Pulmonary Valve Replacement

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1 Indications/Timing and Type of Interventions: Pulmonary Valve Replacement Lars Sondergaard, MD, DMSc Professor of Cardiology Rigshospitalet Copenhagen, Denmark

2 Case 15 years old girl with tetralogy of Fallot Repaired at 1 year of age using transannular patch

3 Free pulmonary regurgitation Transannular patch in TOF repair AHA November 5 th 2007

4 Case 15 years old girl with tetralogy of Fallot Repaired at 1 year of age using transannular patch Asymptomatic, active football player Unwell, palpitation, vomiting after football match Called for ambulance

5 Case DC shock

6 Free pulmonary regurgitation RV RV PA LV

7 Case 15 years old girl with tetralogy of Fallot Repaired at 1 year of age using transannular patch Asymptomatic, active football player Unwell, palpitation, vomiting after football match Called for ambulance Homograft and ICD

8 Case 15 years old girl with tetralogy of Fallot Repaired at 1 year of age using transannular patch Asymptomatic, active football player Unwell, palpitation, vomiting after football match Called for ambulance Homograft and ICD Could this SCD have been prevented?

9 Freedom form ventricular tachycardia p > Gatzoulis et al. Lancet 2000; 356:975-81

10 Risk factors for arrhythmia and SCD RV pressure >60 mmhg TR >mild PR >mild Gatzoulis et al. Lancet 2000; 356:975-81

11 Free pulmonary regurgitation Free PR may eventually cause ventricular dysfunction exercise intolerance arrhythmias and sudden death late after repair of tetralogy of Fallot Important to maintaining a competent valve, but optimal timing of intervention remains to be established AHA November 5 th 2007

12 Animal study on free PR and RV

13 Study protocol Group A PR Valve Group B PR Valve Group C PR Valve Control group D 0 1/12 2/12 3/12 4/12

14 Increase in RVEDVI (but not duration of PR) is predictor for non-recovery of RV RVESVI 75 ml/m 2 RVEDVI 200 ml/m 2 Change in RV volume index from baseline to PVR (ml/m 2 ) Ersboell et al. Int J Cardiol. 2013; 167:

15 Optimal timing of PVR Midterm outcomes of PVR in patients with chronic PR were acceptable. PVR should be considered before RVEDVI exceeds 163 ml/m 2 or RVESVI exceeds 80 ml/m 2, with more attention to RVESVI. Lee et al. JACC 2012; 60:

16 PVR for free PR late after repair for TOF Pulmonary valve replacement at mean 21.8 years after TOF repair Therrien et al. JACC 2000; 36:1670-5

17 Pre-operative RVESVI and outcome Good outcome Poor outcome Bokma et al. Eur Heart J. 2016; 37:829-35

18 Risk factors for rapid RV enlargement Increase of RVEDVI >5 ml/m 2 per year Decrease of RVEF 2.2% per year Shin et al. EJCTS 2016; 50:464-9

19 Restrictive RV physiology Doppler in RVOT Free PR Atrial systoli Forward flow

20 Palliative shuns before TOF repair Volume loaded ventricles

21 Right bundle branch block

22 QRS duration and syncope/scd QRS >180 ms Gatzoulis MA et al Circulation 1995;92:231-7

23 Impact of PVR on QRS duration Pulmonary valve replacement (N=70) Control group (N=30) 4.7 years 4.9 years 178 ms 176 ms 171 ms 181 ms Therrien et al. Circ 2001; 103:

24 Impact of PVR on clinical arrhythmia 4.7 years 4.9 years Therrien et al. Circ 2001; 103:

25 No effect of PVR on peak oxygen pulse Oxygen pulse = oxygen uptake divide by heart rate R=0.86, p> Legendre et al. CITY 2016; 26:1310-8

26 No effect of PVR on peak oxygen pulse Oxygen pulse = oxygen uptake divide by heart rate R=0.86, p> May reflect irreversible right and/or left ventricle lesions. Encourage pulmonary valve replacement in Patients showing any decrease in peak oxygen pulse during their follow-up Legendre et al. CITY 2016; 26:1310-8

27 Indications for intervention after repair of TOF Optimal timing remains challenging. Longitudinal data are more important than single measurements to assist timing for re-intervention. Normalization of RV size after re-intervention becomes unlikely as soon as the end-diastolic volume index exceeds 160 ml/m 2 ESC Guidelines for the management of grown-up congenital heart disease. EHJ 2010; 31:

28 Indications for intervention after repair of TOF Optimal timing remains challenging. Longitudinal data are more important than single measurements to assist timing for re-intervention. Normalization of RV size after re-intervention becomes unlikely as soon as the end-diastolic volume index exceeds 160 ml/m 2 ESC Guidelines for the management of grown-up congenital heart disease. EHJ 2010; 31:

29 Indications for intervention after repair of TOF Class I Class II ACC/AHA 2008 Guidelines for the management of ACHD. Circulation 2008; 118: e

30 Across guidelines Asymptomatic patients Tretter et al. int J Cardiol 2016; 221:916-25

31 Across guidelines Asymptomatic patients CMRI Echocardiography ECG, Holter monitoring Exercise test Tretter et al. int J Cardiol 2016; 221:916-25

32 Strategy Follow-up of patients with free PR after repair of TOF should include regular: Function class, ECG, echocardiography etc. Exercise test CMRI Consider PVR in Symptomatic patients Asymptomatic patients where longitudinal data shows deterioration

33 Type of interventions Surgical PVR Homograft Contegra graft Stented bioprosthetic valves Transcatheter PVR Balloon expandable Self-expandable

34 Balloon expandable THV Medtronic Melody valve Edwards SAPIEN XT Transcatheter Heart Valve

35 PVR for PR in conduit-free RVOT N = 23 Median age 12.2 years (range years) Median weight 40 kg (range kg) Cools et al. Int J Cardiol 2015; 186:129-35

36 Self-expanding THV Medtronic Harmony valve Native (conduit free) RVOT Venus P valve Native RVOT & conduits

37 Harmony TPV Device Porcine Pericardial Tissue Valve AOA treated tissue 22 mm ID Tissue valve mounted on self-expanding nitinol frame with polyester cloth covering Single device size for EFS Delivery System Coil loading catheter 1 size: 25 Fr Loading funnel to collapse valve prior to sheathing Implanter Summit 2016 HARMONY TM TPV Early Feasibility Study

38 Pre-implantation angiogram

39 Post-implantation angiogram

40 Should we intervene earlier? Tretter et al. int J Cardiol 2016; 221:916-25

41 Should we intervene earlier? Pulmonary valve replacement as soon as an adult size bioprosthesis can be used? Tretter et al. int J Cardiol 2016; 221:916-25

42 Issues with regards to earlier PVR Benefits Long-term preservation of RV function less exercise intolerance, arrhythmia, SCD transcatheter PVR may be possible (initial or later) Problems Needs multiple replacements during lifetime mean life-span of 15 years for bioprosthesis Transcatheter PVR may not be possible Procedural risks Endocarditis, stent fractures

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