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
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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
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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
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Pulmonary artery banding for CAVC is extraordinarily rare, or should be.
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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 preexisting severe MR.

Technical maneuvers to decrease MR tend to produce MS
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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.
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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
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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, mildmod 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.
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LOS vs. Age AVC MCH 1995-2005

140 120 100 LOS 80 60 40 20 0 0 50 100 150 200 Age in days 250 300 350 400

LOS after AVC MCH 1995-2005
70 60 50 LOS in days 40 30 20 10 0 1 mth 2 mth 3 mth 4 mth Age in mths 5 mth mean LOS

3

median LOS

6

6

16

8

10
6 mth

23
7-12 mth

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