Selective Management Strategy for Neonates with Interrupted Aortic Arch Mitigates Future Left Ventricular Outflow Tract Obstruction Risk

Transcription

1 Selective Management Strategy for Neonates with Interrupted Aortic Arch Mitigates Future Left Ventricular Outflow Tract Obstruction Risk Bahaaldin Alsoufi, Brian Schlosser, Courtney McCracken, Ritu Sachdeva, William Border, Brian Kogon, William Mahle, Kirk Kanter Children s Healthcare of Atlanta, Emory University, Atlanta, GA

2 Background Single stage repair of IAA: standard treatment strategy Improved survival LVOT obstruction: common and challenging complication LVOT reoperation: associated with significant mortality and reoperation risk Association between LVOT obstruction and initial morphology / LVOT dimensions

3 Proportion of patients (%) Alive without LVOT reoperation Many early reoperations CHSS study, (n=453) Years since admission LVOT reoperation Death 38% 34% 28% McCrindle et al, JTCVS 2005

4 Proportion of patients (%) Alive without 2 nd LVOT reoperation Death after reoperations Death 2 nd LVOT reoperation Years since first LVOT procedure 37% 35% 28% McCrindle et al, JTCVS 2005

5 Aims Report outcomes of selective management strategy that addresses LVOT risk at time of IAA repair Survival Reoperation: LVOT, arch, all cardiac Competing risks analysis: Death without LVOT reoperation LVOT reoperation Remaining alive without LVOT reoperation

6 Patients N=77 ( ) Age (IQR) 7 days (5-10) Weight (IQR) 3.0 Kg ( ) Weight 2.5 Kg 20 (26%) Prematurity 36 Wk 12 (16%) Genetic syndromes 46 (60%) DiGeorge 41 (53%)

7 Surgeries Standard IAA repair and VSD closure: N=53 Selective Patch cerebral arch augmentation perfusion

8 Surgeries LVOT enlargement with conal resection: N=7 Suzuki et al, Circulation 2006

9 Surgeries LVOT bypass with DKS / arch: Single stage: Yasui n=3/17 Staged: Norwood followed by Rastelli: n=14/17 Kanter, OTTCVS 2010

10 Patients Standard repair (n=53) Conal resection (n=7) LVOT bypass (n=17) p- value Age (days) 7 (5 10) 8 (5 11) 8 (5 10) 0.91 Male gender 3.0 ( ) 3.4 ( ) 3.2 ( ) 0.19 Weight (Kg) 36 (67.9%) 6 (85.7%) 15 (88.2%) 0.22 Weight < 2.5 Kg 8 (15.4%) 3 (42.9%) 1 (5.9%) 0.10 Prematurity 36 weeks 30 (56.6%) 5 (71.4%) 11 (64.7%) 0.71 Genetic syndromes 7 (5 10) 8 (5 11) 8 (5 10) 0.91

11 Patients Standard repair (n=53) Conal resection (n=7) LVOT bypass (n=17) p- value Age (days) 7 (5 10) 8 (5 11) 8 (5 10) NS Male gender 3.0 ( ) 3.4 ( ) 3.2 ( ) NS Weight (Kg) 36 (67.9%) 6 (85.7%) 15 (88.2%) NS Weight < 2.5 Kg 8 (15.4%) 3 (42.9%) 1 (5.9%) NS Prematurity 36 weeks 30 (56.6%) 5 (71.4%) 11 (64.7%) NS Genetic syndromes 7 (5 10) 8 (5 11) 8 (5 10) NS

12 Patients Standard repair (n=53) Conal resection (n=7) LVOT bypass (n=17) p- value IAA type B 76% 100% 88% Aberrant R SCVA 42% 57% 71% BAV 61% 86% 88% AV Z score < AV annulus - weight < AV CSA (cm 2 /m 2 ) < LVOT CSA (cm 2 /m 2 ) LVOT / Desc. AO Asc. Ao Z score Proximal arch Z score

13 Patients Standard repair (n=53) Conal resection (n=7) LVOT bypass (n=17) p- value IAA type B 76% 100% 88% Aberrant R SCVA 42% 57% 71% BAV 61% 86% 88% AV Z score < AV annulus - weight < AV CSA (cm 2 /m 2 ) < LVOT CSA (cm 2 /m 2 ) LVOT / Desc. AO Asc. Ao Z score Proximal arch Z score

14 Patients Standard repair (n=53) Conal resection (n=7) LVOT bypass (n=17) p- value IAA type B 76% 100% 88% Aberrant R SCVA 42% 57% 71% BAV 61% 86% 88% AV Z score < AV annulus - weight < AV CSA (cm 2 /m 2 ) < LVOT CSA (cm 2 /m 2 ) LVOT / Desc. AO Asc. Ao Z score Proximal arch Z score

15

16 LVOT REOPERATION

17 Persistent risk

18 Steady increase in LVOT reoperations 76%

19 Proportion of patients (%) Alive and free from LVOT Reoperation At 1 year: At 10 years: 7% dead, 4% LVOT reoperation 9% dead, 21% LVOT reoperation LVOT Reoperation Death prior to LVOT Reoperation Years since initial operation

20 Freedom from LVOT reoperation 100% 90% 80% 70% 60% 50% 40% 30% 20% 10% 0% LVOT Bypass Standard Repair Conal Resection N at Risk: Years Since Surgery Completion

21 ARCH REOPERATION

22 93%

23 ALL CAUSE CARDIAC REOPERATION

24 60%

25 Freedom from Cardiac Reoperation 100% 90% 80% 70% 60% 50% 40% 30% 20% 10% 0% R LVOT bypass: conduit change Conal resection: LVOTO Standard Repair LVOT Bypass Conal resection Years Since Initial Surgery

26 SURVIVAL

27 86%

28 Patient Survival 100% 90% 80% 70% 60% 50% 40% 30% 20% 10% 0% Standard Repair Conal resection LVOT Bypass Years Since Initial Surgery

29 Summary A selective management strategy that is customized to the degree of aortic valve and subaortic area narrowing: Decreases LVOT reoperation need Prolongs interval to LVOT reoperation Decreases LVOT reoperation complexity Decreases mortality risk following LVOT reoperation The effect of aortic valve and LVOT narrowing on increased LVOTO risk is neutralized with LVOT bypass procedures however it continues to be the highest following conal resection The trade off in LVOT bypass is reoperation for conduit change