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目的评估婴儿期主动脉弓离断(IAA)的诊断及术前处理。方法回顾性分析本院2001年1月-2007年11月诊断为IAA的53例婴儿临床表现、辅助检查[超声心动图(Echo)、螺旋CT、MRI、心血管造影等]、术前处理、手术治疗预后等临床资料。结果男38例,女15例;年龄1d～12(3.05±3.53)个月,新生儿占50.94%;体质量2.18～10.0(4.32±1.60)kg。首发症状:90.57%患儿有气促,83.02%患儿有喂养困难;83.02%患儿有不同程度的充血性心功能不全,37.74%患儿心功能Ⅲ级。病例均有不同程度的股动脉搏动减弱。53例均行Echo检查,Echo检查确诊为IAA38例;提示IAA或重度主动脉缩窄(CoA)6例,经CT检查诊断为IAA;提示重度CoA9例,经CT或手术诊断为IAA。33例行CT检查,其中15例行手术者,心血管畸形与手术所见一致。3例行MRI检查。7例行心血管造影。结合Echo、CT、MRI、心血管造影及手术结果,53例中A型35例,B型15例,C型3例。术前处理主要为维持动脉导管开放、抗心力衰竭治疗及支持治疗。经适当的内科处理后,大部分患儿充血性心力衰竭症状有不同程度改善。手术治疗26例,存活16例,围术期死亡10例,主要死因为严重低心排出量。结论Echo对本病的诊断有一定的局限性,Echo结合CT或MRI可以替代Echo结合心导管造影诊断IAA的传统方法,方便、安全。合适的术前处理有利于患儿顺利过渡到手术,有利于手术的成功。
Objective To evaluate the diagnosis and preoperative treatment of infantile aortic arch disconnection (IAA). Methods The clinical data of 53 infants diagnosed with IAA from January 2001 to November 2007 in our hospital were retrospectively analyzed. [Echocardiography, spiral CT, MRI, angiography, etc.] Surgical treatment of prognosis and other clinical data. Results There were 38 males and 15 females. The age ranged from 1 d to 12 (3.05 ± 3.53) months, with the newborn accounting for 50.94% and the body weight ranging from 2.18 to 10.0 (4.32 ± 1.60) kg. The first symptom: 90.57% of children with shortness of breath, 83.02% of children have feeding difficulties; 83.02% of children with varying degrees of congestive heart failure, 37.74% of children with cardiac function grade Ⅲ. Cases have varying degrees of femoral pulse weakened. 53 cases were performed Echo examination, Echo examination confirmed IAA 38 cases; prompted IAA or severe aortic coarctation (CoA) in 6 cases, diagnosed by CT IAA; prompted severe CoA 9 cases, diagnosed by CT or IAA. 33 cases underwent CT examination, of which 15 cases underwent surgery, cardiovascular malformations and surgical findings. 3 routine MRI examination. 7 routine cardiovascular angiography. According to the results of Echo, CT, MRI, cardiovascular angiography and surgery, 35 cases were type A, 15 cases were type B and 3 cases were type C in 53 cases. Preoperative management is mainly to maintain the open duct, anti-heart failure treatment and supportive treatment. After appropriate medical treatment, the majority of children with congestive heart failure symptoms have varying degrees of improvement. Surgical treatment of 26 cases, 16 cases of survival, 10 cases of perioperative deaths, the main cause of death was severe low cardiac output. Conclusion Echo has certain limitations in the diagnosis of this disease. Echo combined with CT or MRI can replace traditional Echo combined with cardiac catheterization in the diagnosis of IAA, which is convenient and safe. Appropriate preoperative treatment is conducive to the smooth transition of children to surgery, is conducive to the success of surgery.