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

Autor:	Bleiweis MS; Department of Surgery, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA.; Department of Pediatrics, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Peek GJ; Department of Surgery, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA.; Department of Pediatrics, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Philip J; Department of Pediatrics, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Fudge JC; Department of Pediatrics, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Sullivan KJ; Department of Pediatrics, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA.; Department of Anesthesia, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Co-Vu J; Department of Pediatrics, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., DeGroff C; Department of Pediatrics, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Vyas HV; Department of Pediatrics, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Gupta D; Department of Pediatrics, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Shih R; Department of Pediatrics, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Pietra BBA; Department of Pediatrics, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Fricker FJ; Department of Pediatrics, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Cruz Beltran SC; Department of Anesthesia, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Arnold MA; Department of Anesthesia, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Wesley MC; Department of Anesthesia, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Pitkin AD; Department of Anesthesia, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Hernandez-Rivera JF; Department of Pediatrics, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Lopez-Colon D; Department of Surgery, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA.; Department of Pediatrics, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Barras WE; Department of Surgery, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Stukov Y; Department of Surgery, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Sharaf OM; Department of Surgery, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA.; Department of Pediatrics, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Neal D; Department of Surgery, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Nixon CS; Department of Surgery, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA.; Department of Pediatrics, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA., Jacobs JP; Department of Surgery, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA.; Department of Pediatrics, Congenital Heart Center, 3463University of Florida, Gainesville, FL, USA.
Jazyk:	angličtina
Zdroj:	World journal for pediatric & congenital heart surgery [World J Pediatr Congenit Heart Surg] 2022 Sep; Vol. 13 (5), pp. 664-675. Date of Electronic Publication: 2022 May 05.
DOI:	10.1177/21501351221088030
Abstrakt:	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.
Databáze:	MEDLINE
Externí odkaz:	Zobrazit plný text záznamu