Repair of the tricuspid valve in hypoplastic left heart syndrome

2006 ◽  
Vol 16 (S3) ◽  
pp. 21-26 ◽  
Author(s):  
Richard G. Ohye ◽  
Carlen A. Gomez ◽  
Caren S. Goldberg ◽  
Holly L. Graves ◽  
Eric J. Devaney ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Hypoplastic Left Heart Syndrome ◽  
Volume Overload ◽  
Ventricular Volume ◽  
Norwood Procedure ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Arterial Insufficiency ◽  
Three Stages ◽  
Left Heart Syndrome

The outlook for patients with hypoplastic left heart syndrome has dramatically improved over the past two decades. Universally fatal only 25 years ago, since that time outcomes for staged palliation have shown consistent improvement. Recent reports show that eight to nine patients from every ten can now leave the hospital after the Norwood procedure.1Attrition following the Norwood procedure, nonetheless, remains significant, with from five to fifteen percent of patients dying between the first and second stages of the Norwood sequence.1–4Only three-quarters of the patients undergoing surgery for hypoplastic left heart syndrome survive after five years, even at the centres reporting the best outcomes for the Norwood procedure.1,5In addition to the deaths, some patients are unable to progress through the three stages of reconstruction, and may require cardiac transplantation, or have no options for further therapy. There are many causes for these mortalities and morbidities following the Norwood procedure, including elevated pulmonary vascular resistance, cardiac arrhythmias, coronary arterial insufficiency, right ventricular failure, right ventricular volume overload due to shunt-dependent physiology, and tricuspid valvar regurgitation. Many of these factors are interrelated, and may form feedback loops, which serve to propagate their adverse effects on patients with hypoplastic left heart syndrome.

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

Pre- and Postoperative Quantitation of Right Ventricular Tissue Doppler Velocities in Infants with Hypoplastic Left Heart Syndrome

2006 ◽  
Vol 23 (4) ◽  
pp. 303-307 ◽  
Author(s):  
Douglas Christensen ◽  
Brian Cardis ◽  
William Mahle ◽  
Reginald Lewis ◽  
Jeryl Huckaby ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Hypoplastic Left Heart Syndrome ◽  
Tissue Doppler ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Ventricular Tissue ◽  
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 > 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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