RV- PA Surgical Valve Choices in Adults longevity and risks

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RV- PA Surgical Valve Choices in Adults longevity and risks ACHD, Queenstown, 2017

Adult Congenital Heart Surgery 1991 8/3/2017 Total = 751 Patients 200 180 160 140 120 100 80 60 40 20 0 Primary Diagnosis ASD TOF HOCM-Myect VSD Pulmonary Valve Stenosis Ebstein Sub-AS TGA RVOTO+/-VSd CAVC Supra-Valvar AS PAPVD Coarct Sinus Valsalva Aneurysm PDA Cor a. anomaly PFO Misc

Surgery For Adult CHD 1991 Feb 2017 (N=751) 60 50 40 30 20 10 0 1991 1993 1995 1997 1999 2001 2003 2005 2007 2009 2011 2013 2015 2017

Ross procedure exclusive pul. Allo 429 TOTAL 668 *EM = 1 PVR with RV PA conduit insertion (pul. Allograft or porcine) Oct 1991 Feb 2017 TOF: re-operation conduit (incl. PA/VSD) 168 1*complete correction 3 other congenital:- pul. valve. stenosis 38 TGA/PS/ VSD 8 P. atresia / IVS 7 Truncus Arteriosus 7 Miscellaneous 11 242

Tetralogy of Fallot (TOF) Malalignment VSD Overriding aorta RV hypertrophy Pulmonary stenosis (variable) infundibulum - pul. Valve - pul. arteries

TOF - Late problems requiring Surgical intervention Pulmonary Valve Replacement (severe PR) Residual pulmonary stenosis (all levels) Residual VSD RVOT aneurysm Aortic enlargement Other uncorrected or overlooked problems

Indications for PVR - all patients have severe PR Severe PR with symptoms (20-40%) RV enlargement or reduced contractility - RVEDVI > 150mls/ sq m (BSA) - RV > double LV EDV (MRI*) Reduced RVEF, no increase with exercise Severe PR with Ventricular arryhthmias PS/PR

Preop Workup Echo + Right Heart Cardiac Catheterization MRI CT (non contrast) CTCA? (if no C. Cath)

Preop CT Thorax ensure safe sternotomy* other pathology (LSVC) MRI good for RVEDVI, quantification PR, pul. stenoses

Why study CA s? - Anomalous LAD arising Courses across RVOT 3% of TOF cases Op note possibly lost off RCA Potential for damage at reoperation

RPA stenosis

Options for PVR Pulmonary or aortic allograft valve ( tissue bank) Bioprosthetic valve - Medtronic Freestyle porcine

Aortic Allografts, and Stented Bioprostheses Reason: poor haemodynamics poor durability, Ca++

Contegra Bovine Jugular Valved Conduit (Medtronic) used in paediatric patients available in small sizes high stenosis rate in small sizes - 83% for 12mm graft

RVOT Augmentation

Exc. RVOT Aneurysm

Longitudinal Repair RVOT An.

PR/PS and LPA stenosis RPA stenosis

Diffuse RPA stenosis

LPA Stenosis

Risks - minimal Safe operation, often multiple previous sternotomy 1991-2007; 222pts, zero mortality (0%) Few major early complications CVA x 2 (0.9%) both full recovery AF (10%), sternal infection x1, bleeding x 3 Sternotomy, heart beating, only stop heart if intracardiac shunt (PFO, ASD, VSD)

Pulmonary Allograft Pros and Cons Easy insertion, soft and pliable tissue Difficult procurement tissue banks, sizing etc Durability variable mostly good, 7% mild- mod stenosis Occasional early regurgitation (PR) Some fail early, most still good at 20yrs Tendency to calcify variable Shrinkage valve in valve difficult

Homograft failure (mod-sev PR OR RVSP >40) Factors associated with homograft dysfunction 1 Younger recipient Aortic homograft Small size Age of donor? Tissue type disparity?immune mediated reaction? Pannus, excessive fibrosis keloid analogy 1 Sievers et al Circulation 2010

Should be good V in V candidates Medtronic Freestyle Porcine Root Easy to procure - on the shelf More difficult to insert conduit stiff and non pliable Need RVOT patch usually Higher gradients no regurgitation Unknown long term durability -? 20 yrs

Aim is to set patient up for Valve in Valve Because of severe PR, annulus too big Current strategy for next procedure

RV to PA Conduit use Pulmonary Allograft Medtronic Freestyle 1991 2017 2008 2017 81 pts 141 Mean age 33.0yrs 34.5 (range 17-61) (19-80)

RV-PA Conduit use 1991-2017 No. 25 20 15 10 5 0 91 95 99 3 7 11 15 Pul. allograft = 81 Freestyle = 141 Years Pul Allograft Medtronic "Freestyle" 3-D Column 3

Reoperations No Freedom reop Pulmonary Allograft 3 96% at 15yrs (5, 9, and 15yrs) Medtronic Freestyle 1 99% at 5yrs ( 4yrs small conduit) * Now only insert 25,27 or 29mm diameter size valves

Freedom from redo AVR and/or PVR (n=300) after Ross exclusively use Pulmonary Allo. 15 patients 11 AVR only 3 PVR only 1 AVR &PVR * 20yr Freedom Reop 97%* Skillington et al Ann Thorac Surg 2013

Echo Results Valve Pulmonary Allograft Medtronic Freestyle Time period 1991-2017 2008-2017 No. patients 81 141 Echo available 49 (60%) 119 (84%) Echo last 2yrs 26 (53%) 115 (97%) Mean gradient 11.2 (2-38) 16.6 (2-43) (mmhg) Pulm. Regurg. Nil/trivial 33 Nil/trivial 118 Mild 12 Mild 1 Mod 4 Mod 0

Homograft re-operation after Ross Procedure German-Dutch registry: 0.3% per patient year Meta-analysis: 0.55% per patient year

v

Australian Experience with Freestyle valve - WA European J.of Cardiothoracic Surgery Feb, 2016

Freedom from SVD Mean age 21yrs ( 11 50)

Influence of 1st operation on late results in TOF Longer interval if RA/PA approach Minimal trans annular patch Old RV approach led to earlier PVR (worse RV, PR) Mean age 34yrs, interval 30yrs RPA, LPA problems dealt with leads to later reop

Conclusions Safe surgery zero mortality Beating heart surgery improves recovery Better durability of all RV PA conduits in adults cf children Deal with concomitant lesions: RPA, LPA stenoses most challenging; others straight forward (VSD, RVOTO, RVOT aneurysm) Operate before severe TR occurs

Medtronic Freestyle only 9yrs experience - on the shelf availability+ - sl. more difficult insertion - higher gradients, no regurg. -? better valve in valve Conclusions (cont) RVOT augmentation patch important especially for more rigid prostheses eg Freestyle Pulmonary allograft capable of very good long term durability beyond 20yrs, but occasionally fails early, calcifies and shrinks in 7-10%, availability limited

Late Pulmonary Valve Reoperations Ross 2013 Endocarditis : 3,7,9yrs 3 Tubular Stenosis : 11yrs 1 Mean Pulmonary Valve Gradient 11mmHg at 15 yrs

Proximity of L. Innominate v., Asc. Aorta and enlarged RV to sternum

Residual VSD Usually small, peri-patch may or may not show on echo Step up in Oxygen saturation on RH c. cath

Homograft subject to calcific degeneration Common to shrinkage and Ca++ at inflow Rise in gradients seen in first 2 years then stabilises Greater longevity than when used for RVOT repair in congenital heart disease Orthotopic position Normal pulmonary pressures Fate of homograft? El Hamamsy Lancet 2010

R. Heart Catheterization -Pt with PR and additional PS