Isolated unilateral pulmonary artery agenesis: evaluation of natural and long term evolution after corrective surgery.
Isolated unilateral pulmonary artery agenesis: evaluation of natural and long term evolution after corrective surgery.
复制标题
孤立性单侧肺动脉发育不全：矫正手术后自然和长期演变的评估。
DOI：
10.1590/s0066-782x2006001700005
复制
发表时间：
2006
影响因子：
2.6
通讯作者：
M. Barbero
中科院分区：
文献类型：
--
作者：
E. Atik;C. Tanamati;L. Kajita;M. Barbero
目标
单侧肺动脉发育不全是一种罕见的孤立性异常，自 1978 年以来仅报告了 119 例。一般来说，儿童的病情表现为肺动脉高压（PH），成人则表现为咯血。 17% 的病例进行了肺动脉重建和肺叶切除术等干预措施。我们分析了其中的 4 例，其中 2 例是自然进化的，2 例是手术矫正后晚期 PH 消退的。
方法
该研究包括三名 22、10 和 35 个月大的男孩和一名 20 个月大的女孩。 22个月大的孩子出现右心衰竭（RHF）和紫绀； 10 个月大的婴儿出现 RHF，另外两只则出现劳累性疲劳。所有患者均出现肺PH症状、心电图显示右心室劳损以及心脏肥大。心导管检查显示对侧肺动脉存在全身压力，其中三名儿童存在右侧肺发育不全，一名儿童存在左侧肺发育不全。
结果
在 22 个月大和 10 个月大的婴儿中，可以使用肺动脉至发育不全的对侧肺门之间的 7 毫米直径 Goretex 导管进行肺动脉连续性手术矫正。 PH 体征出现早期和晚期消退，并且儿童在 7 岁和 2.5 岁的随访期间分别保持稳定。两种情况下，左心室和右心室之间的压力比分别为 30% 和 40%。两种情况下，肺灌注分别从 8% 增加到 44% 和 8% 到 23%。为其他患者安排了相同的手术。
结论
该技术已成为类似病例的首选手术方法，即使在存在严重肺动脉高压和对侧肺动脉发育不全的情况下，文献中也很少描述这些病例。
OBJECTIVE
Unilateral pulmonary artery agenesis is an uncommon isolated anomaly and since 1978 only 119 cases have been reported. In general the condition presents as pulmonary hypertension (PH) in children and hemoptysis in adults. Interventions such as pulmonary artery reconstruction and lobectomies were performed in 17% of the cases. We analyzed four of these cases, two in natural evolution and two with late term PH regression after surgical correction.
METHODS
Three 22,10 and 35 month old male children and one 20 month old female child were included in the study. The 22 month old presented right-sided heart failure (RHF) and cyanosis; the 10 month old presented RHF and the other two presented exertion fatigue. All had PH symptoms, right ventricular strain on the EKG and cardiomegaly. Cardiac catheterization showed systemic pressures in the contralateral pulmonary artery, with right-sided agenesis in three of the children and left-sided agenesis in one child.
RESULTS
Surgical correction of pulmonary artery continuity was possible in the 22 month old and 10 month old using a 7 mm diameter Goretex conduit between the pulmonary arteries up to the hypoplastic contralateral pulmonary hilum. There was early and late regression of the PH signs and the children remained stable during follow-up to the ages of 7 and 2.5 years, respectively. The pressure ratio between the left and right ventricles was 30 and 40%, in both cases. Pulmonary perfusion increased from 8 to 44% and from 8 to 23%, in the two cases. The same procedure was scheduled for the other patients.
CONCLUSION
This technique has become the operation of choice for similar cases, that are rarely described in literature, even in the presence of severe PH and contralateral pulmonary artery hypoplasia.