Critical Left Ventricular Outflow Tract Obstruction (LVOTO) Fall Work Weekend Nov. 03
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
Original Cohort Objectives Assess emerging management strategies Identify risk factors predictive of late outcomes Readjust the CHSS calculator
005- Present Entry Criteria Age < 30 days at admission to CHSS institution AV & VA concordance Ductal dependent systemic circulation Excludes patients with AVSD
General Profile 675 patients Total deaths (3%) 0 deaths prior to any intervention (%) 665 index procedures Initial procedure 8% represent intended -VR 7% represent intended -VR
Morphology Based on Baseline Echo Baseline echo morphology N=658 Echos Yes % Hypoplastic Left Ventricle * 5 78% Aortic Valve Stenosis 6 5% Aortic Valve Hypoplasia 66 0% Aortic Valve Atresia 3 8% Mitral Valve Stenosis 3 7% Mitral Valve Hypoplasia 96 30% Mitral Valve Atresia 3% Aortic Coarctation 8 8% Hypoplastic Aortic Arch 97 76% Interrupted Aortic Arch 5 % *35 (%) with severe/atretic LV
Index Procedure Index Procedure N=665 Ross-Konno N=3 Aortic Valvotomy N=0 Yasui N=5 SVR N=58 N=5 Deaths prior to intervention=0
Surgical Description Total of 39 Procedures Code Description N % Total SANO Stage Pallation- Norwood-RV-PA shunt 9 6 MBT Stage Pallation- Norwood-Systemic to PA shunt 3 7 HYBR Stage Palliation- Hybrid 8 9 HYBNR Hybrid to Norwood Cross-over 6 SVCPA Stage SVC-PA Anastomosis 3 HYBSV Stage -Hybrid Single V 37 3 SVBVCO Single V to Biventricular Cross Over 7 AN Stage 3 Fontan 78 3 Heart Transplant 9 SVALV Outflow tract and Arch Repairs 38 3 BAV Balloon Aortic Valvuloplasty - Cath 73 5 YASUI Yasui AVR Aortic Valve replacement 0
MBT SVALV N=3 AVR SVALV AVR 5 3 HYBR 8 8 BAV N=73 AVR 3 SVALV 5 3 HYBSV SVBVCO SVCPA AVR MBT 3 SVCPA SANO AORTIC VALVOTOMY N=0 Dead = 0 (3% total population)
63 SVCPA SVBVCO 6 9 53 SVAL 8 67 70 39 SVCPA 5 SVAL 7 5 HYBV SANO N=7 7 78 6 8 9 3 SVCPA Norwood N= MBT 3 YASUI MBT Dead = 8 (%) SANO Dead= 57 (8%)
HYBRID N=07 3 HYBNR 6 9 HYBR 3 6 SVCPA 6 7 7 7 HYBSV 3 MBT HYBSV 9 3 SVCPA SVBVCO 5 SVCPA 3 YASUI Dead = (6%)
Initial Hybrid Procedure N=07 37 = Bilateral PA band alone 67 = Ductal stent + Bilateral PA band 3 = Ductal stent alone 7 SVBVC, Yasui 6 with subsequent Norwood procedure 0 RV-PA shunts 8 MBT shunts 8 Unknown
OTHERS N=3 AVR N=3 N=5 YASUI N=5 3 Dead = (%)
% survival Survival After Initial Procedure yr=7% N=665 Total Died = 0 68% 75 deaths w/in years of initial procedure Years after initial procedure
Unadjusted Comparison: Based on Index Procedure Index Procedure N=665 Aortic AVR Yasui N=3 Valvotomy N=5 SVR N=0 N=58 N=5 Surgical N=3 Cath N=73 MBT N= RV-PA N=7 Hybrid N=07
% survival Survival: Aortic Valvotomy vs. SVR 8% 68% = Aortic valvotomy (N=0) = Norwood/Hybrid (N=58) Years after initial procedure
% survival Survival after SVR: 3 years 76% 66% 63% = RV-PA Shunt (N=7) = Hybrid procedure (N=07) = MBT shunt (N=) Years after initial procedure
% survival Survival after SVR: 6 years 39/ Hybrid deaths occurred w/in first year 7% 6% 6% = RV-PA Shunt (N=7) = Hybrid procedure (N=07) = MBT shunt (N=) Years after initial procedure
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
Potential Questions What are the differences among 3 subsets of stage I single ventricular repair? What are early risk factors for survival after hybrid? What is driving selection of this population? Transition to Fontan LVOTO vs. TA
Previous CHSS Work Discordant managed BVR patients Survival SAV vs. BAV Hickey et al., McCrindle et al., Ashburn et el.