Stage-1 Hybrid Palliation for High-Risk 2-Ventricle Patients with Ductal-Dependent Systemic Circulation in the Era of High Prenatal Detection

Objective We reviewed our center's prenatal detection and surgical experience with high-risk, 2-ventricle patients, with complex congenital heart disease that underwent stage-1 hybrid palliation. Methods We retrospectively identified those born between March 2008 and March 2021 with 2-ventricle hearts, complex congenital cardiovascular malformations, and ductal-dependent systemic circulation that underwent stage-1 hybrid palliation consisting of surgical bilateral pulmonary artery banding and interventional catheterization placed ductus arteriosus stents. Results We identified 30 patients. Of the 30, 19 (63%) were male. For the 30, median gestational age was 35 weeks (29-39 weeks), and median birth weight was 2.2 kg (0.6-4.5 kg). Of the 30, 1 was transferred from an adjacent state, and 29 were born in Nevada. Of the 29 born in Nevada, overall statewide prenatal detection was 18 of 29 (62%); however, for 2008 to 2011 the prenatal detection rate was 3 of 10 (30%) and 15 of 19 (79%) for 2012 to 2021, P = .03. For the last 5 years, prenatal detection for Nevada-born patients was 8 of 8 (100%). Two full-term newborns, without a prenatal diagnosis, presented postnatally in extremis. For the 30 patients, there were 0 stage-1 hybrid palliation mortalities, 1 subsequent repair mortality, and 3 late nonsurgical deaths. Conclusions Stage-1 hybrid palliation may result in excellent surgical outcomes for high-risk, 2-ventricle patients. Additionally, high rates of population-wide prenatal detection are possible for high-risk congenital heart disease, allowing prenatal planning and possibly reducing postnatal extremis presentations.

Left heart growth and biventricular repair after hybrid palliation

OBJECTIVES We evaluated the outcomes of biventricular repair after initial hybrid palliation performed in small infants with various forms of left ventricle hypoplasia. METHODS Between September 2010 and January 2020, a total of 27 patients had biventricular repair after hybrid palliation at a median age of 11 days. Indications for the hybrid approach included growth promotion of the left ventricle outflow tract and/or the aortic valve in 14 patients and that of the left ventricle in 13 patients. Seven reinterventions and 7 reoperations were performed during the interstage period. Significant growth of left ventricle parameters was noted during the median interstage period of 62 days. Sixteen subjects had aortic arch repair, ventricular septal defect closure and relief of subaortic stenosis; 5 patients had the Ross–Konno procedure; 5 patients underwent the Yasui procedure; and 1 patient had unbalanced atrioventricular septal defect and aortic arch repair. RESULTS Twenty-three patients (85.2%) are alive at a median follow-up of 3.3 years. Two and 3 patients died early and late after achieving biventricular circulation, respectively. There were 22 reinterventions and 15 reoperations after biventricular repair. CONCLUSIONS Hybrid palliation can stimulate left heart growth in some patients with left ventricle hypoplasia. More patients may eventually achieve biventricular circulation than was initially thought. Additional interventions and operations are foreseeable. Despite ventricular rehabilitation, some patients with borderline left ventricles may develop restrictive physiology.

Abstract 14689: Optimal Prenatal Care Coordination is Associated With Fewer Pre-operative Risk Factors in Babies With a Prenatal Diagnosis of HLHS

Introduction: Prenatal diagnosis (PD) of congenital heart disease permits coordination of care to optimize postnatal outcomes. Using phase II registry data from the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), we sought to identify associations between prenatal care coordination (PNC) and pre-operative status among infants with HLHS and variants intended for Stage I Norwood or hybrid palliation. Hypothesis: We hypothesized that suboptimal PNC would be associated with worse pre-operative status. Methods: The NPC-QIC database was queried for all instances of prenatally diagnosed HLHS and variants intended for Stage 1 Norwood or hybrid palliation from 2016 through 2019. Demographic and clinical data pertaining to the prenatal period through the first birthday were obtained. Optimal PNC was defined as (1) a completed interdisciplinary case conference prior to delivery and (2) a note in the medical record documenting closed-loop communication between the OB and cardiology teams. Associations between PNC and outcomes were identified. Results: Among 1441 registry patients with HLHS and variants, 1242 (86%) had PD. Among patients with PD, optimal PNC was achieved in 68%. Preoperatively, infants without optimal PNC were more likely to have any adverse event (50% vs 40%, p<0.001), receive inotropes (19% vs 13%, p=0.007), require mechanical ventilation (22% vs 16%, p=0.016), and were less likely to feed enterally (53% vs 67%, p <0.001). Lower socio-economic status (SES) was associated with a lower likelihood of PD (p<0.001), and African-American race was associated with a lower likelihood of optimal PNC (p=0.006). Conclusions: Among prenatally diagnosed infants with HLHS and variants, optimal PNC was associated with fewer preoperative risk factors. There also appear to be disparities in PD related to SES and in PNC related to race. Additional study is required to understand the complex interplay between PNC and clinical outcomes, as well as identify and address barriers to PD and optimal PNC.

Tricuspid Valve and Right Ventricular Function Throughout the Hybrid Palliation Strategy for Hypoplastic Left Heart Syndrome and Variants

Background: Tricuspid valve (TV) and right ventricular (RV) function are major determinants of morbidity and mortality in patients with hypoplastic left heart syndrome (HLHS). We sought to retrospectively evaluate these parameters throughout the hybrid palliation strategy. Methods: From 2002 to 2018, 203 patients with HLHS and variants presented for hybrid stage I (HS1). Echocardiographic evaluation of tricuspid regurgitation (TR) and RV function was assessed at multiple time points. Clinical outcomes including tricuspid valvuloplasty, transplantation, and death were reviewed. Results: The most prevalent HLHS subtype was aortic atresia/mitral atresia. The presence of significant TR and/or RV dysfunction was 14.78% and 9.36%, respectively, at the time of initial HS1. There were 185 survivors following HS1 (91.13%, n = 185/203), while 147 patients underwent comprehensive stage II or bidirectional Glenn shunt (72.41%, n = 147/203). Tricuspid valvuloplasty was undertaken in nine patients (4.86%, n = 9/185). Ultimately, 100 patients underwent the Fontan procedure. The odds of development of significant TR and/or RV dysfunction were not statistically different throughout the stages of palliation (TR: odds ratio [OR] = 0.14-0.25, P = .5260; RV dysfunction: OR = 0.02-0.13, P = .3992). However, the risk of death and/or transplant was 2.5- to 3.8-fold when either were present alone or in combination (TR: OR = 2.58, P = .0356; RV dysfunction: OR = 3.84, P = .0262). Transplant-free survival at 15 years was 44.8%. Conclusion: Following hybrid palliation for HLHS, the majority of survivors have normal RV and TV functions. Tricuspid valvuloplasty was required in few patients. Once significant TR and/or RV dysfunction ensues, there is a two- to three-fold risk of death and/or transplant.