Asset Details
Do you wish to reserve the book?
Mid-Term Outcomes of Patients With Hypoplastic Left Heart Syndrome and Left Ventricle-Coronary Artery Fistula
Hey, we have placed the reservation for you!
By the way, why not check out events that you can attend while you pick your title.
Oops! Something went wrong.
Looks like we were not able to place the reservation. Kindly try again later.
Are you sure you want to remove the book from the shelf?
Mid-Term Outcomes of Patients With Hypoplastic Left Heart Syndrome and Left Ventricle-Coronary Artery Fistula
Do you wish to request the book?
Mid-Term Outcomes of Patients With Hypoplastic Left Heart Syndrome and Left Ventricle-Coronary Artery Fistula
Please be aware that the book you have requested cannot be checked out. If you would like to checkout this book, you can reserve another copy
We have requested the book for you!
Your request is successful and it will be processed during the Library working hours. Please check the status of your request in My Requests.
Oops! Something went wrong.
Looks like we were not able to place your request. Kindly try again later.
Journal Article
Mid-Term Outcomes of Patients With Hypoplastic Left Heart Syndrome and Left Ventricle-Coronary Artery Fistula
2025
Overview
Infants with hypoplastic left heart syndrome (HLHS) with mitral stenosis/aortic atresia (MS/AA) have worse outcomes compared to other anatomic variants; this may be related to left ventricle-coronary artery (LV-CA) fistula. We reviewed patients with HLHS (MS/AA) referred to Boston Children's Hospital and managed from birth during 2008 to 2023 and compared those with and without LV-CA fistula defined angiographically. Among 90 patients, 58 (64%) had LV-CA fistula. In total, 66 (73%) of patients underwent surgical stage 1 palliation (S1P) and 22 (24%) underwent hybrid S1P; hybrid S1P was more common in the fistula group (36% vs 6%, p = 0.002). Probability of transplant-free survival at 1 year was 63% (95% CI 49%, 74%) for those with fistula and 78% (95% CI 60%, 89%) for those without. Over a median follow up of 4.3 years [IQR 0.5,7.9], 38 (42%) patients died or underwent transplant. In univariate analysis, lower GA (HR 1.31, 95% CI 1.16, 1.48), lower BW (HR 1.68, 95% CI 1.28, 2.19), initial hybrid S1P (HR 3.50, 95% CI 1.79, 6.84), and need for perioperative ECMO (HR 4.48, 95% CI 2.23, 8.99) were associated with increased risk of death/transplant (p <0.001 for all). The association of LV-CA fistula with death or transplant did not reach statistical significance (HR 1.83, 95% 0.89, 3.76, p = 0.10). Mortality remains high for children with HLHS (MS/AA) and while there was a trend toward worse transplant-free survival for children with LV-CA fistula compared to those without, factors other than LV-CA fistula may contribute.
Publisher
Elsevier Inc,Elsevier Limited
Subject
/ Aorta
/ Children
/ Coronary Vessel Anomalies - surgery
/ Death
/ Extracorporeal membrane oxygenation
/ Female
/ Fistula
/ Fistulae
/ Heart
/ Heart Ventricles - abnormalities
/ Heart Ventricles - diagnostic imaging
/ Humans
/ hypoplastic left heart syndrome
/ Hypoplastic Left Heart Syndrome - complications
/ Hypoplastic Left Heart Syndrome - mortality
/ Hypoplastic Left Heart Syndrome - surgery
/ Infant
/ Male
/ Patients
/ Stenosis
/ Survival
