Critical Left Ventricular Outflow Tract Obstruction (LVOTO) Fall Work Weekend Nov. 2013

Transcription

1 Critical Left Ventricular Outflow Tract Obstruction (LVOTO) Fall Work Weekend Nov. 03

2 Work Weekend Objectives General overview of cohort Generate feasible research question based on current data Early question Develop long term questions of interest Focused chart review and data entry Indication for initial Hybrid procedure

3 Previous CHSS Work Discordant managed BVR patients Survival SAV vs. BAV Hickey et al., McCrindle et al., Ashburn et el.

4 Original Cohort Objectives Assess emerging management strategies Identify risk factors predictive of late outcomes Readjust the CHSS calculator

5 005- Present Entry Criteria Age < 30 days at admission to CHSS institution AV & VA concordance Ductal dependent systemic circulation Excludes patients with AVSD

6 General Profile N % Total Enrollment Initial procedure % Total deaths 4 3% Deaths after intervention 04 30% Deaths prior to intervention 0 % 435 (65%) have had at least one subsequent procedure

7 Morphology Based on Baseline Echo Baseline echo morphology N=658 Echos* Yes % yes No %No Hypoplastic Left Ventricle ** 5 78% 0 Aortic Valve Stenosis 64 5% 404 Aortic Valve Hypoplasia 66 0% 59 Aortic Valve Atresia 34 48% 344 Mitral Valve Stenosis 3 7% 70 Mitral Valve Hypoplasia 96 30% 70 Mitral Valve Atresia 34% 70 Aortic Coarctation 8 8% 45 Hypoplastic Aortic Arch % 00 Interrupted Aortic Arch 5 % 588 *7 patients w/out baseline echo **35 (%) with severe/atretic LV

8 Index Procedure Index Procedure N=665 Ross- Konno N=3 Aortic Valvotomy N=04 Yasui N=5 SVR N=547 HTX N=5 SVCPA N= Deaths prior to intervention=0

9 Surgical Description Code Description N SANO Stage Pallation- Norwood-RV-PA shunt MBT Stage Pallation- Norwood-Systemic to PA shunt HYBR Stage Palliation- Hybrid HYBNR Hybrid to Norwood Cross-over SVCPA Stage SVC-PA Anastomosis HYBSV Stage -Hybrid Single V SVBVCO Single V to Biventricular Cross Over FONTAN Stage 3 Fontan HTX Heart Transplant SVALV Outflow tract and Arch Repairs YASUI Yasui AVR Aortic Valve replacement

10 4 HTX MBT SVALV N=3 AVR SVALV AVR 4 HTX 5 34 HYBR 8 8 CATH N=73 AVR 3 4 SVALV HTX HYBSV 4 FONT HYBV SVCPA AVR MBT 3 SVCPA FONT SANO AORTIC VALVOTOMY N=04 Dead = 0 (3%)

11 63 SVCPA 43 HYBV HTX SVAL FONT 8 67 FONT 70 HTX 39 SVCPA 5 FONT SVAL 7 5 HYBV SANO N=7 FONT 7 FONT HTX 9 SVCPA FONT Norwood N=440 MBT 3 HTX 3 YASUI 4 4 MBT Dead = 84 (%) SANO Dead= 57 (8%) HTX

12 HYBRID N=07 HTX 3 4 HYBNR 6 9 HYBR SVCPA FONT 7 HYBSV 3 FONT MBT HYBSV 9 FONT 3 HTX SVCPA FONT SVBVCO 5 HTX 4 SVCPA 3 FONT YASUI Dead = 4 (6%)

13 Initial Hybrid Procedure N=07 37 = Bilateral PA band alone 67 = Ductal stent + Bilateral PA band 3 = Ductal stent alone 5 SVBVC, Yasui 6 with subsequent Norwood procedure 0 RV-PA shunts 8 MBT shunts 8 Unknown

14 OTHERS N=4 SVCPA N= AVR N=3 HTX N=5 YASUI N=5 MBT 4 3 SVCPA Dead = (%)

15 % survival Survival After Initial Procedure yr=7% N=665 Total Died = 04 68% 75 deaths w/in years of initial procedure Years after initial procedure

16 Unadjusted Comparison: Based on Index Procedure Index Procedure N=665 Aortic AVR Yasui N=3 Valvotomy N=5 SVR N=04 N=547 HTX N=5 Surgical N=3 Cath N=73 MBT N=3 RV-PA N=7 Hybrid N=07

17 % survival Survival: Aortic Valvotomy vs. SVR 8% 68% = Aortic valvotomy (N=04) = Norwood/Hybrid (N=547) Years after initial procedure

18 % survival Survival after SVR: 3 years 76% 66% 63% = RV-PA Shunt (N=7) = Hybrid procedure (N=07) = MBT shunt (N=3) Years after initial procedure

19 % survival Survival after SVR: 6 years 39/4 Hybrid deaths occurred w/in first year 7% 64% 6% = RV-PA Shunt (N=7) = Hybrid procedure (N=07) = MBT shunt (N=3) Years after initial procedure

20 Moving Forward Define succinct research question Baseline data entry is complete (demographic, st, nd procedures, baseline echo) Allocate resources for focused data entry Chart Review: Indications for Hybrid Norwood Alternative V-V decision deferral Salvage Pre-transplant palliation

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23 Potential Questions What are early risk factors for survival after hybrid? What is driving selection of this population? What are the differences among 3 subsets of stage I single ventricular repair? Should the aortic stenosis calculator be re-evaluated?

24 Hazard: Sano, MBT, Hybrid

25 HAZARD: SVR BVR

26 Hazard Hazard for death after initial procedure N=665 Years after initial procedure

27 Background Wide spectrum of morphology Evolving surgical techniques New tools at our disposal Individualized management strategies not well defined

28 Gaps in Knowledge Optimal management Single ventricular repair Biventricular repair Borderline population Late outcomes

29 General Profile 675 patients 665 index procedures Initial procedure 8% represent single ventricular repair 5% represent biventricular repair