Abstract 2597: Aortic Atresia Is Associated with an Inferior Postoperative Systemic, Cerebral and Splanchnic Oxygen Transport Status in Neonates with Hypoplastic Left Heart Syndrome after the Norwood Procedure

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Gencheng Zhang ◽  
Glen Van Arsdell ◽  
Helen Holtby ◽  
Sally Cai ◽  
Osman Al Radi ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Oxygen Transport ◽  
Norwood Procedure ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Arterial Blood ◽  
Aortic Atresia ◽  
Inferior Status ◽  
Splanchnic Oxygen ◽  
Left Heart Syndrome

Aortic atresia (AA) has been identified as a risk factor for mortality after the Norwood procedure. We compared postoperative systemic and regional (cerebral and splanchnic) oxygen transport profiles in neonates with hypoplastic left heart syndrome with or without AA after the Norwood procedure. Systemic oxygen consumption (VO 2 ) was measured using respiratory mass spectrometry postoperatively for 72 hrs in 17 neonates (n=9 in AA group and 8 in non-AA group). Arterial, superior vena cava, and pulmonary venous blood gases and pressures were measured at 2– 4 hr intervals to calculate cardiac output (CO), systemic vascular resistance (SVR), oxygen delivery (DO 2 ) and oxygen extraction ratio (ERO 2 ). Cerebral (ScO 2 ) and splanchnic (SsO 2 ) oxygen saturations were measured using near infrared spectroscopy. The doses of dopamine, vasopressin (up to 0.0008 unit/kg/min), phenoxybenzamine and milrinone were noted. When compared to non-AA group, AA group had lower CO (p=0.03), higher systolic arterial blood pressure (p=0.01) and SVR (p=0.002), lower DO 2 (p=0.07), VO 2 (p=0.003) and ScO 2 during the first 30 to 40 hrs. There was no significant difference in SsO 2 (p=0.12, n=7 in AA group and 3 in non-AA group). Despite a similar ERO 2 (p=0.5), AA group had higher lactate over 72 hrs (p=0.01). AA group received higher doses of vasopressin (p=0.005) and milrinone (p<0.0001), lower doses of dopamine (p=0.07), with similar use of phenoxybenzamine. Furthermore, VO 2 negatively correlated with vasopressin (p=0.05), positively correlated with milrinone (p=0.03) and dopamine (p<0.0001). When including the drugs, the inferior status in all the oxygen transport variables remained, and additionally adversely correlated with vasopressin (p from <0.0001 to 0.06), except for ScO 2 that positively correlated with dopamine, but not with other drugs. Aortic atresia is associated with an inferior status of systemic, cerebral and splanchnic oxygen transport in neonates after the Norwood procedure. Aggressive use of vasopressin worsens systemic and splanchnic oxygen transport status. Postoperative management strategies should be directed to improve both systemic and regional oxygen transport in this special group of patients.

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

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
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Norwood Procedure ◽  
Developmental Outcome ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome ◽  
Early Developmental

Hypoplastic Left Heart Syndrome: Natural History in a Geographically Defined Population

PEDIATRICS ◽  
1990 ◽  
Vol 85 (6) ◽  
pp. 977-983
Author(s):  
Cynthia D. Morris ◽  
Jacquelyn Outcalt ◽  
Victor D. Menashe
Keyword(s):  
Natural History ◽  
Hypoplastic Left Heart Syndrome ◽  
Congenital Heart ◽  
Heart Defects ◽  
Norwood Procedure ◽  
Secular Change ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
History Of ◽  
Left Heart Syndrome

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.

GASTROINTESTINAL MORBIDITY IN PATIENTS WITH HYPOPLASTIC LEFT HEART SYNDROME FOLLOWING THE NORWOOD PROCEDURE

2002 ◽  
Vol 30 (Supplement) ◽  
pp. A73 ◽  
Author(s):  
Howard E Jeffries ◽  
Randall C Wetzel ◽  
Vaughn A Starnes ◽  
Mary McCulley ◽  
David Y Moromisato
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Norwood Procedure ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Left Heart Syndrome

scholarly journals 1233 Can speckle tracking echocardiography predict outcome in hypoplastic left heart syndrome patients?

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
N S Borrelli ◽  
G Di Salvo ◽  
J Sabatino ◽  
A Ibrahim ◽  
M Avesani ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Speckle Tracking ◽  
Area Under The Curve ◽  
Composite Endpoint ◽  
Norwood Procedure ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Systemic Right Ventricle ◽  
Cavopulmonary Anastomosis ◽  
Left Heart Syndrome

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 &gt; 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.

scholarly journals Geometry of the pulmonary arteries before the Fontan operation: can we influence it during the Norwood procedure?

2020 ◽  
Vol 57 (6) ◽  
pp. 1098-1104
Author(s):  
Katarzyna Januszewska ◽  
Pawel Nawrocki ◽  
Anja Lehner ◽  
Julia Stegger ◽  
Felix Kleinerueschkamp ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Fontan Operation ◽  
Pulmonary Arteries ◽  
Norwood Procedure ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Second Stage ◽  
The Right ◽  
Left Heart Syndrome ◽  
The Impact

Abstract OBJECTIVES The right ventricle-to-pulmonary artery (RV-PA) shunt provides stable haemodynamics after the Norwood procedure but can influence development of the central pulmonary arteries (PAs). The goal of this study was to analyse the geometry of the central PAs in children with hypoplastic left heart syndrome before the Fontan operation with respect to the RV-PA shunt site and the type of the second-stage operation. METHODS A total of 161 children with hypoplastic left heart syndrome, median age 2.7 (range 1.3–9.8) years and median weight 12.7 (range 7.6–26.1) kg, underwent the Fontan operation after having had the Norwood procedure with an RV-PA shunt. The patients were divided into 2 groups: left-sided RV-PA (L-RV-PA) (n = 129) with the shunt on the left and right-sided RV-PA (n = 32) with the shunt on the right side of the neoaorta. Angiographic data obtained before the Fontan and all cardiac catheterization interventions were analysed retrospectively. RESULTS Between the second and third stages, as well as directly before the Fontan operation, the L-RV-PA group required more PA catheter interventions (P = 0.001 and P = 0.03). In this group, the minimal left PA diameter was smaller than that in the R-RV-PA group (P = 0.021). Leaving the shunt open until the Fontan operation increased the rate of PA interventions in the L-RV-PA group (P = 0.001), but there is no evidence of the impact on the development of the left PAs (P = 0.075). There is also no evidence that the type of the second-stage procedure influences the intervention rate before the Fontan procedure (P = 0.14). CONCLUSIONS Children who have the L-RV-PA shunt require more PA catheter interventions. The right-sided RV-PA shunt and the subsequent Glenn anastomosis in the place of the shunt are associated with distortion-free and more symmetrical development of the central PAs.

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