婴儿室间隔完整型肺动脉闭锁的食管超声心动图表现及其应用价值

目的：探讨婴儿室间隔完整型肺动脉闭锁（ PA-IVS）的食管超声心动图特征及其应用价值。方法21例PA-IVS患儿，年龄1 d至3个月，均行食管超声心动图检查。以手术所见结果为标准，评价食管超声心动图诊断PA-IVS的准确率，总结患儿的手术方案及其食管超声心动图表现。结果（1）21例患儿均手术证实为PA-IVS，食管超声心动图检查的诊断准确率为95．24％（20／21）。（2）21例患儿均为左位心，19例（90．5％）存在肺动脉瓣完全性闭锁，20例（95．2％）肺动脉发育较好；14例（66．67％）存在卵圆孔未闭，7例（33．33％）房间隔缺损，18例（85．7％）伴有动脉导管未关闭；4例（19．0％）存在右室与冠状动脉交通，但仅2例（9．5％）为右室依赖的冠状动脉循环（RVDCC）。（3）12例患儿三尖瓣 Z值＞－2，采取瓣膜切开术或跨瓣环补片治疗；5例患儿三尖瓣Z值为－3～－2，采取体肺分流术加肺动脉流出道重建术治疗；3例患儿三尖瓣Z值＜－4，采取单心室修补术或体肺分流术治疗；仅1例患儿术中改变术式。经治疗后21例患儿临床症状体征明显改善。结论食管超声心动图可清晰显示 PA-IVS患儿的肺动脉闭锁情况，多数患儿合并其他心脏畸形，少数存在RVDCC；食管超声心动图检查对PA-IVS的诊断率高，可对后期手术方案的制订提供可靠的指导依据。

Manifestations and application value of esophageal echocardiography in infants with complete pulmonary atresia with intact ventricular septum

Objective To explore the characteristics and application value of esophageal echocardiography in infants with complete pulmonary atresia with intact ventricular septum(PA-IVS) .Methods Twenty-one infants with PA-IVS aged one day to three months underwent esophageal echocardiography.And the accuracy of esophageal echocardiography for the diagnosis of PA-IVS was evaluated with a standard of surgical result.The surgical plan and manifestations of esophageal echocardiography were summarized .Results ①All of 21 infants with PA-IVS were confirmed by surgery,and the diagnostic accuracy of esophageal echocardiography was 95.24%(20/21).②Sinistrocardia was observed in 21 infants,complete pulmonary valve atresia in 19 infants(90.5%),and well-developed pulmonary artery in 20 infants(95.2%).Acleistocardia existed in 14 infants(66.67%),atrial septal defect in 7 infants(33.33%),and patent ductus arteriosus in 18 infants(85.7%).Of 4 infants (19.0%) with communicating branches between right ventricle and coronary artery , right ventricle-dependent coronary circulation ( RVDCC ) existed in 2 infants(9.5%).③Twelve infants with Z value of tricuspid valve >-2 underwent valvotomy or trans-annular patch.Five infants with Z value between -3 and -2 underwent systemic-to-pulmonary artery shunt combined with pulmonary artery outflow tract reconstruction . Three infants with Z value <-4 underwent single ventricle repair or systemic-to-pulmonary artery shunt.Conversion of surgical approach occurred in only one case.The symptoms and signs improved after surgery in21 infants.Conclusion Esophageal echocardiography can display the pulmonary valve atresia clearly .Most of the infants with PA-IVS were complicated with other cardiac deformities ,and a few infants with RVDCC.Esophageal echocardiography achieves a high accuracy for the diagnosis of PA -IVS,and can provide a reliable guidance basis for formulating the surgical plans in a later stage .