TY - JOUR
T1 - A Comprehensive Approach to the Management of Patients With HLHS and Related Malformations
T2 - An Analysis of 83 Patients (2015-2021)
AU - Bleiweis, Mark S.
AU - Peek, Giles J.
AU - Philip, Joseph
AU - Fudge, James C.
AU - Sullivan, Kevin J.
AU - Co-Vu, Jennifer
AU - DeGroff, Curt
AU - Vyas, Himesh V.
AU - Gupta, Dipankar
AU - Shih, Renata
AU - Pietra, Biagio “Bill” A.
AU - Fricker, Frederick Jay
AU - Cruz Beltran, Susana C.
AU - Arnold, Michael A.
AU - Wesley, Mark C.
AU - Pitkin, Andrew D.
AU - Hernandez-Rivera, Jose F.
AU - Lopez-Colon, Dalia
AU - Barras, Wendy E.
AU - Stukov, Yuriy
AU - Sharaf, Omar M.
AU - Neal, Dan
AU - Nixon, Connie S.
AU - Jacobs, Jeffrey P.
N1 - Publisher Copyright:
© The Author(s) 2022.
PY - 2022/9
Y1 - 2022/9
N2 - 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.
AB - 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.
KW - hybrid
KW - hypoplastic left heart syndrome (HLHS)
KW - Norwood
KW - ventricular assist device (VAD)
UR - https://www.scopus.com/pages/publications/85134756400
U2 - 10.1177/21501351221088030
DO - 10.1177/21501351221088030
M3 - Article
C2 - 35511494
AN - SCOPUS:85134756400
SN - 2150-1351
VL - 13
SP - 664
EP - 675
JO - World Journal for Pediatric and Congenital Heart Surgery
JF - World Journal for Pediatric and Congenital Heart Surgery
IS - 5
ER -