Cardiac Center in Evolution: Management of Single Ventricle
A Comprehensive Approach to the Management of Patients With HLHS and HLHS-Related Malformations: Analysis of 100 Consecutive Neonates (2015-2023) with 2.6% (2/77) Norwood (Stage 1) Operative Mortality
Saturday, January 27, 2024
10:34 AM – 10:46 AM CT
Location: Stars at Night Ballroom 1
M. S. Bleiweis1, J. Co-Vu1, J. Philip1, J. Fudge1, H. Vyas1, A. Pitkin1, G. Janelle1, K. Sullivan1, C. DeGroff1, D. Gupta1, B. Pietra1, F. Fricker1, S. Cruz Beltran1, J. Hernandez-Rivera1, G. J.. Peek1, L. Wilson1, Y. Stukov1, O. M. Sharaf2, C. Nixon1, J. P. Jacobs1 1University of Florida, Gainesville, Florida 2University of Florida College of Medicine, Gainesville, Florida
Mark S. Bleiweis, M.D. University of Florida Gainesville, Florida, United States
Disclosure(s): No financial relationships to disclose
Purpose: We report our comprehensive approach designed to maximize survival and optimize the utilization of donor hearts in patients with HLHS and HLHS-related malformations with functionally univentricular ductal-dependent systemic circulation, and we describe our outcomes with this approach in 100 consecutive neonates (including 77 who underwent primary Norwood [Stage 1] Operation). Methods: 100 consecutive neonates with HLHS and HLHS-related malformations (2015-2023) were stratified into 3 pathways:
Pathway 1: 77/100=77%% of neonates were classified as standard risk and underwent initial Norwood (Stage 1) palliation.
Pathway 2: 10/100=10% of neonates were classified as high-risk patients with risk factors other than major cardiac risk factors (such as severe necrotizing enterocolitis or Turner Syndrome [45,XO]). Pathway 2 patients 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.
Pathway 3: 13/100=13% of neonates were classified as high-risk patients with major cardiac risk factors (coronary fistulas with ventricular-dependent coronary circulation or severe atrioventricular valvar regurgitation). Ten pathway 3 patients were bridged to transplantation with initial combined Hybrid Stage 1 palliation and pulsatile ventricular assist device (VAD) insertion (HYBRID+VAD). (Three patients early in the series were bridged to transplantation with prostaglandin.) Results: Overall mortality at 1 year=9/100=9% (Figure 1 and Table 1).
Pathway 1: Operative Mortality for standard-risk patients undergoing initial Norwood (Stage 1) Operation was 2/77=2.6%. Of 75 survivors of Norwood (Stage 1): 66 successful underwent Glenn, 2 underwent successful biventricular repair, 1 underwent successful cardiac transplantation, and 6 await Glenn.
Pathway 2: Operative Mortality for high-risk patients with risk factors other than major cardiac risk factors undergoing initial Hybrid Stage 1 palliation without VAD was 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 patients, 70% (7/10) underwent successful cardiac transplantation and are alive today and 30% (3/10) died while awaiting transplantation on VAD. Median length of VAD support was 134 days (mean =136, range=56-226). (Tiw of the 3 patients early in the series who were bridged to transplantation with prostaglandin underwent successful cardiac transplantation and one died while awaiting transplantation. Conclusion: A comprehensive approach to the management of patients with HLHS or HLHS-related malformations with functionally univentricular ductal-dependent systemic circulation is associated with Operative Mortality after Norwood of 2/77=2.6% and an overall one-year mortality of 9/100=91%. A subset of 10/100 patients (10.%) were stabilized with HYBRID +VAD while awaiting transplantation. VAD facilitates survival on the waiting list during prolonged wait times.
Our comprehensive approach offers the optimal operation for each neonate, maximizes survival, and optimizes the utilization of donor hearts.
Identify the source of the funding for this research project: NONE