Shu-Chien Huang1, En-Ting Wu2, Shyh-Jye Chen3, Chi-Hsiang Huang4, Jin-Chung Shih5, Hen-Wen Chou6, Chung-I Chang6, Ing-Sh Chiu6, Yih-Sharng Chen6. 1. Department of Surgery, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan. Electronic address: cvshuang@gmail.com. 2. Department of Pediatrics, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan. 3. Department of Medical Imaging, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan. 4. Department of Anesthesiology, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan. 5. Department of Obstetrics and Gynecology, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan. 6. Department of Surgery, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan.
Abstract
BACKGROUND: Neonates with severe forms of Ebstein anomaly present a surgical challenge, and the Starnes operation as single ventricle palliation is highly advocated. Cone reconstruction for tricuspid valvuloplasty (TVP) has become a widely accepted technique, although very few cases of TVP have been reported in neonates. This report describes a surgical strategy for neonatal Ebstein anomaly, with an aim toward biventricular repair. METHODS: Since 2007, 7 neonates or young infants with severe Ebstein anomalies have received TVP at the National Taiwan University Hospital, Taipei, Taiwan. The principle of cone reconstruction was applied with mobilization of all three leaflets and reattachment to the normal tricuspid annulus. The atrialized right ventricle was not plicated. In patients with pulmonary stenosis, the interatrial communication was not totally closed (n = 5), and a systemic-pulmonary shunt was added if needed (n = 3). RESULTS: All patients presented with intractable heart failure or severe cyanosis requiring mechanical ventilation, or both. All patients had marked adherence of the anterior leaflet to the right ventricular free wall. Intracardiac anomalies including ventricular septal defect (n = 2) and tetralogy of Fallot (n = 1) were also repaired simultaneously. Six of the 7 patients (86%) survived. There were no late deaths or repeat TVPs for a median follow-up of 4.3 years (range, 0.8 to 9.9 years). CONCLUSIONS: Reconstruction of the tricuspid valve is an acceptable surgical strategy in patients with severe neonatal Ebstein anomaly. Fenestrated atrial septal defect and systemic-pulmonary shunt can help overcome anatomic pulmonary stenosis and high pulmonary resistance in the neonatal period. This surgical strategy has a good survival outcome and preserves the possibility of complete biventricular repair.
BACKGROUND: Neonates with severe forms of Ebstein anomaly present a surgical challenge, and the Starnes operation as single ventricle palliation is highly advocated. Cone reconstruction for tricuspid valvuloplasty (TVP) has become a widely accepted technique, although very few cases of TVP have been reported in neonates. This report describes a surgical strategy for neonatal Ebstein anomaly, with an aim toward biventricular repair. METHODS: Since 2007, 7 neonates or young infants with severe Ebstein anomalies have received TVP at the National Taiwan University Hospital, Taipei, Taiwan. The principle of cone reconstruction was applied with mobilization of all three leaflets and reattachment to the normal tricuspid annulus. The atrialized right ventricle was not plicated. In patients with pulmonary stenosis, the interatrial communication was not totally closed (n = 5), and a systemic-pulmonary shunt was added if needed (n = 3). RESULTS: All patients presented with intractable heart failure or severe cyanosis requiring mechanical ventilation, or both. All patients had marked adherence of the anterior leaflet to the right ventricular free wall. Intracardiac anomalies including ventricular septal defect (n = 2) and tetralogy of Fallot (n = 1) were also repaired simultaneously. Six of the 7 patients (86%) survived. There were no late deaths or repeat TVPs for a median follow-up of 4.3 years (range, 0.8 to 9.9 years). CONCLUSIONS: Reconstruction of the tricuspid valve is an acceptable surgical strategy in patients with severe neonatal Ebstein anomaly. Fenestrated atrial septal defect and systemic-pulmonary shunt can help overcome anatomic pulmonary stenosis and high pulmonary resistance in the neonatal period. This surgical strategy has a good survival outcome and preserves the possibility of complete biventricular repair.
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