Anatomic Repair of Ebstein's Malformation: Lessons Learned With Cone Reconstruction


a Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Republic of Korea
f Department of Thoracic and Cardiovascular Surgery, Seoul National University Children's Hospital, Seoul, Republic of Korea
b Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea
c Division of Pediatric Cardiology, Severance Cardiovascular Hospital, Yonsei University Health System, Seoul, Republic of Korea
d Department of Pediatrics, Sejong Cardiovascular Institute, Sejong General Hospital, Bucheon, Republic of Korea
e Division of Pediatric Cardiology, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Republic of Korea

* Address correspondence to Dr Bae, Department of Pediatrics, Seoul National University Children's Hospital, 101 Daehang-Ro, Jongno-gu, Seoul 110-744, Republic of Korea (Email:

Background: Primary pulmonary vein stenosis or atresia (PVS/A) is a rare entity with a high mortality rate. The aim of this study was to elucidate the clinical characteristics, progression, and prognostic factors of primary PVS/A in children.

Methods: We reviewed the medical records of patients who had primary PVS/A with normally connected pulmonary veins (PVs) at five pediatric cardiology centers in Korea between 1995 and 2010.

Results: A total of 34 cases were identified. The median age at diagnosis was 12.0 months. During the follow-up period (median, 18 months; range, 2 to 185 months), PVS/A progressed to previously uninvolved veins in 9 patients. Surgical interventions were performed on 29 patients (venoplasty on 25 and pneumonectomy on 4). Nineteen of the patients who underwent venoplasty had restenosis after a median of 2 months. The sutureless technique did not reduce the rate of restenosis, progression of the disease to previously uninvolved PVs, or mortality rate. The mortality rate was 46.7%, the median age of death was 10.8 months, and the median interval between diagnosis and death was 3.0 months. In univariate analysis, predictors of death included involvement of at least three PVs, bilateral PV involvement, infancy-onset PVS/A, restenosis after surgery, and progression to previously uninvolved PVs. In multivariate analysis, significant risk factors for death were involvement of at least three PVs (hazard ratio, 8.8; p < 0.0001) and progression to uninvolved PVs (hazard ratio, 4.2; p = 0.014).

Conclusions: Primary PVS/A may carry a significant risk of recurrent and progressive PV obstruction or death even after surgical venoplasty.

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