Outcomes of surgical management of Ebstein anomaly and tricuspid valve dysplasia in critically ill neonates and infantsCentral MessagePerspective

Objective: To describe the surgical outcomes in neonates and infants who had surgery for Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD). Methods: Retrospective chart review for all patients who underwent surgery for EA or TVD during the index hospitalization after birth at our institution...

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Main Authors: V. Reed LaSala, MD, Edward Buratto, MD, PhD, Halil Beqaj, MD, Isabel Aguirre, Julian Maldonado, Nimrod Goldshtrom, MD, Andrew Goldstone, MD, PhD, Matan Setton, MD, Ganga Krishnamurthy, MD, Emile Bacha, MD, David M. Kalfa, MD, PhD
Format: Article
Language:English
Published: Elsevier 2023-12-01
Series:JTCVS Open
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2666273623002206
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author V. Reed LaSala, MD
Edward Buratto, MD, PhD
Halil Beqaj, MD
Isabel Aguirre
Julian Maldonado
Nimrod Goldshtrom, MD
Andrew Goldstone, MD, PhD
Matan Setton, MD
Ganga Krishnamurthy, MD
Emile Bacha, MD
David M. Kalfa, MD, PhD
author_facet V. Reed LaSala, MD
Edward Buratto, MD, PhD
Halil Beqaj, MD
Isabel Aguirre
Julian Maldonado
Nimrod Goldshtrom, MD
Andrew Goldstone, MD, PhD
Matan Setton, MD
Ganga Krishnamurthy, MD
Emile Bacha, MD
David M. Kalfa, MD, PhD
author_sort V. Reed LaSala, MD
collection DOAJ
description Objective: To describe the surgical outcomes in neonates and infants who had surgery for Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD). Methods: Retrospective chart review for all patients who underwent surgery for EA or TVD during the index hospitalization after birth at our institution from January 2005 to February 2023. Results: Fifteen symptomatic neonates and infants who had surgery for EA or TVD were included, 8 with EA and 7 with TVD. Eleven patients (73%) and 3 patients (20%) required preoperative inotropes and extracorporeal membrane oxygenation, respectively. Nine patients (60%) had a Starnes procedure and 6 patients (40%) had tricuspid valve repair (TVr). Mortality at last follow-up was 27% overall (n = 4/15), 22% after Starnes (n = 2/9) and 33% after TVr (n = 2/6), without a significant difference despite a greater-risk profile in the Starnes group. Postoperative day 1 lactate level was associated with mortality on Cox regression (hazard ratio, 1.45; P = .01). Three of 9 patients who had a Starnes procedure were or will be converted to a cone repair (1.5/2-ventricle repair). Conclusions: Mortality after surgery for EA or TVD during the index hospitalization after birth is still significant in the current era and is associated with a greater lactate level at postoperative day 1. The Starnes procedure and TVr had comparable outcomes despite a greater-risk profile in the Starnes group. An initial single-ventricle approach does not preclude conversion to biventricular or 1.5-ventricle repair.
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spelling doaj.art-cbc02df1ae40452ca824d4672817a9772023-12-20T07:38:06ZengElsevierJTCVS Open2666-27362023-12-0116629638Outcomes of surgical management of Ebstein anomaly and tricuspid valve dysplasia in critically ill neonates and infantsCentral MessagePerspectiveV. Reed LaSala, MD0Edward Buratto, MD, PhD1Halil Beqaj, MD2Isabel Aguirre3Julian Maldonado4Nimrod Goldshtrom, MD5Andrew Goldstone, MD, PhD6Matan Setton, MD7Ganga Krishnamurthy, MD8Emile Bacha, MD9David M. Kalfa, MD, PhD10Section of Pediatric and Congenital Cardiac Surgery, Division of Cardiac, Thoracic, and Vascular Surgery, New York-Presbyterian Morgan Stanley Children’s Hospital, Columbia University Medical Center, New York, NYSection of Pediatric and Congenital Cardiac Surgery, Division of Cardiac, Thoracic, and Vascular Surgery, New York-Presbyterian Morgan Stanley Children’s Hospital, Columbia University Medical Center, New York, NYCollege of Physicians and Surgeons, Columbia University, New York, NYCollege of Physicians and Surgeons, Columbia University, New York, NYCollege of Physicians and Surgeons, Columbia University, New York, NYDivision of Neonatology, Department of Pediatrics, New York-Presbyterian Morgan Stanley Children’s Hospital, Columbia University Medical Center, New York, NYSection of Pediatric and Congenital Cardiac Surgery, Division of Cardiac, Thoracic, and Vascular Surgery, New York-Presbyterian Morgan Stanley Children’s Hospital, Columbia University Medical Center, New York, NYDivision of Cardiology, Department of Pediatrics, New York-Presbyterian Morgan Stanley Children’s Hospital, Columbia University Medical Center, New York, NYDivision of Neonatology, Department of Pediatrics, New York-Presbyterian Morgan Stanley Children’s Hospital, Columbia University Medical Center, New York, NYSection of Pediatric and Congenital Cardiac Surgery, Division of Cardiac, Thoracic, and Vascular Surgery, New York-Presbyterian Morgan Stanley Children’s Hospital, Columbia University Medical Center, New York, NYSection of Pediatric and Congenital Cardiac Surgery, Division of Cardiac, Thoracic, and Vascular Surgery, New York-Presbyterian Morgan Stanley Children’s Hospital, Columbia University Medical Center, New York, NY; Address for reprints: David M. Kalfa, MD, PhD, Section of Pediatric and Congenital Cardiac Surgery, Division of Cardiac, Thoracic, and Vascular Surgery, Department of Surgery, New York-Presbyterian Morgan Stanley Children’s Hospital, 3959 Broadway, Babies North, Suite 274, New York, NY 10032.Objective: To describe the surgical outcomes in neonates and infants who had surgery for Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD). Methods: Retrospective chart review for all patients who underwent surgery for EA or TVD during the index hospitalization after birth at our institution from January 2005 to February 2023. Results: Fifteen symptomatic neonates and infants who had surgery for EA or TVD were included, 8 with EA and 7 with TVD. Eleven patients (73%) and 3 patients (20%) required preoperative inotropes and extracorporeal membrane oxygenation, respectively. Nine patients (60%) had a Starnes procedure and 6 patients (40%) had tricuspid valve repair (TVr). Mortality at last follow-up was 27% overall (n = 4/15), 22% after Starnes (n = 2/9) and 33% after TVr (n = 2/6), without a significant difference despite a greater-risk profile in the Starnes group. Postoperative day 1 lactate level was associated with mortality on Cox regression (hazard ratio, 1.45; P = .01). Three of 9 patients who had a Starnes procedure were or will be converted to a cone repair (1.5/2-ventricle repair). Conclusions: Mortality after surgery for EA or TVD during the index hospitalization after birth is still significant in the current era and is associated with a greater lactate level at postoperative day 1. The Starnes procedure and TVr had comparable outcomes despite a greater-risk profile in the Starnes group. An initial single-ventricle approach does not preclude conversion to biventricular or 1.5-ventricle repair.http://www.sciencedirect.com/science/article/pii/S2666273623002206congenital heart diseaseEbstein anomalytricuspid valve dysplasiatricuspid valvesingle ventricleStarnes
spellingShingle V. Reed LaSala, MD
Edward Buratto, MD, PhD
Halil Beqaj, MD
Isabel Aguirre
Julian Maldonado
Nimrod Goldshtrom, MD
Andrew Goldstone, MD, PhD
Matan Setton, MD
Ganga Krishnamurthy, MD
Emile Bacha, MD
David M. Kalfa, MD, PhD
Outcomes of surgical management of Ebstein anomaly and tricuspid valve dysplasia in critically ill neonates and infantsCentral MessagePerspective
JTCVS Open
congenital heart disease
Ebstein anomaly
tricuspid valve dysplasia
tricuspid valve
single ventricle
Starnes
title Outcomes of surgical management of Ebstein anomaly and tricuspid valve dysplasia in critically ill neonates and infantsCentral MessagePerspective
title_full Outcomes of surgical management of Ebstein anomaly and tricuspid valve dysplasia in critically ill neonates and infantsCentral MessagePerspective
title_fullStr Outcomes of surgical management of Ebstein anomaly and tricuspid valve dysplasia in critically ill neonates and infantsCentral MessagePerspective
title_full_unstemmed Outcomes of surgical management of Ebstein anomaly and tricuspid valve dysplasia in critically ill neonates and infantsCentral MessagePerspective
title_short Outcomes of surgical management of Ebstein anomaly and tricuspid valve dysplasia in critically ill neonates and infantsCentral MessagePerspective
title_sort outcomes of surgical management of ebstein anomaly and tricuspid valve dysplasia in critically ill neonates and infantscentral messageperspective
topic congenital heart disease
Ebstein anomaly
tricuspid valve dysplasia
tricuspid valve
single ventricle
Starnes
url http://www.sciencedirect.com/science/article/pii/S2666273623002206
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