Abstract

Introduction: Unplanned intervention before stage 2 palliation (S2P) in patients with single ventricle (SV) lesions with dual unobstructed outflows palliated with pulmonary artery banding (PAB) is common. The factors contributing to intervention and its association with successfully undergoing S2P are unclear. Hypothesis: Early intervention is more common when PAB is placed at a younger age and with a lower initial PAB pressure gradient. Methods: Retrospective single center study of patients with SV lesions undergoing PAB between Jan 2000 and Dec 2020. Association with reintervention and successful S2P was modeled using exploratory cause-specific hazard regression. A multivariable model was developed adjusting for clinical and statistically relevant predictors. The cumulative proportion of patients undergoing intervention were summarized using a competing risk model. Results: A total of 77 patients - 53% female, 86% term and 12% syndromic - underwent PAB at median (IQR) 47 (24-66) days and 3.73 (3.2-4.5) kg. Within 18 months of PAB, 60 (78%) reached S2P, 9 (12%) died, 1 (1%) transplanted and 7 (9%) were alive without S2P. Intervention occurred in 18 (23%) prior to S2P (10/18) or death (4/18); 16 (21 %) occurred within 6 months of PAB. Intervention included PAB adjustment (n=7), conversion to DKS/BTT shunt (n=5), balloon atrial septostomy (n=4) and addition of a BTT shunt (n=2). Repeat intervention was required in 4 patients. Intervention was associated with genetic syndrome (p= 0.03) and lower weight at PAB (p=0.02) but not with age at PAB, pre/postoperative oxygen saturation, PAB length or gradient. Presence of genetic syndrome was the only risk factor associated with not reaching S2P (p=0.02). Conclusions: Intervention is common following PAB and associated with presence of genetic disorder or smaller patient size. Children with genetic syndrome aiming to undergo S2P are at higher risk for intervention and death and may benefit from alternate treatment strategy.

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