title: Outcomes of neonatal Ebstein's anomaly without right ventricular forward flow.
abstract: In neonates with Ebstein's anomaly and absent right ventricular forward flow, pulmonary valve morphology is normal or abnormal. Although initial postnatal presentations of these 2 conditions are similar, clinical courses and therapeutic strategies for each category differ greatly.
  
  
  
  Among 29 neonates with Ebstein's anomaly without right ventricular forward flow on initial postnatal echocardiography, 16 had a normal pulmonary valve and 13 had an abnormal pulmonary valve.
  
  
  
  During the postnatal follow-up of the normal pulmonary valve group, right ventricular forward flow commenced approximately 10 days after birth (1-15 days). The ductus arteriosus was surgically ligated in 3 neonates to facilitate right ventricular forward flow. Biventricular or 1 1/2 ventricular physiology was eventually achieved in 14 patients in the normal pulmonary valve group (14/16, 88%) and 2 patients in the abnormal pulmonary valve group (2/13, 15.3%). With respect to the preoperative echocardiographic findings, the normal pulmonary valve group had a significantly larger pulmonary valve annulus (8.2 ± 1.4 mm in the normal pulmonary valve group and 6.4 ± 1.8 mm in the abnormal pulmonary valve group, P = .002) and smaller cardiothoracic ratio (0.79 ± 0.05 in the normal pulmonary valve group and 0.85 ± 0.07 in the abnormal pulmonary valve group, P = .03). Mild to moderate pulmonary regurgitation was present in all patients (16/16, 100%) in the normal pulmonary valve group, but 3 patients (3/13, 23%) in the abnormal pulmonary valve group also had pulmonary regurgitation. On logistic regression analysis, only pulmonary valve annulus size remained as an indicator of a normal pulmonary valve (P = .03).
  
  
  
  In patients with Ebstein's anomaly and absent right ventricular forward flow, large pulmonary valve annulus size indicated a normal pulmonary valve. Patients with a normal pulmonary valve showed better survival and had a higher probability of achieving biventricular hemodynamics.
authors.affiliation: Division of Pediatric Cardiology, Asan Medical Center, Seoul, South Korea.
authors.forename: Jae Suk
authors.initials: JS
authors.lastname: Baek
authors.affiliation: Division of Pediatric Cardiology, Asan Medical Center, Seoul, South Korea.
authors.forename: Jeong Jin
authors.initials: JJ
authors.lastname: Yu
authors.affiliation: Seoul Women's College of Nursing, Seoul, South Korea.
authors.forename: Yu Mi
authors.initials: YM
authors.lastname: Im
authors.affiliation: Division of Pediatric Cardiac Surgery, Asan Medical Center, Seoul, South Korea. Electronic address: tjyun@amc.seoul.kr.
authors.forename: Tae-Jin
authors.initials: TJ
authors.lastname: Yun
mesh_terms: D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000401:mortality; D004437:Ebstein Anomaly / Q000000981:diagnostic imaging / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D006439:Hemodynamics; D006801:Humans; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008026:Ligation; D016014:Linear Models; D016015:Logistic Models; D008297:Male; D011664:Pulmonary Valve / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000503:physiopathology*; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D016277:Ventricular Function, Left; D016278:Ventricular Function, Right
keywords: Ebstein's anomaly; echocardiography; pulmonary valve