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

Abstract

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.

A Single-Institutional Experience with 36 Children Smaller Than 5 Kilograms Supported with the Berlin Heart Ventricular Assist Device (VAD) over 12 Years: Comparison of Patients with Biventricular versus Functionally Univentricular Circulation

“…In patients with functionally univentricular hearts, we prefer pulsatile VAD rather than continuous-flow VAD [7][8][9][10][11][12][13] because we believe that:…”

“…Because of these potentially long waiting times, it is also reasonable to stabilize these high-risk patients with functionally univentricular hearts with VAD while awaiting transplantation. [7][8][9][10][11][12][13] This approach facilitates early extubation and optimization for transplantation while on VAD in this high-risk population. [7][8][9][10][11][12][13] In very high-risk patients with functionally univentricular circulation, primary transplantation can be considered rather than surgical palliation [7][8][9][10][11][12][13] because rescue transplantation after failed surgical palliation is associated with worse outcomes.…”

“…[7][8][9][10][11][12][13] This approach facilitates early extubation and optimization for transplantation while on VAD in this high-risk population. [7][8][9][10][11][12][13] In very high-risk patients with functionally univentricular circulation, primary transplantation can be considered rather than surgical palliation [7][8][9][10][11][12][13] because rescue transplantation after failed surgical palliation is associated with worse outcomes. [15][16][17][18][19][20] Survival after transplantation for HLHS or HLHS-related malformations is better with primary transplantation in comparison to transplantation after previous cardiac surgery.…”

“…9 During the same era that these nine high-risk patients with HLHS or HLHS-related malformations underwent hybrid + sVAD, 62 standard-risk patients underwent Norwood (Stage 1) at the University of Florida with an operative mortality of 3.2% (2/62). 12 The remaining three patients in this current manuscript with HLHS or HLHS-related malformations underwent VAD insertion after having undergone:…”

“…[1][2][3][4][5][6] Over the past four years, our program has published a series of manuscripts describing our evolving approach to providing support with a "Single Ventricle-Ventricular Assist Device" (sVAD) to patients with functionally univentricular circulation. [7][8][9][10][11][12][13] These seven previous publications describe the evolving details of our techniques for preemptive sVAD utilization in functionally univentricular neonates with unfavorable cardiac anatomy and extreme risk of cardiac compromise before or during staged palliation, who have either ductaldependent pulmonary circulation or ductal-dependent systemic circulation. Neonates with functionally univentricular ductal-dependent pulmonary circulation undergo combined palliation + sVAD insertion, while neonates with functionally univentricular ductal-dependent systemic circulation undergo combined hybrid + sVAD insertion.…”