Kimberly A Holst1, Joseph A Dearani2, Sameh M Said1, Ryan R Davies3, Christian Pizarro4, Christopher Knott-Craig5, T K Susheel Kumar5, Vaughn A Starnes6, S Ram Kumar7, Sara K Pasquali8, Dylan P Thibault9, James M Meza10, Kevin D Hill9, Karen Chiswell9, Jeffrey P Jacobs11, Marshall L Jacobs12. 1. Department of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota. 2. Department of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota. Electronic address: jdearani@mayo.edu. 3. Department of Cardiovascular and Thoracic Surgery, UT Southwestern Medical Center and Children's Health, Dallas, Texas. 4. Department of Pediatric Cardiothoracic Surgery, Nemours Children's Health System, Wilmington, Delaware. 5. Department of Pediatric Cardiothoracic Surgery, Le Bonheur Children's Hospital and University of Tennessee Health Science Center, Memphis, Tennessee. 6. Department of Surgery, Keck School of Medicine, University of Southern California, Los Angeles, California. 7. Department of Surgery, Keck School of Medicine, University of Southern California, Los Angeles, California; Heart Institute, Children's Hospital Los Angeles, Los Angeles, California. 8. Department of Pediatrics, University of Michigan, Ann Arbor, Michigan. 9. Duke Clinical Research Institute, Duke University School of Medicine, Durham, North Carolina. 10. Duke Clinical Research Institute, Duke University School of Medicine, Durham, North Carolina; Division of Cardiovascular and Thoracic Surgery, Department of Surgery, Duke University Medical Center, Durham, North Carolina. 11. Division of Cardiovascular Surgery, Johns Hopkins All Children's Hospital, St. Petersburg, Florida. 12. Division of Cardiac Surgery, Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.
Abstract
BACKGROUND: Ebstein anomaly (EA) encompasses a broad spectrum of morphology and clinical presentation. Those who are symptomatic early in infancy are generally at highest risk, but there are limited data regarding multicentric practice patterns and outcomes. We analyzed multiinstitutional data concerning operations and outcomes in neonates and infants with EA. METHODS: Index operations reported in The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010 to 2016) were potentially eligible for inclusion. Analysis was limited to patients with diagnosis of EA and less than 1 year of age at time of surgery (neonates ≤30 days, infants 31 to 365 days). RESULTS: The study population included 255 neonates and 239 infants (at 95 centers). Among neonates, median age at operation was 7 days (interquartile range, 4 to 13 days) and the majority required preoperative ventilation (61.6%, n = 157). The most common primary operation performed among neonates was Ebstein repair (39.6%, n = 101), followed by systemic-to-pulmonary shunt (20.4%, n = 52) and tricuspid valve closure (9.4%, n = 24). Overall neonatal operative mortality was 27.4% (n = 70), with composite morbidity-mortality of 51.4% (n = 48). For infants, median age at operation was 179 days (interquartile range, 108-234 days); the most common primary operation for infants was superior cavopulmonary anastomosis (38.1%, n = 91) followed by Ebstein repair (15.5%, n = 37). Overall operative mortality for infants was 9.2% (n = 22) with composite morbidity-mortality of 20.1% (48). CONCLUSIONS: Symptomatic EA in early infancy is very high risk and a variety of operative procedures were performed. A dedicated prospective study is required to more fully understand optimal selection of treatment pathways to guide a systematic approach to operative management.
BACKGROUND:Ebstein anomaly (EA) encompasses a broad spectrum of morphology and clinical presentation. Those who are symptomatic early in infancy are generally at highest risk, but there are limited data regarding multicentric practice patterns and outcomes. We analyzed multiinstitutional data concerning operations and outcomes in neonates and infants with EA. METHODS: Index operations reported in The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010 to 2016) were potentially eligible for inclusion. Analysis was limited to patients with diagnosis of EA and less than 1 year of age at time of surgery (neonates ≤30 days, infants 31 to 365 days). RESULTS: The study population included 255 neonates and 239 infants (at 95 centers). Among neonates, median age at operation was 7 days (interquartile range, 4 to 13 days) and the majority required preoperative ventilation (61.6%, n = 157). The most common primary operation performed among neonates was Ebstein repair (39.6%, n = 101), followed by systemic-to-pulmonary shunt (20.4%, n = 52) and tricuspid valve closure (9.4%, n = 24). Overall neonatal operative mortality was 27.4% (n = 70), with composite morbidity-mortality of 51.4% (n = 48). For infants, median age at operation was 179 days (interquartile range, 108-234 days); the most common primary operation for infants was superior cavopulmonary anastomosis (38.1%, n = 91) followed by Ebstein repair (15.5%, n = 37). Overall operative mortality for infants was 9.2% (n = 22) with composite morbidity-mortality of 20.1% (48). CONCLUSIONS: Symptomatic EA in early infancy is very high risk and a variety of operative procedures were performed. A dedicated prospective study is required to more fully understand optimal selection of treatment pathways to guide a systematic approach to operative management.
Authors: Marshall L Jacobs; Jeffrey P Jacobs; Kevin D Hill; Sean M O'Brien; Sara K Pasquali; David Vener; S Ram Kumar; Karen Chiswell; James D St Louis; John E Mayer; Robert H Habib; David M Shahian; Felix G Fernandez Journal: Ann Thorac Surg Date: 2019-07-20 Impact factor: 4.330
Authors: Lindsay R Freud; Doff B McElhinney; Brian T Kalish; Maria C Escobar-Diaz; Rukmini Komarlu; Michael D Puchalski; Edgar T Jaeggi; Anita L Szwast; Grace Freire; Stéphanie M Levasseur; Ann Kavanaugh-McHugh; Erik C Michelfelder; Anita J Moon-Grady; Mary T Donofrio; Lisa W Howley; Elif Seda Selamet Tierney; Bettina F Cuneo; Shaine A Morris; Jay D Pruetz; Mary E van der Velde; John P Kovalchin; Catherine M Ikemba; Margaret M Vernon; Cyrus Samai; Gary M Satou; Nina L Gotteiner; Colin K Phoon; Norman H Silverman; Wayne Tworetzky Journal: J Am Heart Assoc Date: 2020-10-20 Impact factor: 5.501
Authors: Rodrigo Freire Bezerra; Juliana Torres Pacheco; Sônia Meiken Franchi; Rosangela Belbuche Fittaroni; José Francisco Baumgratz; Rodrigo Moreira Castro; Luciana da Fonseca da Silva; José Pedro da Silva Journal: Arq Bras Cardiol Date: 2022-08 Impact factor: 2.667