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Prof Ahmet Ă&#x2021;elebi 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

 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

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

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The most common reason of conduit dysfunction needing surgery is stenosis

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

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

minimize the total number of open heart surgeries

intervening before right ventricle dysfunction

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

Can we delay the surgical reoperations

by catheter interventions? 

PPVR; for stenosis, regurgitation or both

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Balloon angioplasty (BA); for stenosis

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

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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

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

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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

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Confirms the most stenotic part of the conduit

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Stent migrations observed less frequently in predilated pts

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

Cranial 35o

900 lateral

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

Cranial 35o

900 lateral

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

Cranial 35o

900 lateral

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

Caudal 35o LAO 10

900 lateral

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

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

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

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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

 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

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

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

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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

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

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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

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

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RV/LV diameter ratio ≥1.5 on MRI

Asymptomatic pts 

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

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

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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

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

Melody valve TPV delivery systems; up to BIB sizes

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

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

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18, 20, 22 mm

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

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

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

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

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

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

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

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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

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

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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 ,

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Btw 20 & 27 mm, for Edwards Sapiens valve

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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

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

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

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

Cranial 35o

900 lateral

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

Cranial 35o

900 lateral

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

Cranial 35o

900 lateral

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

Cranial 35o

900 lateral

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

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

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Eliminates PR, ≤trivial in most of the pts .

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Diastolic PA pressures rise immediately after deployment

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

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Residual stenosis

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Device migration

system in the tendinous cords of



Device misplacement

the TV

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Coronary compression

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Rupture of blood vessels

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Homograft rupture

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Hammock effect/stenosis

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PA obstruction (jailing)

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Local vascular complications

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Entrapment of the delivery

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

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Clinical symptoms improve within 6 months after PPVI.

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

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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

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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

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

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

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

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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

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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

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Impossible in native RVOT after TOF repair enlarged too much

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Regurgitant RVOT are mostly undergone to surgery

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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)

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Smaller conduits (balloon dilated diameter < 16 mm)

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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

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

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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



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

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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



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

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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

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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

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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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