主肺动脉窗25例临床分析

目的 分析主肺动脉窗（APW）的发病、分型和伴发畸形情况，总结诊断及治疗经验。方法 回顾性分析复旦大学附属儿科医院2003至2013年诊治的25例APW患儿的临床症状、病理分型、合并畸形、诊断、治疗和预后资料。结果 25例APW患儿进入分析，男14例，女11例，年龄5 d至13岁（6月龄以下12例）,占同期住院先天性心脏病患儿的0.18%。Ⅰ型5例（20%），Ⅱ型16例（60%），Ⅲ型1例（4%），Ⅴ型3例（12%）。单纯APW 10例，APW合并单纯卵圆孔未闭3例；余12例（48.0%）均合并其他需纠正的心血管畸形，其中合并单纯房间隔缺损（ASD）1例，单纯动脉导管未闭（PDA）2例，单纯室间隔缺损(VSD)3例，右室双出口和ASD 1例，主动脉瓣狭窄和右位主动脉弓1例，肺动脉瓣闭锁伴VSD、右位主动脉弓、永存左上腔静脉1例， 主动脉弓离断及PDA 3例。超声心动图诊断17例，误诊或漏诊8例（32%）中5例通过心导管检查及造影诊断，3例外科术中诊断。25例均有不同程度肺动脉高压，其中2例心导管检查评估为阻力性肺动脉高压，4例家长放弃诊治，其余19例施行一期根治术，术后死亡2例（10.5%）。17例术后随访3个月至9年3个月，平均（23±27）个月，其中15例临床无症状，复查超声心动图APW修补无残余，肺动脉压力逐渐下降至正常。结论 APW缺损主要发生在主动脉远端，常伴有间隔缺损、PDA和主动脉弓病变等；超声心动图为首选检查方法，但必要时需行心导管检查及造影。APW患儿易早期发生肺动脉高压，一经诊断尽早手术，可获得较好的中长期疗效。

Clinical analysis of 25 cases with aortopulmonary window

Objective To study the morbidity, anatomic malformation, diagnosis and management of aortopulmonary window （APW）. Methods The records of 25 patients with APW, aged 5 days to 13 years were reviewed. Results Fourteen cases were male and 11 cases were female, and 12 cases were under 6 months. Windows were situated in the proximal of the semilunar valve （type Ⅰ ） in 4 cases （16%）, and distal of the aorta （type Ⅱ ） in 17 cases （64%）, from proximal to distal （type Ⅲ） in only 1 case （4%）. Three cases were associated with interruption of the aortic arch （IAA） （type V ）. About half patients （52%） were isolated APW （10 cases） or APW only associated with patent foramen ovale （4 cases）, while the other half were associated with cardiac defects, including atrial septal defects （ASD） in 1 case, patent ductus arterious （PDA） in 2 cases, ventricular septal defect （VSD） in 3 cases, double outlet right ventricle （DORV） and ASD in 1 case, aortic stenosis and right aortic arch in 1 case, pulmonary atresia with VSD and right aortic arch, persistent left superior vena cava in 1 case, IAA and PDA in 3 cases. Seventeen cases were diagnosed by echocardiography. However, echocardiography failed in diagnosis of APW in 8 cases （32%）, which was further diagnosed by angiogram in 4 cases, by surgery in 3 cases. All patients had varied degree pulmonary hypertension and 2 older patients were evaluated as pulmonary vascular obstructive disease without indictions for surgery. The parents of 4 patients refused further management. The rest of 19 patients underwent surgical repair and 2 died （ 10.5% ） because of severe associated cardiac anomaly. Three months to 9 years follow-up showed good outcomes including well-doing clinically and satisfactory echocardiography. The pulmonary hypertension was gradually recovered. Conclusions Most APW are in the distal of the aorta, and about half of the patients are associated with septal defects, PDA, IAA, DORV and PA/VSD. Echocardiography is the preferred method for diagnosis; however, ECHO has a relatively high misdiagnosing rate in this type malformation, further investigations such as cardiac catheterization and angiography are needed, especially in those older patients with pulmonary vascular disease. Surgical repair should be considered as soon as APW is diagnosed even in neonate stage, and has a good prognosis in mid- to-long term follow-up.