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Peds > 12. Multiple Cardiac Anomalies- Exam 3 > Flashcards

12. Multiple Cardiac Anomalies- Exam 3 Flashcards

1
Q

Cor Triatriatum=

A

heart with 3 apparent atria (tri-atrial heart)
-the left atrium (cor triatriatum sinistrum) or right atrium (cor triatriatum dextrum) is divided into 2 parts by a fold of tissue, a membrane, or a fibromuscular band

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2
Q

Cor Triatriatum: left atrium is known as

A

cor triatriatum sinistrum

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

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3
Q

Cor Triatriatum: right atrium is known as

A

cor triatriatum dextrum

–extremely rare

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4
Q

Cor Triatriatum- The membrane that separates the atrium into 2 parts varies significantly in size and shape.
•may be what 3 options

A
  1. a diaphragm
  2. funnel-shaped, bandlike, entirely intact (imperforate)
  3. contains 1 or more openings (fenestrations) ranging from small, restrictive-type to large and widely open
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5
Q

when/how does the Cor Triatriatum develop

A

-occurs when the common 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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6
Q

describe the development of the Cor triatriatum dextrum

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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7
Q

he development of the Cor triatriatum dextrum forms a sheet that serves to do what?

A

serves to direct the oxygenated venous return from the IVC across the foramen ovale to the left side of the heart

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8
Q

If sheet formed from the Cor triatriatum dextrum is fenestrated and weblike, then it is referred to as the?

A

Chiari network

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9
Q

Cor Triatriatum mortality rate

A

may exceed 75% in untreated symptomatic infants.

•Severe obstruction = poor prognosis

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10
Q

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

A

due to the severely restrictive opening in the accessory membrane and the association with major cyanotic or acyanotic congenital heart lesions

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11
Q

Cor Triatriatum: surgery notes

A
  • Performed soon after diagnosis
  • Median sternotomy
  • CPB + XC
  • XC time is short
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12
Q

Cor Triatriatum: CPB considerations

A
  • Procedure will be quick if the Pulmonary Veins are not involved
  • Mild to “drift” cooling
  • Circulatory arrest if a small child or Pulmonary veins involved
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13
Q

Cor Triatriatum: arterial and venous cannulation

A

Aortic Arterial cannulation

Bicaval cannulation: (open procedure)

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14
Q

Patent Ductus Arteriosis (PDA)=

A

the ductus arteriosus fails to close normally in an infant soon after birth.
•Leads to abnormal blood flow between the aorta and pulmonary artery (A-P shunt)

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15
Q

Patent Ductus Arteriosis (PDA): describe the flow

A

Allows antegrade flow from the RV to aorta prior to birth

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16
Q

Patent Ductus Arteriosis (PDA): difference in flow if it is open or closed

A

If closes: All flow out the aorta
If open: shunt Ao-PA (L->R due to ↓ PVR)
PDA size determines flow and Qp/QS

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17
Q

Patent Ductus Arteriosis (PDA): Extensive aortic runoff w/low aortic diastolic pressure will cause what?

A

organ hypoperfusion

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18
Q

Patent Ductus Arteriosis (PDA) is common in what demographics (4)

A
  • affects girls more often than boys.
  • common in premature infants and those with neonatal respiratory distress syndrome.
  • seen in Down’s syndrome
  • common in babies with congenital heart problems, such as hypoplastic left heart syndrome (HLHS), transposition of the great vessels (TGV/TGA), and pulmonary stenosis
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19
Q

Patent Ductus Arteriosis (PDA): If a large PDA is not corrected, then what can happen

A

-the pressures in the pulmonary arteries may become very high do to volume from the aorta.
-Shunt reversal can occur
•This situation is called “Eisenmenger’s syndrome”, a condition which may result from several similar abnormalites.

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20
Q

Eisenmenger’s syndrome=

A

Shunt reversal

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21
Q

Patent Ductus Arteriosis (PDA): goal of treatment? what the exception?

A
  • The goal of treatment, if the rest of circulation is normal or close to normal, is to close the PDA.
  • In the presence of certain other heart problems, such as HLHS the PDA may actually be lifesaving and medicine may be used to prevent it from closing
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22
Q

Patent Ductus Arteriosis (PDA): Sometimes, a PDA may close on its own. Premature babies have a high rate of closure within the first __ years of life. In full-term infants, a PDA rarely closes on its own after the first few ____

A

2 years

weeks

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23
Q

Patent Ductus Arteriosis (PDA): treatment via cath lab to close it

A
  • A transcatheter device closure is a minimally invasive procedure that uses a thin, hollow tube.
  • The doctor passes a small metal coil or other blocking device through the catheter to the site of the PDA. This blocks blood flow through the vessel.
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24
Q

Patent Ductus Arteriosis (PDA): treatment via OR to close it

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
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25
Q

Patent Ductus Arteriosis (PDA): treatment to keep it open

A

Prostaglandin E1 (PGE1),is known pharmaceutically as alprostadil

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26
Q

Patent Ductus Arteriosis (PDA): when is PGE1 given

A

prior to balloon atrial septostomy or surgery.

•therapy with PGE1 is continued until balloon atrial septostomy or cardiac surgery is done

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27
Q

Patent Ductus Arteriosis (PDA): how long does it take for PGE1 to take effect

A

the ductus will reopen within 30 minutes to 2 hours after starting PGE1

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28
Q

Patent Ductus Arteriosis (PDA): CPB considerations

A

Not a pump case when existing alone
▫Done in NICU/Peds ICU
Frequently seen with other anomalies in surgery

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29
Q

Patent Ductus Arteriosis (PDA): An _________ may have to be done if the balloon procedure fails. Done immediately with ____ or _____.

A

atrial septectomy

TAPVR, HLHS

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30
Q

Goals of Palliative Shunts:

A
  1. Increase pulmonary blood flow
  2. Decrease pulmonary artery blood flow
  3. Improve mixing
  4. Reduce ventricular work
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31
Q

Shunts to Increase Pulmonary Blood Flow=

A 
Classic Blalock-Taussig shunt
Modified Blalock-Taussig shunt
Central
Waterston
Pott's
Brock
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32
Q

Classic Blalock-Taussig shunt=

A

Subclavian to PA

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33
Q

Modified Blalock-Taussig shunt=

A

(gore-tex graft) Subclavian to PA

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34
Q

Central=

A

Ascending aorta to main PA (gore-tex graft)

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35
Q

Waterston=

A

Ascending aorta to RPA

36
Q

Pott’s=

A

Descending aorta to LPA

37
Q

Brock=

A

Pulmonary valvotomy, closed

38
Q

Brock=

A

Pulmonary valvotomy, closed

39
Q

Shunts to decrease pulmonary artery blood flow=

A

PA Banding

40
Q

Shunts to Increase Mixing=

A

Blalock-Hanlon
Rashkind
Open Atrial Septectomy

41
Q

Blalock-Hanlon=

A

blade septectomy – cath lab procedure

42
Q

Rashkind=

A

balloon septostomy

43
Q

Open Atrial Septectomy=

A

usually a concomitant procedure

44
Q

Balloon Septostomy (Palliation)=

A

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)

45
Q

Shunts to Decrease Ventricular Work:

A

Bi-Directional Glenn Shunt

46
Q

Anomalous Left Coronary Artery to Pulmonary Artery (ALCAPA)=

A

rare malformation in which the left coronary artery originates from the pulmonary artery
• leads to severe coronary hypoperfusion and left ventricular dysfunction when PVR falls in the postnatal period

47
Q

ALCAPA: In fetal/early neonatal life, the left coronary artery (LCA) from the pulmonary artery (PA) is well tolerated because of what 3 things?

A

PAP = systemic pressure (leading to antegrade flow in both the anomalous LCA and the normal RCA)
•When PAP

48
Q

ALCAPA: when PAP = systemic pressure- this leads to what

A

leading to antegrade flow in both the anomalous LCA and the normal RCA

49
Q

ALCAPA: when PAP

A

flow in the LCA decreases and then reverses, which leads to myocardial ischemia and infarction

50
Q

ALCAPA: As long as PVR is high, coronary perfusion is maintained even if what happens?

A

the anomalous artery carries desaturated blood coming from the pulmonary artery. When pulmonary artery pressure falls, perfusion of the left coronary artery becomes inadequate

51
Q

ALCAPA: If the LCA is dominant and if intercoronary collaterals are inadequate–what happens?

A

severe left ventricular dysfunction with ischemic mitral regurgitation develops
–the prognosis is poor

52
Q

ALCAPA: If the RCA is dominant and if collaterals develop efficiently–what happens?

A

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

53
Q

goal of ALCAPA surgery

A

The aim of surgery is to restore a two-coronary-artery circulation system

  • 2 techniques are used
    1. Coronary Reimplantation
    2. Takeuchi procedure
54
Q

Takeuchi procedure=

A

A transpulmonary baffle between the coronary ostium in the PA and the Ao is created.
•Baffles (tunnels) blood across Aorta–>PA

55
Q

ALCAPA: arterial+venous cannulas

A

Aortic Arterial cannulation

Bicaval cannulation: (open procedure)

56
Q

ALCAPA: LV venting

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

57
Q

ALCAPA: cardioplegia

A

Aortic root + ostial:
•The first cardioplegic administration is performed in the aortic root (and thus right coronary artery) and it is completed by direct administration into the anomalous LCA (ostial)
•Both RCA and LCA ostial are given for maintenance doses

58
Q

ALCAPA: temperatures

A

Hypothermia: Normothermic cardiopulmonary bypass

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

59
Q

ALCAPA: It is very useful to create a small ______ as a way to unload the failing left ventricle during the early postoperative period

A

atrial septal defect

60
Q

ALCAPA: terminating bypass

A

After aortic unclamping, a LA line is inserted and weaning from CPB is prepared.
•However, CPB must be prolonged for a while until LA pressure (which is initially high) reaches an acceptably low level, allowing weaning with a moderate inotropic support

61
Q

ALCAPA: when terminating bypass, if the LA pressure does not drop to an acceptable level, what do you do?

A

a left heart VAD or ECMO must be done to allow cardiac assistance for a few days until left ventricular function recovers enough to allow weaning in good hemodynamic conditions.

62
Q

Vascular Rings Double Aortic Arch=

A

Trachea and esophagus are encircled by vascular structures

63
Q

Vascular Rings Double Aortic Arch: Right arch (posterior)=

A

Right common carotid

Right subclavian

64
Q

Vascular Rings Double Aortic Arch: Left aortic arch (anterior)=

A

Left common carotid

Left subclavian

65
Q

Vascular Rings Double Aortic Arch: Surgical division of the vascular ring is indicated in what patients

A

in any patient with symptoms of airway or esophageal compression and in patients undergoing surgery for repair of associated cardiovascular or thoracic anomalies

66
Q

Vascular Rings Double Aortic Arch: Surgical division of the vascular ring is indicated in what patients

A

in any patient with symptoms of airway or esophageal compression and in patients undergoing surgery for repair of associated cardiovascular or thoracic anomalies

67
Q

Vascular Rings Double Aortic Arch: surgical correction steps (3)

A
  1. The chest is entered between the fourth and fifth rib (thoracotomy) (as in the operation for PDA or aortic coarctation)
  2. Upon dissecting the arches off the trachea and esophagus, the arches can be divided. After division the two aortic ends are oversewn with 2 running layers of non-absorbable sutures
  3. Additional relief can be obtained by stitching the lateral wall of the aorta to the adjacent rib to pull it away from the esophagus
68
Q

Vascular Rings Double Aortic Arch: CPB considerations

A

Incision: left lateral thoracotomy
CPB: Off pump

69
Q

Interrupted Aortic Arch (IAA)=

A

absence or discontinuation of a portion of the aortic arch

-3 types

70
Q

Interrupted Aortic Arch (IAA): thought to be a result of faulty development of the aortic arch system during what week of fetal development?

A

fifth to seventh week of fetal development.

–This defect is almost always associated with a large VSD.

71
Q

Interrupted Aortic Arch (IAA)- Type A=

A

interrupted left aortic arch: the arch interruption occurs distal to the origin of the left subclavian artery

72
Q

Interrupted Aortic Arch (IAA)- Type B=

A

interrupted left aortic arch, the interruption occurs distal to the origin of the left common carotid artery.

73
Q

Interrupted Aortic Arch (IAA)- Type C=

A

interrupted left aortic arch, the interruption occurs proximal to the origin of the left common carotid artery

74
Q

An interrupted aortic arch can coexist with any _____

A

ventriculoarterial alignment

75
Q

Interrupted Aortic Arch (IAA): During fetal development: -left ventricular output supplies the?
-right ventricular output supplies the?

A 
left= arterial circulation proximal to the interruption
right= arterial circulation distal to the interruption via the left ductus arteriosus
76
Q

Interrupted Aortic Arch (IAA): Postnatally, this arrangement continues, with the addition of the

A

pulmonary blood flow to the load of the left ventricle

77
Q

Approximately one half of patients with interrupted aortic arch have a hemizygous deletion of a ____ region of chromosome band ____, the most common deletion syndrome in humans

A
  1. 5-3 Mb

22q11. 2

78
Q

Interrupted Aortic Arch (IAA): surgical correction: attention is increasingly focused on the preoperative identification and surgical management of what?

A

the aortic valve and subaortic stenosis found in approximately one half of cases.

79
Q

Interrupted Aortic Arch (IAA): arterial+venous cannula

A

•Arterial: uses 2 aortic cannula: The arterial line is “Y’d” to allow 2 identical cannula (i.e. 2 - 8fr.) to be used to ensure an even split of flow
▫Circulation is divided into upper and lower body
•Venous: Single Venous

80
Q

Interrupted Aortic Arch (IAA): cardioplegia

A

The first cardioplegic administration is performed in the aortic root.
•Subsequent doses may be ostial

81
Q

Interrupted Aortic Arch (IAA): temperatures

A

Hypothermia: DHCA to allow arch and head vessel reconstruction (rarely antegrade cerebral and retrograde cerebral perfusion since head vessels rarely have to be cut off)

82
Q

Interrupted Aortic Arch (IAA): temperatures

A

Hypothermia: DHCA to allow arch and head vessel reconstruction (rarely antegrade cerebral and retrograde cerebral perfusion since head vessels rarely have to be cut off)

83
Q

Aortic Coarctation=

A

due to an abnormality in development of the embryologic left fourth and sixth aortic arches
•Congenital narrowing with varying degrees of hypoplasia can exist

84
Q

Aortic Coarctation: treatment

A

Balloon Aortoplasty

85
Q

Aortic Coarctation: Four predominant repairs

A
  1. Left subclavian patch angioplasty
  2. Resection: End-to-end anastamosis
  3. Subclavian translocation
  4. Patch angioplasty
86
Q

Aortic Coarctation: CPB considerations

A
  • Incision: posterior left lateral thoracotomy
  • Aorta is clamped, cut and re-sewn off CPB
  • CPB: Off pump standby or LHB
  • In rare cases of severe coarctation left heart bypass can be utilized from LA to descending aorta

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