房室间隔缺损(AVSD)合并Ebstein’s畸形少见,占AVSD的0.3％,文献仅报道18例。AVSD的形式分部分性、中间性和完全性,前二者多见,患者通过手术修补可得以存活,后者文献仅见2例。作者报道了完全性AVSD并Ebstein’s畸形1例,女,1天,因Down’s综合征、青紫、轻度呼吸窘迫和心脏杂音入院。体检:重度紫绀,血氧饱和度为73％,胸骨右缘收缩期杂音4级,心尖部隆隆样舒张期杂音。胸片显示心影扩大,心胸比0.76。心电图表现额面QRS Atrioventricular septal defect (AVSD) with Ebstein’s deformity is rare, accounting for 0.3% of AVSD, reported in the literature only 18 cases. AVSD sub-part of the form, the nature and completeness of the former two more common, the patient survived by surgical repair, the latter only 2 cases of literature. The authors reported 1 case of complete AVSD and Ebstein’s malformation, female, 1-day admission due to Down’s syndrome, bruising, mild respiratory distress, and heart murmur. Physical examination: severe cyanosis, oxygen saturation of 73%, sternum systolic murmur right edge of 4, apical droop diastolic murmur. Chest X-ray showed enlarged heart ratio of 0.76. ECG showed frontal QRS