小儿三尖瓣下移畸形的手术效果分析

Surgical repairing of Ebstein anormaly in children

目的＆JLEbstein畸形的手术纠治是当前的难点，本文意在探讨小儿Ebsetin畸形手术纠治的方法和分析手术效果。方法回顾性分析2000年1月至2014年12月间于复旦大学附属儿科医院心血管中心就诊并手术的Ebstein畸形患儿45例，其中男28例，女17例，年龄0．2～169个月，平均（44．2±41．1）个月，含新生儿6例。体重2．4～60kg，平均（17．6±12．8）kg，SP02在60％～99％，平均（87．8±8．8）％，其中26．7％（12／45）存在较明显的紫绀，22．2％（10145）存在明显心功能不全，合并Wolff-Parkinson-White预激综合征3例，房性早搏2例。结果全组45例患儿接受了心脏手术，6例新生儿中5例在非体外循环下接受了改良B-T分流术，2例于术后早期死亡。39例患儿在体外循环下行三尖瓣成形术，Danielson法12例，Carpentier法21例，Cone法6例，无早期和晚期死亡。37例患儿于门诊密切随访，随访1．5个月至15年，7例因为三尖瓣反流加重进行了再手术。5、10、15年免再干预率分别为86．5％、81．1％、81．1％。按照不同手术方式分为3组进行比较，Carpentier组和Danielson组比较发现，Carpentier组手术年龄偏小[（45．0±33．7）个月比（80．8±40．2）个月，P=0．010]，体重偏轻[（17．3±11．3）艇比（29．5±10．5）kg，P=0．004]，所需主动脉阻断时间长[（44．9±4．7）min比（30．8±5．3）min，P=0．ooo]，术后恢复时间无差异，随访术后再干预率差异无统计学意义。Cone组和Carpentier组比较发现，Cone组手术年龄更小[（12．2±7．3）个月比（45．0±33．7）个月，P=0．000]，所需的体外循环时间[（89．2±12．0）min比（73．0±15．5）min，P=0．027]，主动脉阻断时间更长[（56．3±6．1）min比（44．9±4．7）min，P=0．ooo]，术后恢复时间差异无统计学意义，随访三尖瓣反流轻，无一例需要再干预，但是与Carpentier组相比差异无统计学意义，可能与病例数少（仅有6例），随访时间偏短有关。结论Cone手术较其他手术方式更为复杂，手术时间较长，但是适用于小年龄儿童Ebstein畸形的矫治，早中期效果满意，仍需密切随访。

Objective To report our experiences of treating pediatric Ebstein anomaly with various approaches. Methods The clinical data were reviewed for 45 surgical patients of Ebstein anomaly at our hospital between January 2000 and December 2014. There were 6 neonates. The mean age was （44. 2 ± 41.1 days） （0. 2±169 months）, mean body weigh （17. 6 ± 12. 8） （2. ±60） kg and mean oxygen saturation （SPO2） （87. 8 ± 8. 8） % （60-99） %. There were obvious cyanosis （n = 12）, heart failure （n = 10）, Wolff-Parkinson-White syndrome （n = 3） and atrial arrhythmia （n = 2）. Results Among 6 neonates, 5 underwent modified B-T shunt and another one had valve repair by commissure plasty. And there were two early deaths. The other 39 patients underwent tricuspid valvuloplasty via Danielson （n = 12）, Carpentier （n = 21） and cone （n = 6） without early-and-late mortality. And 37 children were followed up for 1.5 - 180 months. Seven were re-operated due to tricuspid regurgitation. Freedom from re-intervention was 86. 5 %, 81.1%, 81.1% at 5, 10, 15 years post-operation. Compared to Danielson group, Carpentier group had younger age （45.0 ± 33. 7 vs 80. 8±40. 2 m, P = 0. 010）, less weight （17. 3 ± 11.3 vs 29. 5 ± 10. 5 kg, P = 0. 004） and longer aortic cross clamp duration （44. 9 ± 4. 7 vs 30. 8 ± 5.3 min, P = 0. 000） with similar recovery time and re-intervention rate. Compared to Carpentier group, Cone group had younger age （12. 2 ± 7. 3 vs 45.0 ± 33. 7 m, P = 0. 000）, longer cardiopulmonary duration （89. 2 ± 12. 0 min vs 73.0 ± 15.5 min, P = 0. 027） and longer cross clamp duration （56. 3 ± 6. 1 vs 44. 9 ±4. 7 rain, P = 0. 000）. None needed reintervention with gentle-to-mild tricuspid valve regurgitation. Conclusions Cone is a much more complicated and time-consuming procedure than two other surgical approaches. However it yields excellent short-term outcomes for young patients. Further follow-up are warranted.