One surgeon’s observations on

neonatal surgery for Complete

Atrio-Ventricular Canal

Redmond P. Burke, M.D., FACS

Chief, Division of Cardiovascular Surgery
The Congenital Heart Institute
Miami Children’s Hospital and Arnold Palmer Hospital
www.pediatricheartsurgery.com
 Factors influencing timing of surgery
for CAVC
 The combination of shunting at atrial and ventricular levels, with an
additional volume load produced by AV valve regurgitation, produces
symptomatic heart failure in infancy.
 Yamaki et al reported early pulmonary vascular changes in children with
AVC and recommended repair AVC within the first 6 months of life to
prevent long-term morbidity.
 Children with Down syndrome are more vulnerable to early pulmonary
vascular changes. Therefore, early repair of AVC to prevent permanent
pulmonary vascular changes in children with Down syndrome has been the
practice in many surgical centers.
 Hanley et al reviewed the results of surgical repair of AVC and found a
drop in mortality from 25% to 3% over the last 20 years and provided
support for aggressive approach for repair in the first 3 months of life. (and
that was about thirteen years ago)
 Over the past decade, we have moved to earlier repairs
for CAVC.

Age at Surgery as Related to Surgical Date

350

300

250

200

150

100

50

0
6/15/1994 10/28/1995 3/11/1997 7/24/1998 12/6/1999 4/19/2001 9/1/2002 1/14/2004 5/28/2005 10/10/2006

Median age at surgery: 1995-1999: 165 days; 2000-2005: 125 days

Median POS: 1995-1999: 8 days; 2000-2005: 7 days
Given these pressures to repair earlier, particularly in an
era of early complete repair for more complex lesions,
why are we not performing routine neonatal AVC
repairs?
 We do successfully operate on neonates with CAVC, although
usually when they have other problems
 Arch obstruction
 AV valve insufficiency
 FTT (feeding tubes and g-tubes are a good indication for surgery)
 Absent these indications, there may be some good technical
reasons to wait a few months for complete repair
 Technical thoughts pertinent to
neonatal CAVC repair

1. Pulmonary artery banding for CAVC is

extraordinarily rare, or should be.
 Maybe for multiple muscular VSD’s?
 Maybe for single ventricle path
Are we not performing neonatal repair because it would
require a long period of circulatory arrest? My fingers
as size reference.
 No, the CAVC repair can be readily performed without circ
arrest, without a cluttered or bloody operative field. Again, that’s
my fingertip, and the initial image of the CAVC defect with stay
sutures in the valve leaflets.
I’m preparing to divide the superior
leaflet between stay sutures.
The Dacron VSD patch is secured with running
suture, then the valves will be reattached to the
crest of the VSD patch.
 Final finger shot, the atrial component of
the CAVC has been closed with the child’s
own pericardium.
 The Achilles heel of these neonatal repairs
is the quality of the valve reconstructions.
 There may be a tradeoff when
reconstructing a neonatal left AV
valve, particularly one with pre-
existing severe MR.
 Technical maneuvers to decrease MR
tend to produce MS
 Commissural plication
 Cleft closure
 Ring insertion
 In some forms of AVC, the cleft is
the main opening of the AV valve
 The left lateral leaflet (what would be
the posterior leaflet in a normal
valve) forms much less of the valve
circumference.
 Valves with single or closely spaced
papillary muscles, or double orifices,
may rely on the cleft for valve
opening.
Valve repair at 4 days is significantly different
than at 4 months
 1 mo Downs 2.6kg • 4 mo Downs 5kg
The difference between sewing Jello™ and sewing cloth. Look at the
newborn’s tissue on the left, versus the 4 month old’s on the right.
2 days old 4 months old
 Why do you have to get the repair
right?
 Plan B isn’t great – there is no room
for a mechanical valve in a newborn’s
mitral annulus.
 The LA hasn’t had time to enlarge
 The pulmonary veins will be
obstructed
 The LVOT will be obstructed
 You can easily hit the conduction
system
 You can easily hit the circumflex
coronary artery
 Mitral Valve Prosthetic Rings aren’t
an option either, although considered
essential for adult valvuloplasty, they
would restrict annular growth and you
can’t buy one of the shelf in these
sizes anyway)
 Patient Valve Mismatch is
Automatic in neonatal AV valve
replacement
What are the clinical effects of patient-prosthesis mismatch?
Patient-prosthesis mismatch generates high transvalvular pressure gradients through normally
functioning prosthetic valves. Patient-prosthesis mismatch has been associated with:
Higher transprosthetic pressure gradients1, 3, 4, 8
LV outflow obstruction and persistent LV
hypertrophy1, 3-5, 7-9
Decreased late survival1, 6, 7
Decreased NYHA functional class improvement1, 7
Sudden death6, 8, 10-12
Decreased quality of life4, 11
Higher incidence of late adverse complications4, 13
Increased early and late mortality2, 5
Increased bleeding complications14
Increased risk of congestive heart failure15
Increased risk of reoperation16

Increased Risk of Mortality

According to a recent study, the risk of short-term mortality at 30 days increased 2.1 fold with
moderate patient-prosthesis mismatch, 11.4 fold with severe patient-prosthesis mismatch, and 77.1
fold for patients with severe patient prosthesis mismatch and left ventricular ejection fraction less
than 40%.2
A Tale Of Two Babies: How anecdotal
success may lead us astray.

 Easy to be tempted by
early repair when
things go well
 6 wk old male 3.8kg
with Down's
Syndrome AVC,
CoAo.
This baby underwent Neonatal
repair of IAA/AVC
Patient Post-op course was uncomplicated. LA line and chest tubes removed 11/21. Pt
extubated 11/23. Transferred to the floor 11/26.
Discharged POD 9.

No residual atrial septal defect. No residual ventricular septal defect. Trivial right sided
atrioventricular valve regurgitation. Mild residual left sided atrioventricular valve
regurgitation. No residual ductal flow. No residual coarctation. Qualitatively fair to good
left ventricular systolic function. Qualitatively fair to mildly depressed right ventricular
systolic function. No pericardial effusion.
Ready for discharge on the
ninth day after surgery.
Using the anecdotal method of
case management
 The next patient with this lesion
should do well
 And maybe we should be doing the
straightforward canals electively as
neonates
 Emboldened by success, we expect the next
patient to do just as well.

 Case presentation: 2.1

kg Downs CAVC/CoAo
undergoes surgery at
age 2 days
 The Patient struggles
postoperatively
 We Investigate early

 The post-op course was difficult, the baby

was unable to be weaned from ventilator or
off inotropic support. ECHO at that time
revealed a moderate residual VSD, mod
TR, RV HTN, mild MV regurgitation.
 A Cath was done on POD 12, confirming
systemic RV pressures with Qp/Qs of 3:1,
Residual VSD
 And repair the problems
 We returned to the OR on postop day
16 for repair of residual VSD, and
tricuspid valvuloplasty.
 She was subsequently weaned off
inotropic support. Last ECHO 8/6/03:
PFO, x2 small resrictive VSD, mild-
mod TR, cleft MV w/mild regurg,
mild MS, mild RVH, good bivent
function
 Discharged postop day 68
 Follow-up at two years, off all cardiac
meds, normal growth curve and
activity level, no residual VSD’s,
mild MR/TR.
 And at the next cath conference
 You decide not to perform the next AVC
repair electively in the neonatal period
 Actually, it doesn’t come up because the
senior cardiologists have seen enough and are
now actively hiding all the patient’s they
diagnose with AVC until they are 3 months
old.
 Based on our experience, what do we tell the
parents of newborn babies with CAVC?
How do we make evidence based decisions as a
congenital heart team?
We use our continuous outcomes measurement
tool to give accurate answers to these questions.
It is apparent in the slides that follow, that
newborn repairs have been associated with
prolonged hospital stays.

www.pediatricheartsurgery.com
 LOS vs. Age AVC MCH 1995-2005

140

120

100

80
LOS

60

40

20

0
0 50 100 150 200 250 300 350 400
Age in days
 LOS after AVC MCH 1995-2005

70

60

50
LOS in days

40

mean LOS
30
median LOS
3
20

10
6 6 16 8 10 23
0
1 mth 2 mth 3 mth 4 mth 5 mth 6 mth 7-12 mth
Age in mths
Number in column is N
 So, what does three months get us?

 Hopefully, valves that

don’t look like this:

Faster recovery
Shorter ventilator
requirement
Shorter CICU stay
Shorter
hospitalization
 Conclusions
 Neonatal CAVC repair can be done when necessary,
gird yourself for a prolonged hospitalization.
 Elective AVC repair can be performed with minimal
risk at three months.
 This three months of annular growth and valve tissue
maturation may enhance the durability and precision
of AV valve reconstruction, and justify a “delayed”
repair.
 Thank you.

www.pediatricheartsurgery.com