Abu-Sulaiman R M, Hashmi A, McCrindle B W, Williams W G, Freedom R M
Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Ontario, Canada.
Cardiol Young. 1998 Oct;8(4):449-54. doi: 10.1017/s1047951100007101.
Anomalous origin of one pulmonary artery from the ascending aorta is an uncommon lesion with an uncertain outcome. We reviewed 16 consecutive children (9 males) presenting with this lesion over a 36 year period at a single institution. Median age at presentation was 2 days (range, birth to 3.2 years). The anomalous pulmonary was the right in 12 and the left in 4, all originating from the proximal ascending aorta, with no patient having stenosis at the origin of the anomalous pulmonary artery. Associated cardiac anomalies were noted in 9 patients. No intervention was attempted in 2 patients: one was deemed inoperable due to complex associated lesions and pulmonary vascular obstructive disease, while the other one died before repair. Surgical intervention was attempted in 14 patients, with 3 intraoperative deaths (21%). Of 11 operative survivors, 8 developed pulmonary arterial stenosis graded severe in 2, moderate in 1 and mild in 5. Patients with severe stenosis required surgical angioplasty, 1 after unsuccessful dilation combined with placement of an endovascular stent. One patient with moderate, and one with mild, stenosis underwent successful transcatheter balloon dilation. The remaining 4 patients with mild stenosis remain unchanged during follow-up. One patient had biopsy evidence of pulmonary vascular obstructive disease at 3.3 years of age. There were no late deaths, giving a total mortality of 25% (4/16).
While early diagnosis and repair of anomalous origin of one pulmonary artery from the ascending aorta is necessary, restenosis of the site of repair is common.
一条肺动脉从升主动脉异常起源是一种罕见病变，其预后不确定。我们回顾了在单一机构36年间连续收治的16例患有该病变的儿童（9例男性）。就诊时的中位年龄为2天（范围：出生至3.2岁）。异常肺动脉位于右侧的有12例，位于左侧的有4例，均起源于升主动脉近端，无患者在异常肺动脉起源处存在狭窄。9例患者存在相关心脏异常。2例患者未尝试干预：1例因复杂的相关病变和肺血管阻塞性疾病被认为无法手术，另1例在修复前死亡。14例患者尝试了手术干预，术中死亡3例（21%）。在11例手术幸存者中，8例出现肺动脉狭窄，其中重度2例，中度1例，轻度5例。重度狭窄患者需要手术血管成形术，1例在扩张失败并置入血管内支架后进行。1例中度狭窄和1例轻度狭窄患者经导管球囊扩张成功。其余4例轻度狭窄患者在随访期间病情无变化。1例患者在3.3岁时活检有肺血管阻塞性疾病证据。无晚期死亡病例，总死亡率为25%（4/16）。
虽然对于一条肺动脉从升主动脉异常起源需要早期诊断和修复，但修复部位再狭窄很常见。
