenital Defects & Pediatrics IA (17 decks)

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