SHA24/071002

Prof Ahmet Çelebi MD Dr Siyami Ersek Hospital for Cardiology & Cardiovascular Surgery Deparment of Pediatric and Congenital Cardiology Istanbul, Turkey GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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 Pulmonary atresia with ventricular septal defect,  Tetralogy of Fallot with coronary abnormality  Absent pulmonary valve syndrome,  Common arterial trunk (truncus arteriosus)  Rastelli-type repair of transposition with VSD and PS  DORV with pulmonary atresia or severe PS  During the Ross operation.  Conventional repair of C -CTGA

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

The lifespan of RV-PA conduits is limited by  Kinking or sternal compression  Getting smaller relatively in growing patient  Endothelial proliferation and luminal narrowing,  Progressive degeneration and calcification



Eventually, conduit dysfunction occurs requiring replacement, at 5 to 15 years following surgery (>50% within ten years)

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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ď‚Ą

May be due to conduit stenosis, valve regurgitation or both (mixed)

ď‚Ą

The most common reason of conduit dysfunction needing surgery is stenosis

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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Surgical RVOT

Conduit or RVOT

reconstruction

dysfunction

4-5 times re-operations during their lifespans

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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minimize the total number of open heart surgeries

intervening before right ventricle dysfunction

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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Can we delay the surgical reoperations

by catheter interventions? 

PPVR; for stenosis, regurgitation or both



Balloon angioplasty (BA); for stenosis



Stent placement; for stenosis

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

In conduit stenosis

 Symptomatic pts, regardless of pressure gradient  Asymptomatic Pts ▪ RV-PA pressure gradient >50 mmHg ▪ RVp >60 mmHg ▪ RV to LV systolic pressure ratio > 2/3 GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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ď‚Ą

BA is associated with limited and transient success owing to rapid recoil of the conduit wall.

ď‚Ą

Stenting is superior compared to BA alone in terms of both decreasing the pressure gradient and delaying the reintervention, but in expense of free PR

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

It does not cause PR, at least not as much as stenting



the pts are less exposed to the more dangerous effects of the free PR



We can delay stenting by BA, but in only small number of pts for a certain time

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

Gives an idea whether the lesion is dilatable or not



Confirms the most stenotic part of the conduit



Stent migrations observed less frequently in predilated pts

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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Cranial 35o

900 lateral

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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Cranial 35o

900 lateral

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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Cranial 35o

900 lateral

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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Caudal 35o LAO 10

900 lateral

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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Cranial 35o

900 lateral

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

Can delay the surgical replacement as decreasing RV pressure until the smaller pts growing appropriate weight



Free PR is well tolerated in early period since RV hypertrophy and poor compliance of RV,



But, close follow-up is necessary for the RV volumes and functions with MRI GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

Prestenting has been performed just before PPVR  in order to prevent stent fracture  constituting a secure landing zone

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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 Because of the development of free PR ± stenosis  RV volume overload  mixed severe dysfunction

 eventually valve replacement will have to be necessary

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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RV volume overload

YES YES

RV dilatation/aneurysm Elimination by Intervention RV dysfunction (might be irreversible

NO NO

RV failure, ventricular arrhythmias and sudden death GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

Since 2000 in that Bonhoeffer et al reported the first experimental and clinical human application of a PPVR



It has become a feasible, safe and effective treatment in selected pts.



It is one of the most exciting and rapidly developing field in interventional pediatric cardiology GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

Patient History (detailed)  First diagnosis?, which operations?, which conduits?, how

nominal diameter of the conduit?  Symptoms



Echocardiography  RV-PA pressure gradient, RV pressure (calculated by TR)  RV diameter, grading of PR



Magnetic resonance imaging (MRI)  RVOT anatomy  RV volumes, PR fraction, ejection fraction (EF) of RV and LV

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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Five distinctive types of right ventricular outflow tract morphology

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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



Symptomatic patients 

Moderate to severe PR or conduit stenosis



Ventricular arrythmias with RV dilatation



RV/LV diameter ratio ≥1.5 on MRI

Asymptomatic pts 

Severe stenosis (RV pressure >3/4 of systemic pressure)



Severe PR; ▪ A regurgitant fraction > 40% ▪ Enlarged RV; EDV > 150 ml/m2 ▪ Diminished RV EF < 40%



QRS duration > 180 msec

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

There are two available TPV  Bonhoeffer Melody valve (Medtronic, Inc., Minneapolis)  Edwards Sapien valve (Edwards Lifesciences)

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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The Melody TPV is made of a bovine jugular vein sewn inside a 28 mm Cheatham-Platinum stent

Inflow/Proximal End

Outflow/Distal End

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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Melody valve TPV delivery systems; up to BIB sizes



Although the valve diameter is only one size; 18 mm,



But, it is available in three sizes up the BIB catheter;



18, 20, 22 mm

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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The valve crimped on a BIB downsized to a diameter of 6 mm delivered by a retractable sheath requiring a 22Fr delivery system GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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This valve is made of 3 bovine pericardial leaflets sewn inside a stainless steel stent.

Edwards-Sapien valve GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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ď śCurrently, it is available in 23 or 26 mm in diameter in sizes ď śThe heights of them are 14 and 16 mm, respectively

If

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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Edwards EdwardsSapiens Sapienstranscatheter transcatheterpulmonary pulmonaryvalve valvesystem system 

The delivery sheath is 35 cm long;, so after outside the sheath delivery system is unprotected



Requires a 22F to 24F delivery sheath.



The valve is crimped by a tool GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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Edwards Sapien Retroflex 3 Delivery System ESV is delivered by retroflex 3 delivery system uncovered but distal end of the sheath is tapered can flex up to 220 degrees.

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

The patient selection is crucial in the success of this technique



Implant possibility depends on three factors:  Size and distensibility of the RVOT  Morphology of the RVOT or pulmonary trunk;  The position of the coronary arteries

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

Stenotic conduits ± PR is an excellent bed for successful anchoring



A large conduit or RVOT> valve diameter  unsuitable  > 22 mm for Melody and > 26 mm for Edwards-sapiens valves



Native RVOT with transannular patch does not provide a discrete implantation point for safe anchoring GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

Nominal diameters of the conduits should be 

Btw 16 & 22 mm for melody valve ,



Btw 20 & 27 mm, for Edwards Sapiens valve



If the conduit is severe stenotik, nominal diameters may be more than the largest valves

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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The method of the procedure is the same as RVOT stenting, as showed before, until the stage of valve implantation  General anesthesia or deep sedation  Hemodynamic assessment  Angiographic demonstration of RVOT;  Pre-dilatation of conduit and coronary testing angiography during balloon

inflation  PRE-STENTİNG  Hemodynamic assessment and angiographic evaluation after prestenting  If there is residual gradient > 25 mmHg  post dilatation with higher

pressure balloons (such as Atlas or Mullins) may be necessary GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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may decrease the incidence of stent fracture.

acts as a landing zone for the new valve

gives an idea whether

the valve will be dilated properly

may provide a good way for tracking the crimped valve

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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PA

900 lateral GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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Cranial 35o

900 lateral

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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Cranial 35o

900 lateral

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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Cranial 35o

900 lateral

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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Cranial 35o

900 lateral

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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Cranial 35o

900 lateral

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

Reduces RV pressures and RVOT pressure gradient



Eliminates PR, ≤trivial in most of the pts .



Diastolic PA pressures rise immediately after deployment

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

Residual stenosis



Device migration

system in the tendinous cords of



Device misplacement

the TV



Coronary compression



Rupture of blood vessels



Homograft rupture



Hammock effect/stenosis



PA obstruction (jailing)



Local vascular complications



Entrapment of the delivery

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh 47



Clinical symptoms improve within 6 months after PPVI.



Decreased RV pressures and RV-EDV are sustained at one year



RV EF increases after PPVR in stenotic groups but remain unchanged in regurgitant group with dilated RV

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

PR is seen only rarely and in the context of endocarditis  If PR singificant  valve in valve



Most frequent complication is stent fractures (23%, 40%)  If no stenosis or no breakdown stent integrity follow  Significant stenosis  valve in valve  Breakdown of the structural stent integrity  valve in valve

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

Freedom from reoperation of 93 ± 2% at 10 months and 70 ± 13% at 70 months after PPVI, respectively



Freedom from catheter re-intervention at the same intervals was 95 ± 2% at ten and 73 ± 6% seventy months



Currently, surgical-free survival 5 years after is about 70% in one study

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh 50



Almost all reinterventions are due to recurrent obstructions.  Degeneration of biologic valve  BA or valve in valve  Hammock effect (unobserved after modification)  valve in valve  Stent fractures (1/3 of them cause stenosis)  BA/valve in valve



Risk factors for restenosis;  >25 mmHg residual RV-PA pressure gradient ( if there is  BA)  Younger age GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

≈95% of the pts implanted PPV have stenotic RVOT



Impossible in native RVOT after TOF repair enlarged too much



Regurgitant RVOT are mostly undergone to surgery



Relatively small number of pts can benefit from PPVR (≈ 15%) GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

Larger conduits (>22 mm for MV, 27 mm for ESV)



Smaller conduits (balloon dilated diameter < 16 mm)



There are still various unanswered questions about the durability of this valve in the long term (10-15 years?)

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

PPVR has been proven an effective, safe and less invazive procedure in the treatment of dysfunctional RVOT in selected pts, as an alternative to the surgery



Explantation-free survival 5 years after PPVI is about 70%



So, this prolonged conduit lifespan and postponed surgery will reduce the number of multiple open heart operations GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

Improvements have been noted in symptoms and RV volumes, but not in RV systolic function, if it is decreased.



Development of more precise criteria for the timing of PVR in pts with RVOT dysfunction before RV dysfunction should be realized



Better timing of the intervention (before RV dysfunction) may lead to further recovery of the myocardium

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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

PPV will be probably the standard procedure in most of the dysfunctional conduits in the future.



For realizing this, it has to be expanded the scope of the patients  infundibulum reducers for those with dilated RVOT  low-profile but also larger valves should be developed



Further improvements in device design should be focused on also longterm durability to reduce the number of operations to lesser.

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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Al Qethamy H, Momenah T, El Oakley R, Al Redhyan A, Tageldin M, Al Faraidi Y. Minimally invasive transventricular implantation of pulmonary xenograft. J Card Surg. 2008 JulAug;23(4):339-40 Momenah TS, El Oakley R, Al Najashi K, Khoshhal S, Al Qethamy H, Bonhoeffer P. Extended application of percutaneous pulmonary valve implantation. J Am Coll Cardiol.

2009 May 19;53(20):1859-63.

Schreiber C, Hรถrer J, Vogt M, Fratz S, Kunze M, Galm C, Eicken A, Lange R. A new treatment option for pulmonary valvar insufficiency: first experiences with implantation of a selfexpanding stented valve without use of cardiopulmonary bypass. Eur J Cardiothorac Surg. 2007 Jan;31(1):26-30.

Guccione P, Milanesi O, Hijazi ZM, Pongiglione G. Transcatheter pulmonary valve implantation in native pulmonary outflow tract using the Edwards SAPIENโ ข transcatheter heart valve. Eur J GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh Cardiothorac Surg. 2012 May;41(5):1192-4.

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ď&#x201A;Ą

Finally, new devices have to be developed that will allow for PPVI in dilated, distensible outflow tracts, to offer this nonsurgical treatment option to a larger patient population with congenital heart disease.

GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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GHA10th/SHA24th Joint Scientific Conference, Feb 13-16, 2013, Riyadh

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