Ventricular Septal Defect Repair:Ventricular Septal Defect Repair:What a parent should know about What a parent should know about

device closuredevice closure

Redmond P. BurkeThe Congenital Heart InstituteMiami Children’s Hospital and

Arnold Palmer Hospital

PICSPICS20082008

How does my team decide on our How does my team decide on our approach to each patient?approach to each patient?

Patients with VSD’s are Patients with VSD’s are usually diagnosed by usually diagnosed by pediatric cardiologists and pediatric cardiologists and referred to our surgeons or referred to our surgeons or interventional cardiologists interventional cardiologists for treatment.for treatment.

For the past several years, at For the past several years, at weekly program conferences, weekly program conferences, we have discussed every we have discussed every patient presented for VSD patient presented for VSD closure.closure.

Our approach is to treat each Our approach is to treat each patient with the most patient with the most effective, and least traumatic effective, and least traumatic approach available.approach available.

Like you, we strive to make Like you, we strive to make Evidence Based DecisionsEvidence Based Decisions

““The Literature” is a nice guide, but The Literature” is a nice guide, but we use our own results for clinical we use our own results for clinical decision making whenever possible, decision making whenever possible, finding them to be more relevant.finding them to be more relevant.

Our outcomes are reported on our Our outcomes are reported on our website at website at www.pediatricheartsurgery.comwww.pediatricheartsurgery.com

Total Cases (N)Total Cases (N) 387387

Mortality (N)Mortality (N) 11 0.25%0.25%

Transient CHB (N)Transient CHB (N) 33 0.77%0.77%

Requiring Pacemaker (N)Requiring Pacemaker (N) 11 0.25%0.25%

Median Weight (kg)Median Weight (kg) 7.17.1

Median Age (days)Median Age (days) 269269 (4d-24yrs)(4d-24yrs)

Median Post-op Stay Median Post-op Stay (days)(days) 44

No instance of complete heart block in last 353 casesThis experience similar to GOS review of >2000 pts (.6% CHB)

Ann Thorac Surg. 2006 Sep;82(3):948-56; discussion 956-7.

Includes:Perimembranous VSDSubarterial VSDSupracrystal VSD

Current MCH surgical Results for VSD closure: 1995-2008

Is surgical heart block a real risk?Is surgical heart block a real risk?

Absolutely, but this risk is clearly surgeon Absolutely, but this risk is clearly surgeon specific.specific.

Conduction disturbances (and most other Conduction disturbances (and most other complications) can be viewed as a marker complications) can be viewed as a marker of a surgeon’s gentleness, finesse, of a surgeon’s gentleness, finesse, technique, and experience.technique, and experience.

You might have a program where the risk You might have a program where the risk of surgical conduction problems and heart of surgical conduction problems and heart block after VSD repair will lead you to block after VSD repair will lead you to make different decisions than us.make different decisions than us.

Is the conduction system likely to be injured Is the conduction system likely to be injured during surgical repair?during surgical repair?

Terrifying history – AV node injury Terrifying history – AV node injury was lethal in early surgical was lethal in early surgical experience. Surgeons are trained experience. Surgeons are trained to avoid to avoid anyany contact with the contact with the septum during VSD repairseptum during VSD repair

Modern techniques – continuous Modern techniques – continuous suture in the endocardium, no suture in the endocardium, no touch technique on the septum touch technique on the septum • Shallow stitching close to the Shallow stitching close to the

rim of the ventricular septal rim of the ventricular septal defect eliminates injury to the defect eliminates injury to the right bundle branch.right bundle branch.

Ann Thorac Surg. 2004 Ann Thorac Surg. 2004 Jun;77(6):2259-60; author Jun;77(6):2259-60; author reply 2260.reply 2260.

No routine pacing wires, but easy No routine pacing wires, but easy to do if neededto do if needed

Spontaneous cardioversion is the Spontaneous cardioversion is the rule in the ORrule in the OR

If you’re seeing a lot of heart block, If you’re seeing a lot of heart block, you don’t need a new approach, you don’t need a new approach, you need a new surgeon.you need a new surgeon.

Circulation. 2006;113:2775-2781.

So is our typical patient with PMVSD a So is our typical patient with PMVSD a candidate for device repair? No.candidate for device repair? No.

Small baby 2.52 Small baby 2.52 kg with FTTkg with FTT

Large PMVSDLarge PMVSD Associated Associated

lesions (50%)lesions (50%) All pericardial All pericardial

repairrepair Never touch the Never touch the

conduction areaconduction area Visualize and Visualize and

preserve the preserve the aortic and aortic and tricuspid valvestricuspid valves

Never put an Never put an instrument across instrument across the defectthe defect

Never use Never use circulatory arrestcirculatory arrest

What information is available on What information is available on Device Closure? Not much.Device Closure? Not much.

PintoPinto KapoorKapoor ThanaopoulosThanaopoulos AnilAnil LiLi CarminatiCarminati AroraArora DuronpisitkulDuronpisitkul ZabalZabal HijaziHijazi WalshWalsh Pawelec-WoitalikPawelec-Woitalik

* Indicates prospective randomized study

Why do we have to be careful extrapolating Why do we have to be careful extrapolating these early device results? They picked the these early device results? They picked the

easy patients.easy patients. The early device experience is in very The early device experience is in very

big patients with small defects, followed big patients with small defects, followed for a short period of timefor a short period of time• 7.7y/25kg (Fu)7.7y/25kg (Fu)

In contrast, our surgical experience is in In contrast, our surgical experience is in small babies with big defects, followed small babies with big defects, followed for decades: for decades: • Our mean age/wt for surgical repair of PMVSD Our mean age/wt for surgical repair of PMVSD

is 9mo/7kg, the defects are usually the size of is 9mo/7kg, the defects are usually the size of the aortic valve annulus, and the patients the aortic valve annulus, and the patients have FTT.have FTT.

As we have seen with ASD device As we have seen with ASD device closure, sex, and surgery, size affects closure, sex, and surgery, size affects performance performance

Is this experience with small defects in big patients misleading? Yes, I Is this experience with small defects in big patients misleading? Yes, I think smaller patients with larger defects are a serious challenge to think smaller patients with larger defects are a serious challenge to

device design and performance.device design and performance.

Vascular access and catheter manipulation is easier.Vascular access and catheter manipulation is easier. Older patients with small defects have had time to develop a Older patients with small defects have had time to develop a

cushion of tricuspid tissue around the defect, creating a buffer from cushion of tricuspid tissue around the defect, creating a buffer from the aortic valve. This is not the case in younger patients, where up the aortic valve. This is not the case in younger patients, where up to half of the defect diameter is aortic valve annulus.to half of the defect diameter is aortic valve annulus.

7yo , LOS 3d small defect surrounded by fibrous scar

2 mo 4.2kg, LOS 5 days half the circumference of the defect is aortic valve annulus

Are Surgical materials safe over time? Are Surgical materials safe over time? Absolutely.Absolutely.

It’s freeIt’s free We have 5 decades of We have 5 decades of

experience with the material, experience with the material, and no uncertainty about long and no uncertainty about long term effects in the circulation.term effects in the circulation.

No erosion into adjacent No erosion into adjacent structuresstructures

Anchored by continuous Anchored by continuous suture, patch dehiscence is suture, patch dehiscence is very rare, embolization neververy rare, embolization never

Residual leak rareResidual leak rare Each patch is customized to Each patch is customized to

the patient’s defect, very low the patient’s defect, very low profileprofile

No long term anticoagulation No long term anticoagulation or antibiotic prophylaxisor antibiotic prophylaxis

Pericardial Patch

Will device materials be safe over Will device materials be safe over time? No way to know, it will take time? No way to know, it will take

decades to find out.decades to find out.

Device closure is with Device closure is with Nitinol/fabric (cost - $4500 )Nitinol/fabric (cost - $4500 )

Early studies are encouraging at Early studies are encouraging at 14 months (Kong)14 months (Kong)

Anchoring with radial Anchoring with radial pressure/double disc/fibrosis. Not pressure/double disc/fibrosis. Not always stable, embolization always stable, embolization reported in several early series. reported in several early series. Retrieval is traumatic.Retrieval is traumatic.

Residual leak common early, with Residual leak common early, with subsequent closure over timesubsequent closure over time

The patient is forced to conform The patient is forced to conform to the available device size. You to the available device size. You must select from preset sizes and must select from preset sizes and shapes.shapes.

Will devices require Will devices require anticoagulation or long term anticoagulation or long term antibiotics?antibiotics?

PMVSD Device (Amplatzer)

My gut feelingMy gut feeling

If you gave me any available device, and If you gave me any available device, and asked me to place it in a PM VSD under asked me to place it in a PM VSD under direct vision, with the heart arrested, and I direct vision, with the heart arrested, and I could suture it in the best possible could suture it in the best possible position, so that it didn’t touch the aortic position, so that it didn’t touch the aortic valve, the tricuspid valve or the valve, the tricuspid valve or the conduction system, and completely closed conduction system, and completely closed the defect…the defect…

I couldn’t do it. (I could do it with a I couldn’t do it. (I could do it with a pericardial patch though, pretty damn fast pericardial patch though, pretty damn fast too.)too.)

Do devices work anywhere in the Do devices work anywhere in the ventricular septum?ventricular septum?

SureSure Picture a 39 week female born at Picture a 39 week female born at

2.18 kg with a small PMVSD and a 2.18 kg with a small PMVSD and a large muscular VSD, and coarctation large muscular VSD, and coarctation with arch hypoplasiawith arch hypoplasia

Repair at day of life 4. Repair at day of life 4.

We choose a hybrid procedure, Surgical arch We choose a hybrid procedure, Surgical arch repair, and Device Closure of the Muscular Defect, repair, and Device Closure of the Muscular Defect,

we’re not closed minded about this stuff.we’re not closed minded about this stuff.

So what’s my greatest concern? Devices So what’s my greatest concern? Devices could destroy the aortic valve.could destroy the aortic valve.

Most PMVSD are contiguous Most PMVSD are contiguous with the aortic valve annulus.with the aortic valve annulus.

PMVSD device engineers face a PMVSD device engineers face a conundrum - how to secure a conundrum - how to secure a device in a high pressure flow device in a high pressure flow zone with a low profile on the zone with a low profile on the high pressure side (to avoid aortic high pressure side (to avoid aortic valve injury) without increasing valve injury) without increasing radial force (risk to conduction radial force (risk to conduction and aortic valve).and aortic valve).

What will be the long term effects What will be the long term effects of subvalvar turbulence and/or of subvalvar turbulence and/or device abrasion on the aortic device abrasion on the aortic annulus or leaflets?annulus or leaflets?

ASD devices in proximity to the ASD devices in proximity to the aorta have eroded into the aorta aorta have eroded into the aorta (Chun, 2003)(Chun, 2003)

What is the surgical experience with What is the surgical experience with foreign material under the aortic valve?foreign material under the aortic valve?

Subaortic stenosis after VSD Subaortic stenosis after VSD repair is a rare progressive lesion repair is a rare progressive lesion (Cicini) We have seen several (Cicini) We have seen several patients with subaortic patients with subaortic obstruction and aortic obstruction and aortic insufficiency from fibrous insufficiency from fibrous reaction to Dacron VSD patches. reaction to Dacron VSD patches. We now use pericardium We now use pericardium exclusively.exclusively.

Our experience with Subaortic Our experience with Subaortic membrane suggests that creating membrane suggests that creating turbulence and fibrosis under the turbulence and fibrosis under the aortic valve can progressively aortic valve can progressively damage the valve, and necessitate damage the valve, and necessitate valve replacement.valve replacement.

Typical proximity of PMVSD to Aortic Valve Typical proximity of PMVSD to Aortic Valve observed during surgery: 17 Month oldobserved during surgery: 17 Month old

How close is the aortic valve with a small defect? How close is the aortic valve with a small defect? Very close.Very close.

Is there misperception of the Is there misperception of the conduction system anatomy?conduction system anatomy?

EP studies might give the impression that there is a conduction area that can be avoided by device placement

What’s the real Achille’s Heel for device What’s the real Achille’s Heel for device closure in the membranous septum? closure in the membranous septum? The risk of Permanent Heart Block.The risk of Permanent Heart Block.

Anatomic studies show discrete conduction Anatomic studies show discrete conduction bundles just under the endocardium, along bundles just under the endocardium, along

the margin of a PMVSDthe margin of a PMVSD

A device in contact with the margin of a PMVSD, will be in contact with the His Bundle

Why is the conduction system likely to be Why is the conduction system likely to be damaged during and after device closure?damaged during and after device closure?

Early device experience suggests the conduction system will be injured frequently. (Zhonghua, Kapoor, Fu, Carminati)

Technique of dual wires and stiff sheath across the septum applies direct uncontrolled pressure trauma to the septum (violating surgical principles)

The PMVSD device is then implanted directly on the conduction area with unmeasured and uncontrolled radial and compressive force

This creates the potential for early (pressure) and late (fibrotic) injury to the conduction system

Late CHB at 5 months and 1 year after device placement has been reported. (Caminiti)

How do we explain the How do we explain the delayed onset of CHB delayed onset of CHB

seen after device seen after device closure?closure?

JACK L. TITUSAnatomy of the Conduction SystemCirculation, Jan 1973; 47: 170 - 177

Chronic fibrosis of the AV Chronic fibrosis of the AV node is a mechanism for node is a mechanism for CHB and has been CHB and has been related to compressionrelated to compression

Will the Tricuspid Valve be injured by PMVSD Will the Tricuspid Valve be injured by PMVSD device closure?device closure?

During surgical repair, tricuspid During surgical repair, tricuspid valve chords are preserved.valve chords are preserved.

The TV septal leaflet chords are The TV septal leaflet chords are always near the margin of a always near the margin of a PMVD and will always be in PMVD and will always be in contact with the larger RV contact with the larger RV occluder disc. Over time, the occluder disc. Over time, the discs could damage the TV discs could damage the TV leaflets and chords creating TS leaflets and chords creating TS or TI.or TI.

Acute TV chord rupture has Acute TV chord rupture has been reported during device been reported during device closure of PMVSD. (Fu)closure of PMVSD. (Fu)

With all the attention on the left With all the attention on the left side of the septum to avoid the side of the septum to avoid the aortic valve and conduction aortic valve and conduction system, the TV will take a system, the TV will take a beating.beating.

5 y/o with Hx of VSD. TEE postop, no TR.

Does surgical closure of PMVSD create LVOT turbulence or obstruction?

Typical surgical repair: 1 mo, 2.52kg, Typical surgical repair: 1 mo, 2.52kg, pericardial patch repair of PMVSD, discharged pericardial patch repair of PMVSD, discharged home on POD 7.home on POD 7.

Preop echo, PMVSD, no turbulence subAS or AI

Postop echo, no VSD, no turbulence under aortic valve. subAS or AI

Is LVOT turbulence and obstruction more Is LVOT turbulence and obstruction more likely to occur with PMVSD Device Closure?likely to occur with PMVSD Device Closure?

Based on the device Based on the device design requirements, design requirements, devices are likely to devices are likely to protrude into the LVOT and protrude into the LVOT and create turbulencecreate turbulence

This could create This could create immediate LVOTO, or late immediate LVOTO, or late LVOTO with fibrous LVOTO with fibrous ingrowth and device shape ingrowth and device shape changeschanges

Surgical Treatment will Surgical Treatment will require device removal, require device removal, which will create complete which will create complete heart blockheart block

Simone Rolim, et al, Arq. Bras. Cardiol.Simone Rolim, et al, Arq. Bras. Cardiol.  v.86 n.2  São Paulo fev. 2006v.86 n.2  São Paulo fev. 2006

Is procedural trauma so much less than surgery Is procedural trauma so much less than surgery that we should accept these uncertainties that we should accept these uncertainties

regarding device closure?regarding device closure?

Surgery is performed in OR Surgery is performed in OR under GA/TEEunder GA/TEE

Bypass subjects the body to Bypass subjects the body to inflammation, time relatedinflammation, time related

Small risk of cerebral Small risk of cerebral embolizationembolization

Incision creates a visible Incision creates a visible cosmetic injury unrelated to cosmetic injury unrelated to patient sizepatient size

Hospital stays from 2 to 5 Hospital stays from 2 to 5 days. Patients usually days. Patients usually completely stable completely stable hemodynamically in the OR, hemodynamically in the OR, episodes of uncontrolled episodes of uncontrolled hypotension very rare.hypotension very rare.

Hemodynamically significant Hemodynamically significant residual lesion is rareresidual lesion is rare

Device closure in Cath Lab under Device closure in Cath Lab under GA/TEEGA/TEE

Fluoroscopy, subjects the body to Fluoroscopy, subjects the body to Ionizing radiation, with no safe Ionizing radiation, with no safe duration of exposureduration of exposure

Small risk of cerebral embolizationSmall risk of cerebral embolization Vascular access creates invisible Vascular access creates invisible

functional injuries to major blood functional injuries to major blood vessels, (DVT, ischemia) worse in vessels, (DVT, ischemia) worse in small patients – arterial occlusion small patients – arterial occlusion in 16% (Kulkarni) in 16% (Kulkarni)

Hospital stays from 1 to 5 days. Hospital stays from 1 to 5 days. Patients reported to be unstable in Patients reported to be unstable in OR due to OR due to arrhythmia/hypotension/bleeding arrhythmia/hypotension/bleeding (Kapoor)(Kapoor)

Hemodynamically significant Hemodynamically significant residual lesion also pretty rareresidual lesion also pretty rare

ConclusionsConclusions Surgical closure of PMVSD is safe and effective.Surgical closure of PMVSD is safe and effective. Early uncontrolled, non-randomized trials of Early uncontrolled, non-randomized trials of

device closure for selected older patients with device closure for selected older patients with small defects raise significant concerns about small defects raise significant concerns about potential short and long term injury to the Aortic potential short and long term injury to the Aortic and Tricuspid valves, the left ventricular outflow and Tricuspid valves, the left ventricular outflow tract, and the conduction system.tract, and the conduction system.

There is no convincing evidence to suggest that There is no convincing evidence to suggest that device closure of PMVSD will produce less lifelong device closure of PMVSD will produce less lifelong trauma or be more effective than surgical closure. trauma or be more effective than surgical closure.

Thank YouThank You