Redmond P. Burke, M.D., FACSRedmond P. Burke, M.D., FACSChief, Division of Cardiovascular SurgeryChief, Division of Cardiovascular SurgeryThe Congenital Heart InstituteThe Congenital Heart InstituteMiami Children’s Hospital and Arnold Palmer HospitalMiami Children’s Hospital and Arnold Palmer Hospitalwww.pediatricheartsurgery.comwww.pediatricheartsurgery.com

One surgeon’s observations on One surgeon’s observations on neonatal surgery for Complete neonatal surgery for Complete Atrio-Ventricular CanalAtrio-Ventricular Canal

Factors influencing timing of surgery Factors influencing timing of surgery for CAVCfor CAVC

The combination of shunting at atrial and ventricular levels, with an The combination of shunting at atrial and ventricular levels, with an additional volume load produced by AV valve regurgitation, produces additional volume load produced by AV valve regurgitation, produces symptomatic heart failure in infancy.symptomatic heart failure in infancy.

Yamaki et al reported early pulmonary vascular changes in children with Yamaki et al reported early pulmonary vascular changes in children with AVC and recommended repair AVC within the first 6 months of life to AVC and recommended repair AVC within the first 6 months of life to prevent long-term morbidity. prevent long-term morbidity.

Children with Down syndrome are more vulnerable to early pulmonary Children with Down syndrome are more vulnerable to early pulmonary vascular changes. Therefore, early repair of AVC to prevent permanent vascular changes. Therefore, early repair of AVC to prevent permanent pulmonary vascular changes in children with Down syndrome has been the pulmonary vascular changes in children with Down syndrome has been the practice in many surgical centers.practice in many surgical centers.

Hanley et al reviewed the results of surgical repair of AVC and found a 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 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 support for aggressive approach for repair in the first 3 months of life. (and that was about thirteen years ago)that was about thirteen years ago)

Age at Surgery as Related to Surgical Date

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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 daysMedian POS: 1995-1999: 8 days; 2000-2005: 7 days

Over the past decade, we have moved to earlier repairs for CAVC.

Given these pressures to repair earlier, particularly in an Given these pressures to repair earlier, particularly in an era of early complete repair for more complex lesions, era of early complete repair for more complex lesions,

why are we not performing routine neonatal AVC why are we not performing routine neonatal AVC repairs?repairs?

We do successfully operate on neonates with CAVC, although We do successfully operate on neonates with CAVC, although usually when they have other problemsusually when they have other problems Arch obstructionArch obstruction AV valve insufficiencyAV valve insufficiency FTT (feeding tubes and g-tubes are a good indication for surgery)FTT (feeding tubes and g-tubes are a good indication for surgery)

Absent these indications, there may be some good technical Absent these indications, there may be some good technical reasons to wait a few months for complete repairreasons to wait a few months for complete repair

Technical thoughts pertinent to Technical thoughts pertinent to neonatal CAVC repairneonatal CAVC repair

1.1. Pulmonary artery banding for CAVC is Pulmonary artery banding for CAVC is extraordinarily rare, or should be.extraordinarily rare, or should be.

Maybe for multiple muscular VSD’s?Maybe for multiple muscular VSD’s? Maybe for single ventricle pathMaybe for single ventricle path

Are we not performing neonatal repair because it would Are we not performing neonatal repair because it would require a long period of circulatory arrest? My fingers require a long period of circulatory arrest? My fingers

as size reference.as size reference.

No, the CAVC repair can be readily performed without circ No, the CAVC repair can be readily performed without circ arrest, without a cluttered or bloody operative field. Again, that’s arrest, without a cluttered or bloody operative field. Again, that’s my fingertip, and the initial image of the CAVC defect with stay my fingertip, and the initial image of the CAVC defect with stay

sutures in the valve leaflets.sutures in the valve leaflets.

I’m preparing to divide the superior I’m preparing to divide the superior leaflet between stay sutures.leaflet between stay sutures.

The Dacron VSD patch is secured with running The Dacron VSD patch is secured with running suture, then the valves will be reattached to the suture, then the valves will be reattached to the

crest of the VSD patch.crest of the VSD patch.

Final finger shot, the atrial component of Final finger shot, the atrial component of the CAVC has been closed with the child’s the CAVC has been closed with the child’s

own pericardium.own pericardium.

The Achilles heel of these neonatal repairs The Achilles heel of these neonatal repairs is the quality of the valve reconstructions.is the quality of the valve reconstructions.

There may be a tradeoff when There may be a tradeoff when reconstructing a neonatal left AV reconstructing a neonatal left AV valve, particularly one with pre-valve, particularly one with pre-existing severe MR.existing severe MR.

Technical maneuvers to decrease MR Technical maneuvers to decrease MR tend to produce MStend to produce MS

Commissural plicationCommissural plication Cleft closureCleft closure Ring insertionRing insertion

In some forms of AVC, the cleft is In some forms of AVC, the cleft is the main opening of the AV valvethe main opening of the AV valve

The left lateral leaflet (what would be The left lateral leaflet (what would be the posterior leaflet in a normal the posterior leaflet in a normal valve) forms much less of the valve valve) forms much less of the valve circumference.circumference.

Valves with single or closely spaced Valves with single or closely spaced papillary muscles, or double orifices, papillary muscles, or double orifices, may rely on the cleft for valve may rely on the cleft for valve opening.opening.

Valve repair at 4 days is significantly different Valve repair at 4 days is significantly different than at 4 monthsthan at 4 months

1 mo Downs 2.6kg1 mo Downs 2.6kg • 4 mo Downs 5kg

The difference between sewing Jello™ and sewing cloth. Look at the 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.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 Why do you have to get the repair right?right?

Plan B isn’t great – there is no room Plan B isn’t great – there is no room for a mechanical valve in a newborn’s for a mechanical valve in a newborn’s mitral annulus.mitral annulus.

The LA hasn’t had time to enlargeThe LA hasn’t had time to enlarge The pulmonary veins will be The pulmonary veins will be

obstructedobstructed The LVOT will be obstructedThe LVOT will be obstructed You can easily hit the conduction You can easily hit the conduction

systemsystem You can easily hit the circumflex You can easily hit the circumflex

coronary arterycoronary artery Mitral Valve Prosthetic Rings aren’t Mitral Valve Prosthetic Rings aren’t

an option either, although considered an option either, although considered essential for adult valvuloplasty, they essential for adult valvuloplasty, they would restrict annular growth and you would restrict annular growth and you can’t buy one of the shelf in these can’t buy one of the shelf in these sizes anyway)sizes anyway)

Patient Valve Mismatch is Patient Valve Mismatch is Automatic in neonatal AV valve Automatic in neonatal AV valve

replacementreplacementWhat 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 MortalityAccording 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 A Tale Of Two Babies: How anecdotal success may lead us astray.success may lead us astray.

Easy to be tempted by Easy to be tempted by early repair when things early repair when things go wellgo well

6 wk old male 3.8kg with 6 wk old male 3.8kg with Down's Syndrome AVC, Down's Syndrome AVC, CoAo.CoAo.

This baby underwent Neonatal This baby underwent Neonatal repair of IAA/AVCrepair 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 Ready for discharge on the ninth day after surgery.ninth day after surgery.

Using the anecdotal method of Using the anecdotal method of case managementcase management

The next patient with this lesion The next patient with this lesion should do wellshould do well

And maybe we should be doing the And maybe we should be doing the straightforward canals electively as straightforward canals electively as neonatesneonates

Emboldened by success, we expect the next Emboldened by success, we expect the next

patient to do just as wellpatient to do just as well..

Case presentation: 2.1 Case presentation: 2.1 kg Downs CAVC/CoAo kg Downs CAVC/CoAo undergoes surgery at undergoes surgery at age 2 daysage 2 days

The Patient struggles The Patient struggles postoperativelypostoperatively

We Investigate earlyWe 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 problemsAnd 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 conferenceAnd at the next cath conference

You decide not to perform the next AVC You decide not to perform the next AVC repair electively in the neonatal periodrepair electively in the neonatal period

Actually, it doesn’t come up because the Actually, it doesn’t come up because the senior cardiologists have seen enough and are senior cardiologists have seen enough and are now actively hiding all the patient’s they now actively hiding all the patient’s they diagnose with AVC until they are 3 months diagnose with AVC until they are 3 months old.old.

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Based on our experience, what do we tell the Based on our experience, what do we tell the parents of newborn babies with CAVC?parents of newborn babies with CAVC?

How do we make evidence based decisions as a How do we make evidence based decisions as a congenital heart team?congenital heart team?

We use our continuous outcomes measurement We use our continuous outcomes measurement tool to give accurate answers to these questions.tool to give accurate answers to these questions.

It is apparent in the slides that follow, that It is apparent in the slides that follow, that newborn repairs have been associated with newborn repairs have been associated with

prolonged hospital stays.prolonged hospital stays.

LOS vs. Age AVC MCH 1995-2005

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Age in mths

LOS after AVC MCH 1995-2005

mean LOS

median LOS3

6 6 16 8 10 23

Number in column is N

So, what does three months get us?So, what does three months get us?

Hopefully, valves that Hopefully, valves that don’t look like this:don’t look like this:

Faster recovery Faster recovery Shorter ventilator Shorter ventilator requirementrequirementShorter CICU stayShorter CICU stayShorter Shorter hospitalizationhospitalization

ConclusionsConclusions

Neonatal CAVC repair can be done when necessary, Neonatal CAVC repair can be done when necessary, gird yourself for a prolonged hospitalization.gird yourself for a prolonged hospitalization.

Elective AVC repair can be performed with minimal Elective AVC repair can be performed with minimal risk at three months.risk at three months.

This three months of annular growth and valve tissue This three months of annular growth and valve tissue maturation may enhance the durability and precision maturation may enhance the durability and precision of AV valve reconstruction, and justify a “delayed” of AV valve reconstruction, and justify a “delayed” repair.repair.

Thank you.Thank you.

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