PAPVR/TAPVR Flashcards
2 Basic Classifications OF anomolous pulmonary venous connections:
Total Anomalous (TAPVC / TAPVR)
Serious physiologic abnormalities
Partial Anomalous (PAPVC / PAPVR) Mild physiologic abnormality Can be asymptomatic
TAPVR
Oxygenated blood returns from the lungs back to the right atrium or a vein flowing into the right atrium 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)
The symptoms of anomalous pulmonary venous connections vary for each child, but commonly include:
•Cyanosis (a persistent blue or gray tone to the skin, lips, or nails)
•Pale, cool or clammy skin
•Difficult/rapid breathing
•Tachycardia
•Poor appetite and insufficient weight gain (failure to thrive)
•Unusual tiredness or irritability
TAPVR. what is required for survival?
ASD
TAPVR. If the infant is to live,
a large atrial septal defect (ASD) or patent foramen ovale (passage between the left and right atria) must exist to allow oxygenated blood to flow to the left side of the heart and rest of the body.
This L → R Shunt must have a R → L shunt for survival, that shunt is an ASD
All pulmonary veins shunted
L→R (Lungs → RA)
must have
R→L shunt for survival (ASD) All are cyanotic
oxygenation in 4 chambers with ASD
Identical oxygenation
Total anomalous pulmonary venous return (TAPVR) is what type of defect
a congenital (present at birth) heart defect.
Due to abnormal development during the first 8 weeks of pregnancy,
the pulmonary veins are improperly connected.
anomalous venous connections prevelance for TAPVR
- Supracardiac(52%)
2. Cardiac (30%) 3. Infracardiac (12%) 4. Mixed ( 6%)
anomalous venous connections prevelance for TAPVR
- Supracardiac(52%)
2. Cardiac (30%) 3. Infracardiac (12%) 4. Mixed ( 6%)
supracardiac TAPVR
Most common Pulmonary Veins drain: Vertical vein → Lt Brachiocephalic→ SVC
supracardiac TAPVR what you see in x ray
Dilated SVC + Lt vertical vein (snowman heart) ↑ Vasculature ↑ RV volume
intracardiac tapvr
2nd most common Drains into coronary sinus or RA Increased pulmonary vasculature RV overload
type I and II TAPVR survival to adults
20%. rest die in first year
infracardiac TAPVR
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
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
OBSTRUCTIVE TAPVR severity
The severity of this condition depends on whether the pulmonary veins are obstructed
In obstructed TAVPR, the pulmonary veins run into the abdomen, passing through the diaphragm
In obstructed TAVPR, the pulmonary veins run
into the abdomen, passing through the diaphragm
obstructed TAVPR squeezes
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.
obstructive tapvr consequences
Pulmonary venous hypertension & secondary PA & RV hypertension
Less RV & PA volume overload
Pulmonary venous edema
More cyanosis & respiratory distress
Complete mixing
non obstructive tapvr consequences
Similar hemodynamics to a large ASD
L → R shunt magnitude is determined by RV compliance & ASD size
Rt heart & pulmonary volume overload
Complete mixing at RA level
Minimal cyanosis due to large PBF
Slight PA pressure elevation`
In PAPVR, oxygenated blood returns from the
lungs via the pulmonary veins back to the right and also to the left atrium. In other words, the pulmonary return blood is divided between the LA and RA in the return to the heart.
PAVR one or more
of the 4 PV’s drain to the RA Mild or no physiological consequence Associated w/ASD (sinus venosis or secundum)
papvr mixing possiblities
Any mix is possible Of the 4 PV’s: 3:1 2:2
surgical treatment BAS
Balloon atrial septostomy (BAS) has been used with some success to decompress the venous circuit and improve cardiac output in cases of a restrictive inter-atrial communication.
Since an ASD is imperative to survival – it is better if it is non-restrictive
SURGICAL TREATMENT GOAL
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).
Surgical repair is performed emergently i
in the newborn period for newborns with Total Anomalous Pulmonary Venous Return and obstructed pulmonary veins. Some of these children will actually require extracorporeal life support (ECMO) prior to surgery because of their marked hemodynamic instability
Obstructive TAPVR is one of the true pediatric emergencies
PAPVC is normally corrected
without complications.
TAPVC still carries significant
morbidity and mortality in low volume centers, (due to the severe hemodynamic and metabolic compromise).
10-15% of patients undergoing repair of TAPVC
require multiple interventions due to recurrent
stenosis after initial successful correction, (with an increasingly poor outcome at each representation).
Children with TAPVR without obstruction to the pulmonary veins typically undergo
the pulmonary veins typically undergo surgical repair electively days or weeks after the diagnosis is made.
In these children, although the surgery is not emergent, there is generally little benefit to be gained by waiting more than one or two months.
Children with TAPVR without obstruction to the pulmonary veins typically undergo
the pulmonary veins typically undergo surgical repair electively days or weeks after the diagnosis is made.
In these children, although the surgery is not emergent, there is generally little benefit to be gained by waiting more than one or two months.
In Total Anomalous Pulmonary Venous Return, the pulmonary veins frequently return
to a common confluence behind the LA.
The surgical repair takes advantage of this fact. The common PV confluence is
connected to the back of the LA, resulting in a normal connection of PV->LA
All other abnormal routes for pulmonary venous drainage
(are tied off).
As one might guess, the surgical mortality or death 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
a prolonged period of post-operative intensive care.
cannulation
Arterial: aortic
Venous:
PAPVR (larger child) Bicaval
TAPVR (newborn)SingleAtrial
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
profound hypothermia and circulatory arrest
now it can be performed with bicaval cannulation and low flow hypothermic perfusion.
Circulatory arrest has the advantage of 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 circulatory arrest during the most critical portions of the operation to
optimize surgical exposure with a nearly bloodless field.
Similarly some centers have advocated the use of extracorporeal membrane oxygenation (ECMO) during
resuscitation.
In few cases, ECMO has been adopted after the repair to
support neonates with residual pulmonary hypertension.
case notes
These children are very sick
Pre and post ECMO are a BIG POSSIBILITY
Birth weights tend to be small
The pulmonary blood flow undergoes a BIG change and can shock the body
(think Qp/Qs)
these cases tend to be
call in
Even post septostomy
they can still struggle ECMO post op is “frequent”
Pulmonary hypertension must decrease in order
for complete restoration of normal circulation
Look for small weight children in severe distress. Impressive acidosis may develop
