Comprehensive Approach to the Management of Patients with HLHS: Analysis of 100 Consecutive Neonates.

BACKGROUND: We report our comprehensive approach to patients with hypoplastic left heart syndrome (HLHS) and describe our outcomes in 100 consecutive neonates. METHODS: One-hundred consecutive neonates (2015-2023) were stratified into 3 pathways: Pathway(1): 77/100=77% were standard-risk and underwent initial Norwood (Stage 1). Pathway(2): 10/100=10% were high-risk with noncardiac risk factors and underwent initial Hybrid Stage 1. Pathway(3): 13/100=13% were high-risk with cardiac risk factors: 10 underwent initial Hybrid Stage 1 + ventricular assist device insertion (HYBRID+VAD), while 3 underwent primary transplantation. RESULTS: One-year mortality=9/100=9%. Pathway(1): Operative Mortality for initial Norwood (Stage 1)=2/77=2.6%. Of 75 survivors of Norwood (Stage 1): 72 underwent successful Glenn, 2 underwent successful biventricular repair, and 1 underwent successful cardiac transplantation. Pathway(2): Operative Mortality for initial Hybrid Stage 1 without VAD=1/10=10%. Of 9 survivors of Hybrid (Stage 1): 4 underwent successful cardiac transplantation, 2 died while awaiting cardiac transplantation, 3 underwent Comprehensive Stage 2 (with 1 death), and 1 underwent successful biventricular repair. Pathway(3): Of 10 HYBRID+VAD: 7/10=70% underwent successful cardiac transplantation and are alive today and 3/10=30% died on VAD while awaiting transplantation. Median VAD support time=134 days (range=56-226). (Two of three patients who were bridged-to-transplant with prostaglandin underwent successful transplantation and one died while awaiting transplantation.) CONCLUSIONS: A comprehensive approach to the management of patients with HLHS is associated with Operative Mortality after Norwood of 2/77=2.6% and an overall one-year mortality of 9/100=9%. 10/100 patients=10% were stabilized with HYBRID+VAD while awaiting transplantation. VAD facilitates survival on the waiting list during prolonged wait times.

15-Year Analysis of Surgical Approaches and Outcomes for Coarctation in 132 Neonates and Infants.

A variety of surgical approaches exist to treat aortic coarctation in neonates and infants. Our institutional approach is designed to match the surgical approach to the individual anatomy of the patient. The objective of this study is to evaluate operative characteristics and outcomes of all neonates and infants who underwent surgical repair of coarctation of the aorta or hypoplastic aortic arch at University of Florida from 2006 to 2021, inclusive, either in isolation or with concomitant repair of atrial septal defect (ASD) and/or ventricular septal defect (VSD). A retrospective review was performed of 132 patients aged 0-1 year who underwent surgical repair of aortic coarctation or hypoplastic aortic arch between 2006 and 2021, inclusive, either in isolation or with concomitant repair of ASD and/or VSD. Patients were divided into two groups based on the surgical approach: Group 1 = Median Sternotomy and Group 2 = Left Lateral Thoracotomy. Continuous variables are presented as median (minimum-maximum); categorical variables are presented as N (%). The most common operative technique in Group 1 was end-to-side reconstruction with ligation of the aortic isthmus. The most common operative technique in Group 2 was extended end-to-end repair. Operative Mortality was one patient (1/132 = 0.76%). Transcatheter intervention for recurrent coarctation was performed in seven patients (7/132 = 5.3%). Surgical re-intervention for recurrent coarctation was performed in three patients (3/132 = 2.3%). From these data, one can conclude that a strategy of matching the surgical approach to the anatomy of neonates and infants who underwent surgical repair of aortic coarctation or hypoplastic aortic arch, either in isolation or with concomitant repair of ASD and/or VSD, is associated with less than 1% Operative Mortality and less than 3% recurrent coarctation requiring reoperation.

Venovenous extracorporeal membrane oxygenation neck cannulation in an infant.

In this video tutorial, we present neck cannulation for venovenous extracorporeal membrane oxygenation using a crescent right atrial double lumen cannula in a 4-kg baby.

Ventricular assist device support in neonates and infants with a failing functionally univentricular circulation.

Some neonates with functionally univentricular hearts are at extremely high risk for conventional surgical palliation. Primary cardiac transplantation offers the best option for survival of these challenging neonates; however, waitlist mortality must be minimized. We have developed a comprehensive strategy for the management of neonates with functionally univentricular hearts that includes the selective use of conventional neonatal palliation in standard-risk neonates, hybrid approaches in neonates with elevated risk secondary to a noncardiac etiology, and neonatal palliation combined with insertion of a single ventricular assist device (VAD) in neonates with elevated risk secondary to a cardiac etiology. Here we describe our selection criteria, technical details, management strategies, pitfalls, and current outcomes for neonates with functionally univentricular hearts supported with a VAD. Our experience shows that extremely high-risk neonates with functionally univentricular hearts who are poor candidates for conventional palliation can be successfully stabilized with concomitant palliation and pulsatile VAD insertion while awaiting cardiac transplantation.

A Comprehensive Approach to the Management of Patients With HLHS and Related Malformations: An Analysis of 83 Patients (2015-2021).

Background: Some patients with hypoplastic left heart syndrome (HLHS) and HLHS-related malformations with ductal-dependent systemic circulation are extremely high-risk for Norwood palliation. We report our comprehensive approach to the management of these patients designed to maximize survival and optimize the utilization of donor hearts. Methods: We reviewed our entire current single center experience with 83 neonates and infants with HLHS and HLHS-related malformations (2015-2021). Standard-risk patients (n = 62) underwent initial Norwood (Stage 1) palliation. High-risk patients with risk factors other than major cardiac risk factors (n = 9) underwent initial Hybrid Stage 1 palliation, consisting of application of bilateral pulmonary bands, stent placement in the patent arterial duct, and atrial septectomy if needed. High-risk patients with major cardiac risk factors (n = 9) were bridged to transplantation with initial combined Hybrid Stage 1 palliation and pulsatile ventricular assist device (VAD) insertion (HYBRID + VAD). Three patients were bridged to transplantation with prostaglandin. Results: Overall survival at 1 year = 90.4% (75/83). Operative Mortality for standard-risk patients undergoing initial Norwood (Stage 1) Operation was 2/62 (3.2%). Of 60 survivors: 57 underwent Glenn, 2 underwent biventricular repair, and 1 underwent cardiac transplantation. Operative Mortality for high-risk patients with risk factors other than major cardiac risk factors undergoing initial Hybrid Stage 1 palliation without VAD was 0/9: 4 underwent transplantation, 1 awaits transplantation, 3 underwent Comprehensive Stage 2 (with 1 death), and 1 underwent biventricular repair. Of 9 HYBRID + VAD patients, 6 (67%) underwent successful cardiac transplantation and are alive today and 3 (33%) died while awaiting transplantation on VAD. Median length of VAD support was 134 days (mean = 134, range = 56-226). Conclusion: A comprehensive approach to the management of patients with HLHS or HLHS-related malformations is associated with Operative Mortality after Norwood of 2/62 = 3.2% and a one-year survival of 75/83 = 90.4%. A subset of 9/83 patients (11%) were stabilized with HYBRID + VAD while awaiting transplantation. VAD facilitates survival on the waiting list during prolonged wait times.

Antibodies to human leukocyte antigens and their association with blood product exposures in pediatric patients undergoing cardiac transplantation.

BACKGROUND AND AIMS: Previous blood product exposures may result in the development of antibodies to human leukocyte antigens (HLA). Pediatric heart transplant recipients who have these antibodies experience increased morbidity and mortality after transplantation. In this study, our aims were to confirm the association of previous allogeneic blood product exposures with the formation of anti-HLA antibodies, determine which blood components pose the greatest risk of developing antibodies, and assess differences in outcomes after transplantation between patients who had anti-HLA antibodies and those who did not. METHODS: This retrospective investigation included all children who underwent cardiac transplantation at Children's Healthcare of Atlanta from January 1, 2015 through December 31, 2018. Chart reviews were performed to collect pertinent data. Anti-HLA antibodies were detected by single antigen bead testing. Antibody burden was tabulated using the calculated panel reactive antibody (cPRA) score immediately prior to transplantation. Statistical analyses were conducted to examine differences based on HLA antibody status and identify associations with outcomes of interest. RESULTS: Our results show a significant association between pretransplant blood product exposures and HLA antibody status. Children with a pretransplant blood product exposure had 7.98 times the odds of developing an anti-HLA antibody compared to those without a pretransplant blood product exposure (p = .01). We also found a significant association between a previous red blood cell (RBC) exposure and HLA antibody status (p = .01) which was not found for other blood component exposures. Patients who were HLA antibody positive were more likely to develop a donor-specific antibody (DSA) after transplantation (p = .04). CONCLUSIONS: Exposure to previous allogeneic blood products affects the development of anti-HLA antibodies in children presenting for heart transplantation. Previous RBC exposures resulted in HLA antibody positivity more than other blood component exposures. Importantly, the presence of HLA antibodies was associated with the development of DSAs post-transplantation. Developing transfusion strategies to reduce allogeneic blood product exposures in children who may need future cardiac transplantation should be a high priority.