Early Developmental Outcome after the Norwood Procedure for Hypoplastic Left Heart Syndrome

1999 ◽  
Vol 38 (8) ◽  
pp. 499-500
Author(s):  
Douglas S. Moodie
PEDIATRICS ◽  
1998 ◽  
Vol 102 (5) ◽  
pp. 1148-1152 ◽  
Author(s):  
Jeffrey H. Kern ◽  
Veronica J. Hinton ◽  
Nancy E. Nereo ◽  
Constance J. Hayes ◽  
Welton M. Gersony

Circulation ◽  
2012 ◽  
Vol 125 (17) ◽  
pp. 2081-2091 ◽  
Author(s):  
Jane W. Newburger ◽  
Lynn A. Sleeper ◽  
David C. Bellinger ◽  
Caren S. Goldberg ◽  
Sarah Tabbutt ◽  
...  

PEDIATRICS ◽  
1990 ◽  
Vol 85 (6) ◽  
pp. 977-983
Author(s):  
Cynthia D. Morris ◽  
Jacquelyn Outcalt ◽  
Victor D. Menashe

Advances in surgical treatment of hypoplastic left heart syndrome with the Norwood procedure and cardiac transplantation have made essential the understanding of the natural history of hypoplastic left heart syndrome. In a geographically defined population, we ascertained the prevalence of hypoplastic left heart syndrome in children born in Oregon from 1971 through 1986. Clinical and anatomic data were extracted from the charts of the 98 affected children and the survival rate was calculated. Hypoplastic left heart syndrome occurred in 0.162 per 1000 live births in Oregon during this period. No syndrome complex was prevalent and 84% were free of other congenital malformations. However, there was an increased occurrence of congenital heart defects in first-degree relatives of probands with hypoplastic left heart syndrome. Of the affected children 15 ± 4% died on the first day of life, 70 ± 5% died within the first week, and 91 ± 3% died within 30 days. No secular change in survival occurred during the study. Palliation with the Norwood procedure was performed in 20 children. Although survival was significantly improved with this surgery (P = .01), the effect was observed principally through 30 days of life and only one of these children remains alive. Hypoplastic left heart syndrome is a lethal congenital heart defect in children and poses management and ethical dilemmas.


2002 ◽  
Vol 30 (Supplement) ◽  
pp. A73 ◽  
Author(s):  
Howard E Jeffries ◽  
Randall C Wetzel ◽  
Vaughn A Starnes ◽  
Mary McCulley ◽  
David Y Moromisato

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
N S Borrelli ◽  
G Di Salvo ◽  
J Sabatino ◽  
A Ibrahim ◽  
M Avesani ◽  
...  

Abstract Funding Acknowledgements EACVI Training Grant Introduction Children with Hypoplastic Left Heart Syndrome (HLHS) have a high mortality (up to 95%) and morbidity. Systemic right ventricle (RV) dysfunction plays a key-role in their outcome. Purpose The aim of this study is to evaluate the accuracy of speckle-tracking echocardiographic (STE) assessment of RV deformation and 2D standard echo parameters in predicting death and need for heart transplantation (HT) in HLHS patients. Methods 31 patients with diagnosis of HLHS successfully completed Norwood (n = 29) or comprehensive Norwood stage II (n = 2) at our Institution between 2015 and 2019. Survival at 6 months was 90.32%, survival at 18 months was 85.72%. We studied 29 HLHS patients (17 male). Patients with HLHS variant (n = 2) were excluded. All the studied patients underwent in-hospital interstage stay. Serial echocardiographic assessment was performed in all the included patients (baseline, one month after Norwood, three months after Norwood, one week before bidirectional cavopulmonary anastomosis [BCPA] and two months after BCPA). From the apical view we measured: tricuspid annulus peak systolic excursion (TAPSE), fractional area change (FAC), longitudinal strain (LS) and strain rate (LSR). Results After a mean follow-up of 1.83 ± 1.16 years, 8 out of 29 of the included patients met the composite endpoint of death/HT. At pre-Norwood assessment, there was no statistical difference in echo measurements between survivors and patients who reached the endpoint. In death/HT group TAPSE and LS declined already at one-month after Norwood procedure evaluation. At one-month evaluation, a TAPSE ≤ 5 mm had a good sensitivity for death/HT (85.71%) and a moderate specificity (66.67%), with an area under the curve of 0.789. Always at one-month evaluation, a Δ LS ([{baseline LS – one-month post Norwood LS}/ baseline LS] *100) of 8.7% showed a 100% sensitivity and good specificity (80.95%) for death/HT, with an area under the curve of 0.888. Multivariate analysis showed that one-month-after-Norwood Δ LS was the best predictor of worse outcome (p = 0.02). Conclusions HLHS patients with Δ LS of > 8.7% at one-month after Norwood procedure had a high likelihood of death or HT. These data encourage the routine use of LS to monitor cardiac function in HLHS patients. Abstract 1233 Figure. HLHS LS and trend of TAPSE and LS.


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