eComment. Two-stage repair of Ebstein's anormaly in a neonate.
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ABSTRACT: We present a rapid two-stage Starnes procedure for a seriously symptomatic neonate with the prenatal diagnosis of Ebstein anomaly. At 16 hours after birth, we performed an emergency operation consisting of main pulmonary artery ligation, plication of the right atrial and right ventricular wall, modified Blalock-Taussig shunt, and patent ductus arteriosus ligation, without cardiopulmonary bypass. At age 12 days, we then performed the Starnes procedure using a glutaraldehyde-treated autologous pericardial patch with a 4-mm fenestration to close the tricuspid valve orifice. The infant's postoperative course was excellent. A rapid two-stage Starnes procedure is useful for treating a seriously symptomatic neonate with Ebstein anomaly.The Annals of thoracic surgery 12/2010; 90(6):2073-5. · 3.45 Impact Factor
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ABSTRACT: Fetal and neonatal Ebstein's anomaly has a poor prognosis, and there are few contemporary reviews of management and outcomes. This study retrospectively reviewed a management algorithm promoting early ductal closure after anatomic pulmonary obstruction had been excluded or relieved in neonatal Ebstein's anomaly from 1995 to 2004. Twenty-eight patients with Ebstein's anomaly were identified, 9 prenatally (8 with hydrops) and 19 postnatally, at a median age of <24 hours. Celermajer index scores predicted a mortality rate of 35%. Prostaglandins were administered to 24 of 28 patients. Prostaglandins were continued for obstructive lesions in 9 until the relief of pulmonary outflow obstructions or aortic coarctation repair. In 8 of 9 patients, prostaglandins were discontinued after intervention. One patient continued receiving prostaglandins until a Blalock-Taussig shunt was performed 3 weeks after surgical valvotomy. Prostaglandins were discontinued in 17 of 24 patients with uneventful ductal constrictions. The ductus arteriosus persisted in 5 patients with hemodynamic instability, of whom 4 underwent ductal ligation, with immediate improvement. In 1 of 24 patients, unrecognized right ventricular outflow tract obstructions became apparent when prostaglandins were discontinued. Prostaglandins were restarted until a primary superior cavopulmonary anastomosis was performed at 7 weeks. The median follow-up period of 26 survivors was 34 months (range 3 to 106), 4 after bidirectional superior cavopulmonary anastomoses and 1 after a Fontan procedure. In conclusion, in neonatal Ebstein's anomaly of the tricuspid valve, prolonged patency of the ductus arteriosus in patients without anatomic outflow tract obstructions is deleterious. "Circular shunts" may develop in patients with pulmonary and tricuspid insufficiency. The ligation or spontaneous constriction of the ductus arteriosus may result in hemodynamic improvement. This approach to neonatal Ebstein's anomaly of the tricuspid valve has yielded a substantial reduction in mortality to 7% in a high-risk cohort.The American Journal of Cardiology 09/2005; 96(6):851-6. · 3.43 Impact Factor
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ABSTRACT: The symptomatic newborn infant with Ebstein's anomaly is in critical condition and is difficult to treat successfully. Furthermore, supraventricular tachyarrhythmia in patients with Ebstein's anomaly may determine the early and late results. We report a successfully treated case of extracardiac total cavopulmonary connection with a right-sided maze procedure after a modified Starnes' operation during the neonatal period.General Thoracic and Cardiovascular Surgery 06/2008; 56(5):233-5.
neither overdistension nor thrombus in the RV. Blood drainage
from the RV appeared to be adequate.
A rare case of neonatal Ebstein’s anomaly with circular shunting
was reported. A two-stage surgical procedure was performed,
and the outcome was good.
Conflict of interest: none declared.
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eComment. Two-stage repair of Ebstein’s anormaly in a neonate
Author: Leo A. Bockeria
Bakoulev Scientific Center for Cardiovascular Surgery, Moscow, Russia
© The Author 2012. Published by Oxford University Press on behalf of the
European Association for Cardio-Thoracic Surgery. All rights reserved.
Ebstein’s anomaly is a congenital heart disease, which rarely requires surgical
intervention in the neonatal period. Therefore, the case involving surgical treat-
ment for neonatal Ebstein’s anomaly with circular shunting by Yohsuke Yanasa et
al. is very interesting .
The clinical symptoms vary in severity depending on the anatomical malforma-
tion. Severe tricuspid regurgitation and right ventricular hypoplasia may produce a
critical state in the neonate. Concomitant pulmonary regurgitation is rare, with the
reversal of blood flow to the right ventricle, resulting in a ’circular shunt’ and ne-
cessitating urgent surgical treatment for severe neonatal Ebstein’s anomaly.
Between 2006 and 2010, 107 patients (including one neonate and one infant)
underwent surgery for Ebstein’s anomaly at Bakoulev Scientific Center for
Cardiovascular Surgery at the Russian Academy of Medical Sciences. In our prac-
tice, due to the decrease in pulmonary vascular resistance, minimal antegrade
blood flow via pulmonary valve was restored but it was not sufficient to ensure ad-
equate oxygen saturation. We performed a modified Blalock-Taussig shunt with a
4-mm graft. Postoperative oxygen saturation was 80% and the baby was dis-
charged. Six months later, a successful one and a half surgical repair was
Surgery in the neonatal period has previously focused on palliation and conver-
sion to single-ventricle physiology. Successful two-ventricle repair with good clinic-
al results can safely be performed in the neonatal period but requires an individual
approach in each case.
 Yanase Y, Watanabe M, Ishikawa N, Higami T. Surgical treatment for neonatal
Ebstein’s anomaly with circular shunting. Interact CardioVasc Thorac Surg
Y. Yanase et al. / Interactive CardioVascular and Thoracic Surgery