Multiple Cardiac Anomalies- Topic 12 Flashcards
Cor Triatriatum
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.
Left Atrium AKA
Cor triatriatum sinistrum
Right Atrium AKA
Cor triatriaum dextrum
Right Atrium AKA
Cor triatriatum dextrum
Right Atrium AKA
Cor triatriatum dextrum
Describe the membrane that separates the atrium into 2 parts in cor triatriatum.
Varies significantly in size and shape
Describe the membrane that separates the atrium into 2 parts in cor triatriatum.
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
Describe the membrane that separates the atrium into 2 parts in cor triatriatum.
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
Cor Triatriatum (Dextrum)
Right atrial
Extremely rare
Cor Triatriatum (Sinistrum)
Left atrial
Misdiagnosed frequently as asthma, mitral stenosis or obstructed pulmonary venous return
Cor Triatriatum (Sinistrum)
Left atrial
Misdiagnosed frequently as asthma, mitral stenosis or obstructed pulmonary venous return
Cor Triatriatum (Sinistrum): Pathophysiology
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
Cor Triatriatum (Sinistrum): Pathophysiology
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
What do others believe that the membrane dividing the left atrium in Cor triatriatum is?
Abnormal growth of the septum primum
What do others believe that the membrane dividing the left atrium in Cor triatriatum is?
Abnormal growth of the septum primum
What unique cases have been reported in cor triatriatum?
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
What unique cases have been reported in cor triatriatum?
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
Cor Triatriatum (Dextrum): Pathophysiology
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
Cor Triatriatum (Dextrum): Pathophysiology
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
Cor Triatriatum (Dextrum): Pathophysiology
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
Why is the morbidity and mortality of cor triatriatum sinistrum high in those who are symptomatic in infancy?
Severely restrictive opening in the accessory membrane and the association with major cyanotic or acyanotic congeital heart lesions
What is the mortality in cor triatriatum sinistrum?
May exceed 75% in untreated symptomatic infants
Severe obstruction = poor prognosis
How soon is surgery performed after diagnosis of cor triatriatum?
Performed soon after diagnosis
What type of surgical entry is performed in correction of cor triatriatum?
Median sternotomy
What are some surgery notes for correcting cor triatriatum?
CPB + XC
XC time is short
What are some surgery notes for correcting cor triatriatum?
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
Cor Triatriatum: Cannulation
Aortic Arterial Bicaval venous (open procedure)
Cor Triatriatum: Cannulation
Aortic Arterial Bicaval venous (open procedure)
Patent Ductus Arteriosus (PDA)
the ductus arteriosus fails to close normally in an infant soon after birth
PDA leads to what?
Abnormal blood flow between the aorta and pulmonary artery (A-P shunt)
PDA Flow
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)
What would cause organ hypoperfusion in PDA?
Extensive aortic runoff w/ low aortic diastolic pressure
What would cause organ hypoperfusion in PDA?
Extensive aortic runoff w/ low aortic diastolic pressure
PDA: Gender
Affects girls more often than boys
PDA: Who is it common in?
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
HLHS
hypoplastic left heart syndrome
TGV/TGA
transposition of the great vessels
PS
pulmonary stenosis
TGV/TGA
transposition of the great vessels
PS
pulmonary stenosis
PDA: Pathophysiology
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
Eisenmenger’s Syndrome
reversal of shunt, may result from several similar abnormalities
Eisenmenger’s Syndrome
reversal of shunt, may result from several similar abnormalities
Goal of Surgical Tx of PDA (Normal otherwise)
(if the rest of circulation is normal or close to normal) is to close the PDA
Goal of Surgical Tx of PDA (other heart problems)
(with problems such as HLHS) the PDA may actually be lifesaving and medicine may be used to prevent it from closing
Can a PDA close on its own?
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
Cath Lab (PDA)
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
OR (PDA)
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
What can be used to close the PDA?
Coil
Surgical ligation
occluder closure
What can be used to close the PDA?
Coil
Surgical ligation
occluder closure
How to keep the PDA open?
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
How to keep the PDA open?
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
What is routinely used in infants with ductus-dependent cardiac lesions to improve circulation prior to balloon atrial septostomy or surgery?
PGE1
How long is therapy with PGE1 continued?
Until balloon atrial septostomy or cardiac surgery is done
When will ductus reopen after starting PGE1?
In most infants, within 30 min to 2 hours after starting PGE1
Will surgical correction of PDA be a pump case when existing alone?
No, done in NICU or Peds ICU
PDA is frequently seen with what?
Other anomalies in surgery
What happens if the balloon procedure fails, when correcting PDA?
An atrial septectomy may have to be done
Done immediately with TAPVR, HLHS
What are the 4 goals of palliative shunts?
Increased pulmonary blood flow
Decrease pulmonary artery blood flow
Improve mixing
Reduce ventricular work
What are the 6 shunts to increase pulmonary blood flow?
Classic Blalock-Taussig shunt Modified Blalock-Taussig shunt Central Waterston Pott's Brock
Classic Black-Taussig Shunt
Subclavian to PA
Modified Blalock-Taussig Shunt
Gore-tex graft
Subclavian to PA
Central
Ascending aorta to main PA (gore-text graft)
Waterston
Ascending aorta to RPA
Pott’s
Descending aorta to LPA
Brock
Pulmonary valvotomy, closed
What shunts are used to decrease pulmonary artery blood flow?
PA banding
Shunts to increase mixing (3)
- Blalock Hanlon
- Rashkind
- Open atrial septectomy
Blalock-Hanlon
Blade septectomy- cath lab procedure
Rashkind
Balloon septostomy
Open Atrial Septectomy
usually a concomitant procedure
Balloon Septostomy (Palliation)
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)
Shunts that Decrease Ventricular Work
Bi-Directional Glenn Shunt
Glenn Shunt
ventricular unloading
ALCAPA
Anomalous Left Coronary Artery to PUlmonary Artery
ALCAPA: WHat is it?
Rare malformation in which the left coronary artery originates from the pulmonary artery
ALCAPA: What does it lead to?
Leads to severe coronary hypoperfusion and left ventricular dysfunction when PVR falls in the postnatal period
ALCAPA: What does it lead to?
Leads to severe coronary hypoperfusion and left ventricular dysfunction when PVR falls in the postnatal period
ALCAPA: Pathophysiology
In fetal/early neonatal life, the LCA from the PA is well tolerated
Why is the LCA from the PA well tolerated in fetal/early neonatal life?
- PAP= systemic pressure (leading to antegrade flow in both the anomalous LCA and the normal RCA)
- PAP
ALCAPA: If LCA is dominant…
and if intercoronary collaterals are inadequate, severe left ventricular dysfunction with ischemic mitral regurgitation develops; the prognosis is poor
ALCAPA: If 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 LCA and the pulmonary artery progressively increases
What is considered to be the standard treatment of ALCAPA?
Surgical correction performed on making a diagnosis of ALCAPA syndrome
WHat is the aim of surgery when correcting ALCAPA syndrome?
To restore a two-coronary-artery circulation system
2 techniques utilized
What are the two techniques of surgically correcting ALCAPA?
- Coronary reimplantation
2. Takeuchi procedure ( creating AP window)
Takeuchi Procedure
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
CPB Considerations: Cannulation ALCAPA
Aortic Arterial Cannulation Bicaval cannulation (open procedure)
ALCAPA Surgery: Temp
Hypothermia: normothermic CPB
Although moderate hypothermia may be necessary to allow low flow bypass if needed
ALCAPA Surgery: 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
ALCAPA Surgery: Cardioplegia
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
ALCAPA Surgery: Cardioplegia
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
