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Excellent Survival And Tricuspid Valve Function At One-year In A Hybrid Stage I Palliation Program For Hypoplastic Left Heart Syndrome
Neil K. Worrall, MD, MA, Kaleena Trimpe, Jason Konyu, Carl Garabedian, MD.
Sacred Heart Childrens Hospital, Spokane, WA, USA.

Objective: The role of hybrid palliation (HS1) of HLHS is unclear. Many centers restrict usage to high-risk patients whereas others apply it liberally. We reviewed our experience in a series of consecutive patients.
Methods: 46 newborns underwent surgical management (40 HS1, 6 primary Norwood (100% survival; not included in analysis)). Twenty were high risk (birth weight <2.5 kg (10; 3≤1.8 kg), intact/highly restrictive atrial septum (6; stented prior to HS1), TAPVR (2), and/or neurological malformation (4)) and 20 standard risk. 53% had aortic atresia. HS1 consisted of bilateral PA banding and trans-PA ductal stenting in a hybrid OR. Balloon septostomy was performed electively for ASD gradient ≥8 mmHg. Comprehensive stage II (de-banding, Norwood, Glenn) was performed at 4-6 months.
Results: (Table) 96% survived S1 (95% hybrid (100% BW<1.8kg and/or obstructed atrial septum), 100% Norwood). Nine required Norwood conversion prior to Glenn (67% survived). No late deaths occurred after reaching Glenn. One-year survival was 70% (67% BW<1.8kg, 80% atrial septal obstruction/intact). Tricuspid valve function is excellent: ≤2+ (mild) TR in all patients at 1 year, one progressed to 3+ by Fontan (repaired), and mean TR was 1.1+ at long-term follow-up.
Conclusions: We demonstrated excellent results with HS1 management of HLHS, particularly in those at high-risk for Norwood. HS1 palliation is an suitable alternative for patients with HLHS and confers excellent long-term tricuspid valve function.

Hybrid Stage I Outcomes
All (40)High Risk (20)Standard Risk (20)
BW (kg; median (range))3.0 (1.7-4.2)2.5 (1.7-4.2)3.2 (2.7-4.2)
Stage I Intubation Time (h)24.6 (0-337)30.3 (6-337)10.8 (0-120)
Stage I POLOS (d)*16 (5-137)28 (11-78)9 (5-35)
Overall Stage I Operative Survival*38/40 (95%)19/20 (95%)19/20 (95%)
Comprehensive Stage II Intubation Time (h)66 (6-353)107 (170-196)65 (6-286)
CS2 POLOS (d)15 (7-39)25 (14-33)10 (7-39)
CS2 Operative Survival21/23 (91%)7/9 (78%)14/14 (100%)
Overall 1 year survival26/37 (70%)9/18 (50%17/20 (85%)
*inclusive of early Norwood conversions


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