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Peds: Congenital Defects (exam 3) > Topic 12: Multiple Cardiac Anomalies > Flashcards

Topic 12: Multiple Cardiac Anomalies Flashcards

1
Q

Cor Triatriatum

A
  • cor triatriatum is a heart with 3 apparent atria (tri-atrial heart)
  • congenital anomaly in which 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

CorTriatriatum

•The membrane that separates the atrium into 2 parts varies significantly in size and shape.•may be:

A

▫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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3
Q

CorTriatriatum – Dextrum

A
  • Right Atrial

* extremely rare

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4
Q

CorTriatriatum - Sinistrum

A
  • Left Atrial

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

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5
Q

Cor-Triatriatum sinistrum

•Current theory holds that cor triatriatum sinistrum occurs when what?

A

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

Cor-Triatriatum sinistrum - pathophysiology

how are they occurring?

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.
•Others believe that the membrane dividing the left atrium is an abnormal growth of the septum primum

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7
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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8
Q

Chiari network.

A

cor triatriatum dextrum 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

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9
Q

The morbidity and mortality of cor triatriatum sinistrum??

A

is high in those who are symptomatic in infancy.
▫-this is due to the severely restrictive opening in the accessory membrane and the association with major cyanotic or acyanotic congenital heart lesions.
•Mortality may exceed 75% in untreated symptomatic infants.
•Severe obstruction = poor prognosis

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10
Q

cor triatriatum surgery notes?

when? how? time?

A
  • Performed soon after diagnosis
  • Median sternotomy
  • CPB + XC
  • XC time is short
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11
Q

cor triatriatum surgery notes?

when? how? time?

A 
•Performed soon after diagnosis
•Median sternotomy
•CPB + XC
•XC time is short - Procedure will be quick if the Pulmonary Veins are not involved
-Correction thru the Foramen Ovale
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12
Q

cor triatriatum - CPB Considerations: Cannulation? temp?

A
  • As with ASD’s/VSD’s:
  • Aortic Arterial cannulation
  • Bicaval cannulation :(open procedure)
  • 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

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)
Allows antegrade flow from the RV to aorta prior
to birth
•If closes: All flow out the aorta
•If open: shunt Ao-PA (L->R due to ↓ PVR) PDA size determines flow and Qp/QS
Extensive aortic runoff w/low aortic diastolic pressure will cause organ hypoperfusion

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14
Q

PDAs common in what patient populations?

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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15
Q

If a large PDA is not corrected that what happens?

A

then 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 abnormalities

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16
Q

“Eisenmenger’s syndrome”

A

Shunt reversal can occur with a PDA due to pressures in PAs may become high bc vol in the aorta
•This situation is called “Eisenmenger’s syndrome”, a condition which may result from several similar abnormalities

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17
Q

PDA closure? goal of treatment?

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.
•Sometimes, a PDA may close on its own. 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

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18
Q

PDA CATH closure?

A

CATH: 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. Such endovascular coils have been used successfully as an alternative to surgery.

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19
Q

PDA OR closure?

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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20
Q

PDA keeping the duct open - pharmacologically

A
  • Prostaglandin E 1 (PGE1),is known pharmaceutically as alprostadil
  • Exogenous prostaglandins can be used to artificially extend the patency of the ductus in neonates where bypassing the defective vessel or continued mixing of oxygenated and unoxygenated blood is needed to provide adequate systemic circulation.
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21
Q

PGE1 will reopen PDA how quick?

A

In most infants, the ductus will reopen within 30 minutes to 2 hours after starting PGE

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22
Q

PGE1 is used in infants with PDA why?

A

-PGE1 is routinely used in infants with ductus-dependent cardiac lesions to improve circulation prior to balloon atrial septostomy or surgery.

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23
Q

PDA closure surgical considerations?
done where?
done immediately when?

A

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

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24
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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25
Q

PDA - an atrial septectomy may be done when?

A

•An atrial septectomy may have to be done if the balloon procedure fails

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26
Q

I. Shunts to Increase Pulmonary Blood Flow:

know all these!

A
  • Classic Blalock-Taussig shunt -Subclavian to PA
  • Modified Blalock-Taussigshunt–(gore-tex graft) Subclavian to PA
  • Central-Ascending aorta to main PA (gore-tex graft)
  • Waterston-Ascending aorta to RPA
  • Pott’s-Descending aorta to LPA
  • Brock-Pulmonary valvotomy, closed
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27
Q

•Classic Blalock-Taussig shunt

A

Subclavian to PA

Shunts to Increase Pulmonary Blood Flow

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28
Q

Modified Blalock-Taussigshunt

A

(gore-tex graft) Subclavian to PA

Shunts to Increase Pulmonary Blood Flow

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29
Q

Central shunt

A

Ascending aorta to main PA (gore-tex graft)

Shunts to Increase Pulmonary Blood Flow

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30
Q

Waterston-shunt

A

Ascending aorta to RPA

Shunts to Increase Pulmonary Blood Flow

31
Q

Pott’s shunt

A

Descending aorta to LPA

Shunts to Increase Pulmonary Blood Flow

32
Q

Brock- shunt

A

Pulmonary valvotomy, closed

Shunts to Increase Pulmonary Blood Flow

33
Q

Shunts to decrease pulmonary artery blood flow :

A

PA Banding

34
Q

Shunts to Increase Mixing: 3

A
  • Blalock-Hanlon blade septectomy–cath lab procedure
  • Rashkind- balloon septostomy
  • Open Atrial Septectomy -usually a concomitant procedure
35
Q

Blalock-Hanlon blade septectomy

A

cath lab procedure

Shunts to Increase Mixing

36
Q

Rashkind

A

balloon septostomy

Shunts to Increase Mixing

37
Q

Open Atrial Septectomy

A

usually a concomitant procedure

Shunts to Increase Mixing

38
Q

Balloon Septostomy (Palliation) - does what

A
  • Another Palliative Procedure
  • Balloon septostomy is the 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)
39
Q

Shunts to Decrease Ventricular Work:

A

•Bi-Directional Glenn Shunt

Ventricular Unloading

40
Q

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA)

A
  • -rare malformation in which the left coronary artery originates from the pulmonary artery
  • -this anomaly leads to severe coronary hypoperfusion and left ventricular dysfunction when PVR falls in the postnatal period
41
Q

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is well tolerated bc what?

A

In fetal/early neonatal life, the left coronary artery (LCA)
from the pulmonary artery (PA) is well tolerated
because:
•PAP = systemic pressure
(leading to antegrade flow in both the anomalous LCA and the normal RCA)
•When PAP

42
Q

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), When PAP

A

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

43
Q

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) When PAP = systemic pressure

A

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

44
Q

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) – As long as PVR is high?

A

coronary perfusion is maintained (even if the anomalous artery carries desaturated blood coming from the pulmonary artery.

45
Q

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) - When pulmonary artery pressure falls?

A

perfusion of the left coronary artery becomes inadequate

46
Q

ALCAPA – If the LCA is dominant and if intercoronary collaterals are inadequate

A

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

47
Q

ALCAPA - If the RCA is dominant and if collaterals develop efficiently

A

•If the RCA is dominant and if collaterals develop efficiently, 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

48
Q

ALCAPA - surgical correction goal?

A

Surgical correction performed on making a
diagnosis of ALCAPA syndrome is considered to
be the standard treatment.
•The aim of surgery is to restore a two-coronary-artery circulation system

49
Q

ALCAPA 2 surgical techniques?

A

Technique I:Coronary Reimplantation

Technique II : Takeuchi procedure (creating an A-P window)

50
Q

Technique II for ALCAPA : Takeuchi procedure (creating an A-P window)

A

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

51
Q 
ALCAPA -Takeuchi -Procedure CPB Considerations: Cannulation
arterial 
Venous
Temp
LV Vent
A
  • Aortic Arterial cannulation
  • Bicaval cannulation:(open procedure)
  • Hypothermia: Normothermic cardiopulmonary bypass
  • although moderate hypothermia may be necessary to allow low flow bypass if needed
  • LV Vent: 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.
52
Q

ALCAPA -Takeuchi -Procedure CPB Considerations: Cannulation

LV Vent?

A

•LV Vent: 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.

53
Q

ALCAPA -Takeuchi -Procedure - CPG?

A

Cardioplegia: 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

54
Q

ALCAPA - Takeuchi Procedure - coming off of bypass?

create what?
what support is needed?

A

Coming Off Bypass
•It is very useful to create a small ASD as a way to unload the failing left ventricle during the early postoperative period.
•After aortic unclamping, a LA line is inserted and weaning from cardiopulmonary bypass is prepared.
•However, cardiopulmonary bypass must be prolonged until LAP (which was high) reaches an acceptably low level, allowing weaning with a moderate inotropic support.
•If this is not the case, 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

55
Q

Vascular Rings Double Aortic Arch

•Trachea and esophagus are encircled by vascular structures

A 
•Right arch (posterior)
   ▫Right common carotid
   ▫Right subclavian
•Left aortic arch (anterior)
   ▫Left common carotid
   ▫Left subclavian
56
Q

Vascular Rings Double Aortic Arch - surgical correction indications?

A

Surgical division of the vascular ring is indicated
in any patient with symptoms of airway or
esophageal compression and in patients
undergoing surgery for repair of associated
cardiovascular or thoracic anomalies

57
Q

Vascular Rings Double Aortic Arch - surgical correction how?

A

The chest is entered between the fourth and fifth rib
(thoracotomy) (as in the operation for PDA or aortic coarctation)
•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
•Additional relief can be obtained by stitching the lateral wall of the aorta to the adjacent rib to pull it away from the esophagus

58
Q

Right Arch W/ left PDA Repair - CPB Considerations

A
  • Incision: left lateral thoracotomy

* CPB: Off pump

59
Q

Interrupted Aortic Arch (IAA) is what?

A

•is the absence or discontinuation of a portion of the aortic arch.

60
Q

Interrupted Aortic Arch (IAA) is the result of what?

A

•Interrupted Aortic Arch is thought to be a result
of faulty development of the aortic arch system
during the 5th-7th week of fetal development. This defect is almost always associated with a large VSD

61
Q

Interrupted Aortic Arch (IAA)

•3 types: based on the site of aortic interruption:`

A
  • Type A - interrupted left aortic arch: the arch interruption occurs distal to the origin of the left subclavian artery.
  • Type B - interrupted left aortic arch, the interruption occurs distal to the origin of the left common carotid artery.
  • Type C -interrupted left aortic arch, the interruption occurs proximal to the origin of the left common carotid artery.
  • interrupted aortic arch can coexist with any ventriculoarterial alignment
62
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.

63
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.

64
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.
•interrupted aortic arch can coexist with any ventriculoarterial alignment

65
Q

Interrupted Aortic arch (IAA) - physiology?
during fetal development?
postnatally?

A

During fetal development, left ventricular output
supplies the arterial circulation proximal to the interruption whereas right ventricular output supplies arterial circulation distal to the interruption via the left ductus arteriosus.
•Postnatally, this arrangement continues, with the addition of the pulmonary blood flow to the load of the left ventricle

66
Q

Approximately one half of patients with interrupted aortic arch have what?

A

a hemizygous deletion of a 1.5- 3 Mb region of chromosome band 22q11.2, the most common deletion syndrome in humans

67
Q

Surgical Correction-IAA

A

Surgical reconstruction of the arch is now relatively straightforward;
▫attention is increasingly focused on the preoperative identification and surgical management of the aortic valve and subaortic stenosis found in approximately one half of cases.

68
Q

IAA Correction - Cannulation
Arterial
Venous
Temp?

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
(KNOW)
•Venous: Single Venous

69
Q

IAA Correction - Cannulation

Temp?

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)

70
Q

IAA Correction - Cardioplegia ?

A

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

71
Q

Aortic Coarctation Etiology

A
  • Coarctation is due to an abnormality in development of the embryologic left fourth and sixth aortic arches
  • Congenital narrowing with varying degrees of hypoplasia can exist
72
Q

Aortic Coarctation - Surgical Correction

•Four predominant repairs:

A
    1. Left subclavian patch angioplasty
    1. Resection: End-to-end anastamosis
    1. Subclavian translocation
    1. Patch angioplasty
73
Q

Aortic Coarctation - CPB Considerations ?

incision? Aox? CPB?

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

Peds: Congenital Defects (exam 3) (4 decks)

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