Pulmonary Regurgitation after TOF Repair. How to Assess and Options of Management? Worakan Promphan, MD.FSCAI.

Transcription

1 Pulmonary Regurgitation after TOF Repair. How to Assess and Options of Management? Worakan Promphan, MD.FSCAI. Queen Sirikit National Institute of Child Health (QSNICH) Bangkok, Thailand

2 How to Assess?

3 5 5 1 Competent pulmonary valve

4 SEVERE Incompetent pulmonary valve

5 14 SEVERE Incompetent pulmonary valve

6 Incompetent pulmonary valve Geva T. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 26.

7 1.Exercise intolerance 2.Arrhythmia/ sudden cardiac death Geva T, et al. JACC 24 RV & LV interaction in Post TOF repair with severe

8 QRS duration and outcomes 18 ms n= 182 Gatzoulis M et al. Circulation 1995;92:

9 Circulation. 28;117:363-37

10 Predictors of Sudden Cardiac Death History Electrophysiologic Hemodynamic Syncope Later age at repair Associated morbidity Activity level QRS Duration Incidence of SVT Sustained SVT Enlargement of RV Severity of PR Severity of TR RV & LV function LVEDP

11 Indications for Pulmonary Valve Replacement Symptoms Asymptomatic Incidental Exercise intolerance Syncope Anticipation of symptoms: Planning for pregnancy Information requires! Electrophysiologic Echocardiographic Cardiac MRI Exercise testing Cardiac cath PS, RVOTO Residual VSD RVOT/PA aneurysm Severe TR AR

12 Non invasive RV assessment Echo as a screener CMR as a reference Echo 1. Volume 2. Function 1. TAPSE 2. FAC 3. TDI 4. Strain and strain rate CMR 1. RV volumes 2. RV flow, PA flow, PV flow 3. Delayed enhancement

13 Echocardiogram 2D-measurements for RV volume Lack of normative data for the different measurements. Poorly correlate with MRI measurements* Interobserver variability is relatively high** Moving towards 3D-volumatrics method * Lai et al. Int J CV Imaging 28 ** Greutmann JASE 21

14 Emanuela R, et al. Eur Heart J 212 Tricuspid annular plane systolic excursion (TAPSE) Normal value > 16 mm Echocardiogram Assessment of RV function

15 TAPSE decreased after TOF repair Years following surgical repair Koestenberger JASE 211

16 Poor correlation between TAPSE- RVEDVi and TAPSE- RVEF in TOF * *Koestenberger JASE 211

17 Echocardiogram Assessment of RV function Reasonably correlated with MRI RVEF* Fractional area change (FAC) Normal value > 35% *Anavekar, Emanuela EchocardIography R, et al. Eur Heart J (EDA-ESA)/EDA

18 Cardiac MRI Assess function PR RF RVEDV(i), LVEDV(i) RVEF, LVEF, etc. Emanuela R, et al. Eur Heart J 212 Assess anatomy RVOT aneurysm Branch PA stenosis Residual shunt, etc.

19 RVEDVi < 14 ml/m2 is (perhaps) what we aim for treatment Am J Cardiol 212;19(3):417-22

20 RVEDVi (CMR) vs. Area RVEDi (Echo) RVEDi 2.43 cm 2 /m 2 sensitivity 64%,specificity 83% Katanyoowong P, et al. WPCCS 213

21 Everything is straight forward? Not really!

22 Not all patients behave the same! Repaired in 1998 Repaired in 2 Both having severe PR

23 Incompetent pulmonary valve 14

24 Incompetent pulmonary valve with RESTRICTIVE RV Stiff RV 14 or less Chaturvedi RR, Redington A. Heart 27;93;88-889

25 Restrictive physiology predicted Smaller heart on CXR Smaller RV Better exercise tolerance Shorter QRS duration Gatzoulis et al. Circulation 1995;92:231-7.

26 How to Assess PR after TOF repair? Clinical symptoms: syncope, exercise intolerance CXR heart size Echocardiogram FAC, RVEDAi, LVEF and other. CMR RVEDVi and other.

27 Indications for PVR (MY PRACTICE!) Symptoms Asymptomatic Incidental Exercise intolerance Syncope Anticipation of symptoms: Planning for pregnancy Increase heart size (CT ratio >.5) (CXR) Plus QRS > 18 ms. (ECG) or RVEDAi > 2 cm 2 /m 2 or worsening of LV systolic function (Echo) or RVEDVi > ml/m 2 (CMR) PS, RVOTO Residual VSD RVOT/PA aneurysm Severe TR AR

28 Options of Management? Scalpel is a gold standard. Catheter is an emerging modality.

29 Geva JCMR 211, 13:9 Perioperative and late mortality of pulmonary valve replacement after TOF Institution Year Number of patient SUNY, Syracus Children s Memorial Hospital, Chicago University of Toronto Mayo Clinic Children s Hospital, Atlanta Leiden University, The Netherlands New England Med Center, Boston University of Zurich, Switzerland Multicenter, The Netherlands University of Toronto University Medical Center, Rotterdam International Society of Congenital Heart Disease Great Ormond Street, London Emony University Children s Hospital, Boston Children s Hospital, Atlanta Operative death Average Length of Follow up Time (year) , Late Death or Transplantation % 2.2%

30 Roderick W, et al. JACC 21;56:

31 Percutaneous valve implantation Melody TM Valve (Medtronic) Edwards SAPIEN TM Valve (Edwards Lifescience) Venus P-valve (Venus MedTech)

32 Percutaneous pulmonary valve implantation Year Valve Number of patient Operative death Average Length of Follow up Time (month) Late Death Italian Society of Pediatric Cardiology Gillespie MJ, et al. COMPASSION study Melody TM Melody TM SAPIEN TM

33 Melody TM Valve Endocarditis 311 cases. Med F/U time 2.5 yrs..88% / patient year McElhinney DB, et al. Circ Cardiovasc Interv 213;6: Zahn E, PCCS213

34 Procedure charge is ~ 5, USD cheaper for PPVI than surgery in Virginia

35 Conclusion CXR is still a useful follow-up tool. Decision making depends on individual circumstances than a set of numbers from the tests. Time for valve replacement. Surgery v/s intervention.