主肺动脉窗合并右肺动脉起源于主动脉和主动脉弓离断患儿诊治分析

The diagnosis and treatment of the aortopulmonary window combined with anomalous origin of the right pulmonary artery from the aorta and interruption of the aortic arch in children

目的探讨主肺动脉窗(APW)合并右肺动脉起源于主动脉(AORPA)及主动脉弓离断(IAA)患儿的诊断与治疗。方法回顾性分析1例APW合并AORPA及IAA患儿的临床资料。结果患儿,女,4个月,外院诊断为室间隔缺损,保守治疗无效,无法撤离呼吸机,入院后超声心动图提示为APW、AORPA、动脉导管未闭、重度肺动脉高压;外科手术后患儿出现少尿,上、下肢动脉压差大,经CT检查提示合并IAA,再次外科手术矫治;术后患儿顺利恢复并出院,随访28个月,生长发育与同龄儿无明显差别。结论 APW合并AORPA及IAA患儿一经诊断需尽早手术治疗,一期外科纠治效果满意,术前应完善心血管造影等检查防止漏诊。

Objective To explore the diagnosis and treatment of the the aortopulmonary window （APW） combined with anomalous origin of the right pulmonary artery from the aorta （AORPA） and interruption of the aortic arch （IAA） in children. Methods The clinical data of one pediatric patient diagnosed of the APW combined with AORPA and IAA in April 2013 was retrospectively analyzed. Results The 4-month-old female patient was diagnosed with ventricular septal defect in local hospital. The conservative treatment was not effective and the ventilator weaning was not successful. After admission, the echocardiography suggested APW, AORPA, patent ductus arteriosus and severe pulmonary hypertension. The oliguria and the big difference of arterial pressure between upper and lower limbs were presented after surgery. A computed tomography scan showed that the patient also had IAA. After a second surgery, the patient recovered and was discharged. During 28 months of the follow-up, the growth and develop- ment of the patient were the same as normal children. Conclusions APW combined with IAA and AORPA require early surgical procedures after diagnosis. Angiocardiography and other tests should be performed to avoid miss-diagnosis before surgery.