Repair of Ebstein's anomaly in neonates and small infants: Impact of right ventricular exclusion and its indications

Shunji Sano, Yasuhiro Fujii, Shingo Kasahara, Yosuke Kuroko, Atsushi Tateishi, Ko Yoshizumi, Sadahiko Arai

Research output: Contribution to journalArticlepeer-review

23 Citations (Scopus)


OBJECTIVES: In cases of severe Ebstein's anomaly, it is essential to determine whether biventricular repair (BVR) or single-ventricle palliation is feasible. Since 1999, in our institution, we have used the novel technique comprising tricuspid valve (TV) closure and right ventricular and right atrial (RV/RA) exclusion to reduce the deleterious effects of an enlarged RV in patients with severe Ebstein's anomaly. However, in cases with good RV function, primary BVR is performed. In the present study, we describe our surgical strategy in the treatment of severely symptomatic neonates with Ebstein's anomaly. METHODS: From June 1999 to October 2011, 12 neonates with a severely symptomatic Ebstein's anomaly underwent surgical repair. The mean age at the first operation was 29 ± 25 (range, 5-92) days; and the mean body weight was 2.8 ± 0.5 (range, 2.0-4.1) kg. The associated anomalies included pulmonary atresia with an intact ventricular septum in 7, critical pulmonary stenosis in 1, ventricular septal defect in 3 and coarctation of the aorta in 1 patient. The mean cardio-thoracic ratio (CTR) was 80 ± 14% (range, 57-98%). Preoperatively, 9 patients had grade IV tricuspid regurgitation (TR), as detected by echocardiography, and 6 required ventilator support. RESULTS: Five patients underwent primary BVR. Seven patients underwent staged palliation using a modified Blalock-Taussig shunt (BT shunt) with/without RV/RA exclusion. There was 1 case each of hospital death and late death. The median follow-up duration in the present study was 6.5 years. Among the 8 patients who underwent TV repair, postoperative TR was trivial or mild in 6 patients, moderate in 1 and absent in 1. After surgery, the mean CTR and serum B-type natriuretic peptide levels decreased to 59 ± 14% (range, 45-70%) and 46 ± 28 (range, 12-83) pg/dl, respectively. CONCLUSIONS: Critically ill neonates with Ebstein's anomaly can be successfully treated using RV/RA exclusion combined with a modified BT shunt in cases where RV function is poor. However, in cases of good RV function, we recommend the use of primary BVR.

Original languageEnglish
Article numberezt465
Pages (from-to)549-555
Number of pages7
JournalEuropean Journal of Cardio-Thoracic Surgery
Issue number3
Publication statusPublished - Mar 2014


  • Cardiac surgery
  • Ebstein's anomaly
  • Neonate
  • Right ventricular exclusion

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine


Dive into the research topics of 'Repair of Ebstein's anomaly in neonates and small infants: Impact of right ventricular exclusion and its indications'. Together they form a unique fingerprint.

Cite this