PAPVR/TAPVR- Topic 13 Flashcards
What are some of the variations that can occur in anamolous pulmonary venous return?
- Partially or entirely connected to RA
* Directly connected or connected via systemic venous return
What are some of the variations that can occur in anamolous pulmonary venous return?
- Partially or entirely connected to RA
* Directly connected or connected via systemic venous return
What is a better term for anamous venous return that “return” or “drainage”?
Anamolous connection
What are the two basic classifications of anamalous pulmonary venous connections?
- TAPVC/TAPVR
2. PAPVC/ PAPVR
What are the two basic classifications of anamalous pulmonary venous connections?
- TAPVC/TAPVR
2. PAPVC/ PAPVR
Describe abnormalities associated with TAPVC/TAPVR?
Serious physiologic abnormalities
Describe the abnormalities and symptoms associated with PAPVC/PAPVR?
Mild physiologic abnormality
Cab be asymptomatic
What is TAPVR?
oxygenated blood returns from the lungs back to the right atrium or a vein flowing into the RA not to the left side of the heart (2 entirely separate circulations)
Common Sympoms in TAPVR
Cyanosis Pale, cool, clammy skin Difficult/rapid breathing Tachycardia Poor appetite and insufficient weight gain (FTT) Unusual tiredness or irritability
What is required for survival in TAPVR?
Large ASD or patent foramen ovale
What type of shunt is inherent in TAPVR?
Left to Right shunt (pulmonary veins to the right side)
What type of shunt is needed for survival in TAPVR?
Right to left shunt (via ASD/patent foramen)
Are the shunts in TAPVR cyanotic or acyanotic?
All are cyanotic
Compare the oxygenation in the four chamber in TAPVR?
Identical oxygenation in 4 chambers (w/ ASD)
TAPVR occurs due to abnormal development during the first ______ weeks of pregnancy, when the pulmonary veins are improperly connected
8
What are the 4 different types of TAPVR? How common are they?
Supracardiac (52%)
Intracardiac (30%)
Infracardiac (12%)
Mixed (6%)
What is the most common type of TAPVR?
Supracardiac
How do pulmonary veins drain in supracardiac TAPVR?
Vertical Vein –> Lt Brachiocephalic –> SVC
What do you see on the x-ray in supracardiac TAPVR?
Dilated SVC + Left verical vein (snowman heart)
Increased vasculature
Increased RV volume
What is the second most common type of TAPVR?
Intracardiac TAPVR
How do the pulmonary vein drain in intracardiac TAPVR?
Drains in to coronary sinus or RA
Increased pulmonary vasculature
What is overloaded in intracardiac TAPVR?
RV overload
What percent of I and II TAPVR survive to adults? (The rest die in the first year)?
20%
How do pulmonary veins drain in infracardiac TAPVR?
Long pulmonary veins course down the esophagus
Empty into portal vein or IVC
Veins constricted through the diaphram (obstruction)
What are some symptoms of infracardiac TAPVR?
Severe CHF (obstruction)
Associated with asplenia
Death in a few days
Asplenia
absence of normal spleen function
Asplenia
absence of normal spleen function
Mixed TAPVR
usually a mix of types I, II, and III
What is the severity of mixed TAPVR?
Can vary significantly; all encompassing mix of whatever does not fit in the other classes
Obstructive TAPVR: Severity
Severity depends on whether the pulmonary veins are obstructed
How do pulmonary veins run in obstructed TAPVR?
Into the abdomen, passing through the diaphragm; this squeezes the veins and narrows them, causing the blood to back up into the lungs (RA, RV pressures increase)
When does obstructive TAPVR cause symptoms?
Causes them early! deadly if not recognized and surgically corrected
Obstructive TAPVR (List)
Pulmonary venous HTN and seconary PA & RV HTN Less RV and PA volume overload PUlmonary venous edema more cyanosis and respiratory distress Complete mixing
Non-obstructive TAPVR (List)
Similar hemodynamics to a large ASD L --> shunt magnitude is determined by RV compliance and ASD size Rt heart and pulmonary volume overload Complete mixing at RA level Minimal cyanosis due to large PBF Slight PA pressure elevation
How does blood flow in PAPVR?
Oxygenated blood returns from the lungs via the pulmonary veins back to the right and also to the left atrium. Pulmonary return blood is divided between the LA and RA in the return to the heart
How many PV’s drain in to RA in PAPVR?
One or more (Any mix is possible; ex. 3:1 2:2 etc)
What is the physiologic consequence in PAPVR?
Mild or none
What is PAPVR associated with?
ASD (sinus venosis or secundum)
Surgical Treatment
Balloon atrial septostomy has been used with some success to decompress the venous circuit and improve cardiac output in cases of restrictive interatrial communication (Rashkind)
Non-restrictive ASD
Surgical Tx Goal
recreate an unobstructed venous inflow to the left side chambers and repair of the associated anomalies such as closure of the atrial septal defect (ASD)
When is surgical treatment perfect emergently?
In newborn period for newborns in TAPVR and obstructed pulmonary veins. Some of these children will actually require ECMO prior to surgery because of their marked hemodynamic instability.
What is one the true pediatric emergencies?
Obstructive TAPVR
PAPVC is normally correct ________ complications.
Without
TAPVC still carries significant morbidity and mortality in low volume centers due to what?
Severe hemodynamic and metabolic compromise
What percent of patients undergoing repair of TAPVC require multiple interventions due to recurrent stenosis after initial successful correction?
10-15% (with and increasingly poor outcome at each representation)
When do children with TAPVR without obstruction to pulmonary veins typically undergo surgical repair?
Electively days or weeks after the diagnosis is made
What is the benefit of waiting more than one or two months in children needing surgery?
Although surgery is not emergent, there is generally little benefit to be gained by waiting more than one or two months
In TAPVR, pulmonary veins frequently return to what?
Common confluence behind the LA; surgical repair takes advantage of this
Common PV confluence is connected to the back of the LA, resulting in what?
Normal connected of PV –> LA
Alll other abnormal routes for pulmonary drainge are tied off
Intracardiac Repair AKA
Warden Procedure
Intracardiac Repair AKA
Warden Procedure
What is the prognosis of surgical reapir?
The surgical mortality or detah rate is higher when surgery is performed emergently in critically ill newborns with obstructed pulmonary venous return; this is because they are very sick going to surgery
Critically ill newborns who do survive the surgery may require what?
Prolonged period of post-op intensive care
CPB Conisderations: Cannulation
Arterial: Aortic
Venous:
PAPVR (Larger child) Bicaval
TAPVR (newborn) Single atrial
CPB Considerations: Temperature
Hypothermia: Circ arrest will be utilized partially or completely
DHCA or intermittent depending on exposure and visualization
What changes have been made in surgery regarding circ arrest?
In the past, almost all infants with TAPVC were reapired using profound hypothermia and circ arrest
Now it can be performed with bicaval cannulation and low flow hypothermic perfusion
What is the advantage of circ arrest?
Allowing a bloodless field with excellent exposure of the pulmonary venous confluence without the need for unnecessary manipulation or clamping of the pulmonary veins
In occasion it’s helpful to introduce what?
Brief periods of circ arrest during surgery during hte most critical periods of the operation to optimize surgical exposure with a nearly bloodless field
What else have some centers advocated during CPB in TAPVR?
Use of ECMO during resuscitation
In few cases, ECMO has been adopted after the repair ot support neonates with residual pulmonary HTN
What are some case notes for TAPVR patients?
These children are very sick
Pre and post ECMO are a big possiblity
Birth weights tend to be small
Pulmonary blood flow undergoes a BIG change that can shock the body (Think Qp/Qs)
What type of cases do these TAPVR surgeries tend to be?
Call in cases
Can they stil struggle post septostomy?
Yes
When is ECMO frequent?
Post op
What must decrease in order for complete restoration of normal circulation?
Pulmonary HTN
What are the patients generally with TAPVR?
SMall weight children in severe distress
What may develop in these kids?
Impressive acidosis
