13. TAPVR/PAPVR- Exam 4 Flashcards
Anomalous congenital connections of the
pulmonary venous system represent a
spectrum of conditions in which the…
pulmonary veins are partially or entirely
connected to the right atrium.
–Directly or via the systemic venous return
what better describes the anatomical situation rather than “drainage” or “return”?
Anomalous connection
which one (TAPVR or PAPVR) has Serious physiologic abnormalities
Total Anomalous (TAPVC / TAPVR)
which one (TAPVR or PAPVR) has Mild physiologic abnormality and Can be asymptomatic
Partial Anomalous (PAPVC / PAPVR)
Total Anomalous (TAPVC / TAPVR)=
Oxygenated blood returns from the lungs back to the RA or a vein flowing into the RA and NOT to the left side of heart.
–In other words, blood simply circles to and from the lungs and never gets out to the body. (2 separate circulations)
Total Anomalous (TAPVC / TAPVR) symptoms
- Cyanosis
- Pale, cool or clammy skin
- Difficult/rapid breathing
- Tachycardia
- Failure to thrive
- Unusual tiredness or irritability
what other defect is present with a TAPVR
ASD or PFO
what 4 things must be present with a TAPVR
- All pulmonary veins shunted L→R (Lungs →RA)
- Must have R→L shunt for survival (ASD)
- All are cyanotic
- Identical oxygenation in 4 chambers (w/ASD)
describe the embryology of TAPVR
Due to abnormal development during the first 8 weeks of pregnancy, the pulmonary veins are improperly connected
what are the 4 classifications of TAPVR
Supracardiac
Cardiac
Infracardiac
Mixed
supracardiac TAPVR % occurance
52% –most common
intracardiac TAPVR % occurance
30% –second most common
infracardiac TAPVR % occurance
12%
mixed TAPVR % occurance
6%
with a supracardiac TAPVR, how do the Pulmonary Veins drain
Vertical vein → Lt Brachiocephalic→ SVC
with a supracardiac TAPVR, what 3 things will you see on the X-Ray
Dilated SVC + Lt vertical vein (snowman heart)
↑ Vasculature
↑ RV volume
with a intracardiac TAPVR, how does it drain and what does this cause
Drains into coronary sinus or RA
- -Increased pulmonary vasculature
- -RV overload
what of % Type I and II TAPVR survive to adults (the rest die in 1st year)
only 20%
with a infracardiac TAPVR, how does it drain and what does this cause
- Long pulmonary veins course down the esophagus
- Empty in portal or IVC
- Veins constricted thru diaphragm (obstructive)
- Severe CHF (obstructive)
- Associated w/asplenia
- Death in a few days
describe a mixed TAPVR
- Usually a mix of types I,II and III
- Severity can vary significantly
- All encompassing mix of whatever does not fit in the other classes
The severity of TAPVR depends on what?
whether the pulmonary veins are obstructed
describe obstructed TAVPR
- the pulmonary veins run 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).
- Causes symptoms early - deadly if not recognized and surgically corrected
Does this describe obstructed or non-obstructed TAPVR? Pulmonary venous HTN & secondary PA & RV
HTN
Obstructed TAPVR
Does this describe obstructed or non-obstructed TAPVR? Similar hemodynamics to a large ASD
NON-Obstructed TAPVR
Does this describe obstructed or non-obstructed TAPVR? L → R shunt magnitude is determined by RV compliance & ASD size
NON-Obstructed TAPVR
Does this describe obstructed or non-obstructed TAPVR? Less RV & PA volume overload
Obstructed TAPVR
Does this describe obstructed or non-obstructed TAPVR? Rt heart & pulmonary volume overload
NON-Obstructed TAPVR
Does this describe obstructed or non-obstructed TAPVR? Pulmonary venous edema
Obstructed TAPVR
Does this describe obstructed or non-obstructed TAPVR? Complete mixing at RA level
NON-Obstructed TAPVR
Does this describe obstructed or non-obstructed TAPVR? Minimal cyanosis due to large PBF
NON-Obstructed TAPVR
Does this describe obstructed or non-obstructed TAPVR? More cyanosis & respiratory distress
Obstructed TAPVR
Does this describe obstructed or non-obstructed TAPVR? Slight PA pressure elevation
NON-Obstructed TAPVR
Does this describe obstructed or non-obstructed TAPVR? Complete mixing
Obstructed TAPVR
Does this describe obstructed or non-obstructed TAPVR? Complete mixing
Obstructed TAPVR
Partial Anomalous (PAPVC / PAPVR)=
-oxygenated blood returns from the lungs via the pulmonary veins back to the RA ans LA.
-In other words, the pulmonary return blood is divided
between the LA and RA in the return to the heart.
what is a PAPVR associated with
ASD (sinus venosis or secundum)
with a PAPVR, how many of the PV’s drain into the RA
One or more of the 4 PV’s drain to the RA
what has been used with some success to decompress the venous circuit and improve cardiac output in cases of a restrictive inter-atrial communication.
Balloon atrial septostomy (BAS)
Since an ASD is imperative to survival – it is better if it is restrictive or non-restrictive
non-restrictive
what is the goal of surgical treatment
The goal of surgical repair is to recreate an unobstructed venous inflow to the left side chambers and repair of the associated anomalies, such as closure of atrial septal defect (ASD).
when is surgical repair performed emergently
in the newborn period for newborns with 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 of the true pediatric emergencies
Obstructive TAPVR
_____ is normally corrected without complications.
PAPVC
_____ still carries significant morbidity and mortality in low volume centers, (due to the severe hemodynamic and metabolic compromise).
TAPVC
____% of patients undergoing repair of TAPVC require multiple interventions due to recurrent ____ after initial successful correction, (with an ______ poor outcome at each representation).
10-15%
stenosis
increasingly
when do Children with TAPVR without obstruction to
the pulmonary veins typically undergo surgical repair electively
days or weeks after the diagnosis is made.
–although the surgery is not emergent, there is generally little benefit to be gained by waiting more than one or two months.
In TAPVR surgical repair, the pulmonary veins frequently return to a common confluence behind the?
LA
- -resulting in a normal connection of PV->LA
- -All other abnormal routes for pulmonary venous drainage are tied off.
what cases have the higher surgical mortality or death rate
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?
a prolonged period of post-operative intensive care
CPB: Arterial + Venous Cannulas
Arterial: aortic
Venous:
–PAPVR (larger child) Bicaval
–TAPVR (newborn) Single Atrial
CPB: Temperatures
- Hypothermia: Circulatory arrest will be utilized partially or completely
- -DHCA or intermittent depending on exposure and visualization
In the past, almost all infants with TAPVC were repaired using _____ and ______. Now it can be performed with ______ and _______ perfusion.
profound hypothermia and circulatory arrest
bicaval cannulation and low flow hypothermic
what is the benefit of using 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
what is the benefit of using 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
On occasion, it is helpful to introduce brief periods of _______ during the most critical portions of the operation to optimize surgical exposure with a nearly bloodless field
circulatory arrest
Similarly some centers have advocated the use of ____ during resuscitation. In few cases, it has been adopted after the repair to support neonates with residual _____.
ECMO
pulmonary hypertension
Case Notes: Pre and Post ____ is a big possibility
ECMO
Case Notes: Birth weights tend to be ____
small
Case Notes: The pulmonary blood flow undergoes a BIG change and can _____ the body
shock
think Qp/Qs
Case Notes: These cases tend to be _____ cases
“CALL IN”
Case Notes: Even post septostomy, they can still _____
struggle
Case Notes: Pulmonary HTN must ______ in order for complete restoration of normal circulation
decrease
Case Notes: Look for small weight children in severe ____
distress
Case Notes: Impressive _____ may develop
acidosis
