Christopher J Knott-Craig1, Thittamaranahalli Kariyappa S Kumar1, Alejandro R Arevalo, Vijaya M Joshi2. 1. 1Division of Pediatric Cardiovascular Surgery,Le Bonheur Children's Hospital,University of Tennessee Health Science Center,Memphis,Tennessee,United States of America. 2. 2Divison of Pediatric Cardiology,Le Bonheur Children's Hospital,University of Tennessee Health Science Center,Memphis,Tennessee,United States of America.
Abstract
OBJECTIVE: Symptomatic neonates with Ebstein's anomaly pose significant challenge. Within this cohort, neonates with associated anatomical pulmonary atresia have higher mortality. We review our experience with this difficult subset. METHODS: A total of 32 consecutive symptomatic neonates with Ebstein's anomaly underwent surgical intervention between 1994 and 2013. Of them, 20 neonates (62%, 20/32) had associated pulmonary atresia. Patients' weights ranged from 1.9 to 3.4 kg. All patients without pulmonary atresia had two-ventricle repair. Of the 20 neonates, 16 (80%, 16/20) with Ebstein's anomaly and pulmonary atresia had two-ventricle repair and 4 had single-ventricle palliation, of which 2 underwent Starnes' palliation and 2 Blalock-Taussig shunts. Six recent patients with Ebstein's anomaly and pulmonary atresia had right ventricle to pulmonary artery valved conduit as part of their two-ventricle repair. RESULTS: Overall early mortality was 28% (9/32). For those without pulmonary atresia, mortality was 8.3% (1/12). For the entire cohort of neonates with Ebstein's anomaly and pulmonary atresia, mortality was 40% (8/20; p=0.05). Mortality for neonates with Ebstein's anomaly and pulmonary atresia having two-ventricle repair was 44% (7/16). Mortality for neonates with Ebstein's anomaly and pulmonary atresia having two-ventricle repair utilising right ventricle to pulmonary artery conduit was 16% (1/6). For those having one-ventricle repair, the mortality was 25% (1/4). CONCLUSIONS: Surgical management of neonates with Ebstein's anomaly remains challenging. For neonates with Ebstein's anomaly and anatomical pulmonary atresia, single-ventricle palliation is associated with lower early mortality compared with two-ventricle repair. This outcome advantage is negated by inclusion of right ventricle to pulmonary artery conduit as part of the two-ventricle repair.
OBJECTIVE: Symptomatic neonates with Ebstein's anomaly pose significant challenge. Within this cohort, neonates with associated anatomical pulmonary atresia have higher mortality. We review our experience with this difficult subset. METHODS: A total of 32 consecutive symptomatic neonates with Ebstein's anomaly underwent surgical intervention between 1994 and 2013. Of them, 20 neonates (62%, 20/32) had associated pulmonary atresia. Patients' weights ranged from 1.9 to 3.4 kg. All patients without pulmonary atresia had two-ventricle repair. Of the 20 neonates, 16 (80%, 16/20) with Ebstein's anomaly and pulmonary atresia had two-ventricle repair and 4 had single-ventricle palliation, of which 2 underwent Starnes' palliation and 2 Blalock-Taussig shunts. Six recent patients with Ebstein's anomaly and pulmonary atresia had right ventricle to pulmonary artery valved conduit as part of their two-ventricle repair. RESULTS: Overall early mortality was 28% (9/32). For those without pulmonary atresia, mortality was 8.3% (1/12). For the entire cohort of neonates with Ebstein's anomaly and pulmonary atresia, mortality was 40% (8/20; p=0.05). Mortality for neonates with Ebstein's anomaly and pulmonary atresia having two-ventricle repair was 44% (7/16). Mortality for neonates with Ebstein's anomaly and pulmonary atresia having two-ventricle repair utilising right ventricle to pulmonary artery conduit was 16% (1/6). For those having one-ventricle repair, the mortality was 25% (1/4). CONCLUSIONS: Surgical management of neonates with Ebstein's anomaly remains challenging. For neonates with Ebstein's anomaly and anatomical pulmonary atresia, single-ventricle palliation is associated with lower early mortality compared with two-ventricle repair. This outcome advantage is negated by inclusion of right ventricle to pulmonary artery conduit as part of the two-ventricle repair.
Entities:
Keywords:
neonate; pulmonary valve; right ventricle; surgical techniques; tricuspid valve
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