Hybrid Therapy for Hypoplastic Left Heart Syndrome Myth, Alternative or Standard?

Transcription

1 Hybrid Therapy for Hypoplastic Left Heart Syndrome Myth, Alternative or Standard? Can Yerebakan, Klaus Valeske, Hatem Elmontaser, Matthias Mueller, Juergen Bauer, Josef Thul, Dietmar Schranz, Hakan Akintuerk Justus-Liebig-University Giessen, Germany AATS 95 th Annual Meeting April, 2015, Seattle, WA, USA

2 No disclosures

3 Hybrid Therapy for HLHS Background Norwood palliation vs. Hybrid palliation

4 Hybrid Therapy for HLHS The Giessen hybrid strategy Neonatal period Giessen hybrid stage I At 4 months Comprehensive Stage II

5 Hybrid Therapy for HLHS Objectives Single institutional retrospective analysis of 118 patients with HLHS Mortality - Early and late survival (including subgroups) Pulmonary artery development (stage II Fontan) Morbidity - Re-interventions on the pulmonary arteries - Outcome of the aortic arch reconstruction

6 Hybrid Therapy for HLHS Patients Giessen hybrid stage I n=182 HLHS (and variants) n=141 HLHC n=41 Htx n=8 Comfort care n=8 (n=7) awaiting BVC n=2 Biventricular correction n=46

7 Hybrid Therapy for HLHS Patients Giessen hybrid stage I with HLHS n=118 AA / MS AA / MA AS / MS AS / MA Others 24% 8% 3% 35% 65% 30%

8 Hybrid Therapy for HLHS Methods Hybrid palliation HLHS Giessen hybrid stage I procedure (n=118) Median age 6 d (0-237) - Median weight 3.2 kg (1.2-7) 1. Surgical BPAB with 3.0/3.5 mm PTFE 2. Ductal stenting in the cath lab h later

9 Hybrid Therapy for HLHS Methods Hybrid palliation HLHS Giessen comprehensive stage II procedure Median age 4.5 mo ( ) - Median weight 5 kg ( ) Concomitant Operative data procedures ASD CPB time, enlargement, min n ( ) (100%) LPA ACC dilation time, min / stent / reconstruction, n (10-163) / 2 / 65 RPA SCP, dilation n / stent / reconstruction, n 191 / (90%) 1 / 16 TVR SCP, time, min 70 5 (4.9%) (17-147) Correction SMP, n of cor triatriatum 20 1 (1%) (20%) Subclavian SMP, time, artery min re-anastomosis/correction 86 3 (3%) (5-170) Postop ECMO therapy, n 5 (4.9%) Delayed sternal closure, n 10 (9.8%) Post-CPB saturation, % 77 (60-90) Post-CPB CVP, mmhg 8 (2-16) Post-CPB PAP, mmhg 17 (7-23) 1. Bilateral debanding with L/RPA dilation/reconstruction 2. Stent removal and aortic arch reconstruction 3. Bidirectional Glenn anastomosis 4. Atrioseptectomy

10 Hybrid Therapy for HLHS Methods Hybrid palliation HLHS Fontan completion Median age 33.7 mo (21-108) - Median weight 13 kg ( ) Concomitant Operative procedures data Fenestration, CPB time, n min (59-192) 4 ACC, mm n 31 (50%) 5 (8 %)...5 ACC mm time, min 4 (6.4%) 58 (44-73) ASD SCP, enlargement n 2 (3.2%) 2 (3.2%) VSD SCP, enlargement time, min 1 (1.6%) 34 (23-45) TVR SMP, n 4 (6.4%) 1 (1.6%) MVR SMP, time, min 1 (1.6%) 27 LPA Postop balloon ECMO dilation therapy, / reconstruction n 6 (9.6%) 1 /(1.6%) 9 (14.5) RPA Delayed balloon sternal dilation closure, / reconstruction n 0 / 7 (11.3%) 0 Aortic Post-CPB arch reconstruction saturation, % (3.2%) (83-100) Glenn Post-CPB takedown CVP1, and mmhg mbt shunt 1 (1.6%) 16 (11-22) Pacemaker Post-CPB implantation CVP2, mmhg 2 (3.2%) 18 (12-25) On beating heart at 34 C mm ring enforced PTFE extracardiac conduit 2. No standard fenestration 3. (PA reconstruction) 4. (AV-Valve repair)

11 Stage III Stage II Stage I 3 deaths (2.5%) Hybrid Therapy for HLHS Results - Mortality Follow-up is complete - median 4.6 years (0-17) Hybrid stage I n=118 2 Htx 8 deaths (6.9%) 4 awaiting stage II 5 deaths (4.9%) Comprehensive Stage II c n=101 3 Htx 5 deaths (5.3%) 0 deaths Fontan operation c n=62 26 awaiting stage III 2 late deaths (one on Htx list) 2 late Htx (both died)

12 Probability of survival Hybrid Therapy for HLHS Results Survival all Patients at risk All patients (n=182) after Giessen hybrid palliation at 1 year = 80 % at 10 years = 79.1% Survival time (years)

13 Probability of survival Hybrid Therapy for HLHS Results Survival HLHS HLHS (n=141) (including comfort care, Htx and BVC) at 1 year = 79.9% at 10 years = 74.5% Patients at risk Survival time (years)

14 Probability of survival Hybrid Therapy for HLHS Results Survival HLHS HLHS (n=118) (excluding comfort care, Htx and BVC) at 1 year = 83.7% at 10 years = 78.2% Patients at risk Survival time (years)

15 Probability of survival Hybrid Therapy for HLHS Results Survival HLHS AA/MA HLHS AA/MA only (n=36) at 1 year = 10 years = 77.4% Patients at risk Survival time (years)

16 Probability of survival Hybrid Therapy for HLHS Results Survival < 2.5 kg BW < 2.5kg (n=33) vs. BW > 2.5kg (n=149) Similar probability of survival p= Survival time (years)

17 LPA diameter (mm) Hybrid Therapy for HLHS Results PA growth in cmri (n=33) RPA diameter (mm) p=0.012 * McGoon ratio p<0.001 * p=0.991 Stage II Fontan Stage II Fontan Stage II Fontan Prior to Fontan operation

18 Probability of freedom from intervention Hybrid Therapy for HLHS Results Freedom from PA intervention Freedom from PA re-intervention after comprehensive stage II at 1 year = 53.8% at 10 years = 32.2% Time after stage II (years)

19 Probability of Freedom from Re-intervention after AAR Hybrid Therapy for HLHS Results Freedom from aortic arch re-intervention 1, ,8 0.8 Probability 0, , , ,0 0.0 (n=46) 9 re-interventions no re-operation Freedom from re-intervention at 10 years = 74% Survival Time (days) (Days)

20 Hybrid Therapy for HLHS Limitations Single institution, retrospective Still short median follow-up Measurement of distal pulmonary arteries in the cmri Limited number of cmri data Availability LPA-stenting

21 Conclusions The hybrid approach emerges for all patients with HLHS as a reasonable alternative to the conventional strategy by the neonatal performance of the Norwood procedure. Successful rehabilitation of the pulmonary arteries after BPAB can be accomplished shown with the outcome of the Fontan operation. Strict interstage follow-up and inter-departmental collaboration is needed. Neurological outcome will probably determine the future role of the hybrid approach in the treatment of HLHS and variants.

22 Thank you for your attention!

23 Hybrid Therapy for HLHS Background

24 Hybrid Therapy for HLHS Biventricular repair

25 Hybrid Therapy for HLHS History in Giessen 1988 first HLHS-TX in University Clinic Giessen (Patient is alive and 26 years old today) Prof.Dr. H.H. Scheld HTX / 56 HLHS-HTX (29%) HTX / 45 HLHS-HTX (48%) HTX / 11 HLHS-HTX (11%)

26 HLHS Outcome < 2500 g HLHS < 2500 g, Ann Arbor Gelehrter et al., Pediatr Cardiol 2011

27 Comparison of Shunt Types in the Norwood Procedure for Single-Ventricle Lesions Richard G. Ohye et al. N Engl J Med 2010;362: North American Centers MBT shunt n=257 pat. RVPA shunt n=274 pat. TX free survival at 12 months 64% (p=0,01) 74% Interventions and complications (p=0,003) (p=0,002) RV size and function at 14 months Similar in two group Follow-up 32+/- 11 months (TX free survival) Nonsignificant difference (p=0,06)

28 Newborn (3.5 kg, 50 cm), LVEDV= 22ml/m 2 AV= 0.49 cm, [0.73 cm ( )] => z-score = -3.1

29 4 mo after Stage I, (6.5 kg, 83 cm), LVEDV= 40 ml/m 2 AV= 9 mm, [0.96 cm ( )] => z-score= -0.64

30 3 years after Comprehensive stage II prior to TCPC LPA

31 LPA stent due to acute thrombosis or stenosis

32 Giessen Hydrid Norwood Procedure Patient over 3kg ; Patient under 3kg; 3,5 mm PTFE prosthesis 3,0 mm PTFE prosthesis

33 Bilateral Pulmonary Arterial Banding 3.5mm graft tube or 3.0mm graft tube RPA LPA Newborn > 3kg or Newborn < 3kg

34 Giessen Hybrid Norwood Procedure After BPAB transfemoral ductal stent implantation 4F wedge cath. 4F + 6F Terumo sheath 4F right Judkins 4F multipurpose Cath. Vygon Vygon 2F arterial needle, cath. Local anesthesia Meaverin + guidewire: 0.014inch floppy wire 0.035inch wire Giessen Colombus

35 Neurological Outcome Mental Development Index MDI MW 100, 1 SD 15 Punkte Psychomotoric Development Index PDI MW 100, 1 SD 15 Punkte

36 50% PDI < 70

37 Motorik PDI MW = 90, n=19, 5% <70

38 Kognition MDI, n=19, MW= 91, 5% <70