The histological findings in transposition of the great artery with severe persistent pulmonary hypertension of the newborn
The histological findings in transposition of the great artery with severe persistent pulmonary hypertension of the newborn
复制标题
新生儿重度持续性肺动脉高压大动脉转位的组织学表现
DOI：
10.1016/j.jccase.2018.01.003
复制
发表时间：
2018
影响因子：
--
通讯作者：
Maruo Yoshihiro
中科院分区：
文献类型：
--
作者：
Hoshino Shinsuke;Somura Junpei;Furukawa Ouki;Yanagi Takahide;Maruo Yoshihiro
新生儿持续性肺动脉高压（PPHN）合并大动脉转位（TGA）对治疗和预后有严重影响，常伴有不良结局。我们报告一例男性足月新生儿TGA合并完整室间隔和严重PPHN，出生后2小时死亡；进一步，我们检查了他的血管组织学。尸检时，肺组织学显示内膜轻度纤维性肥大，小肺动脉内侧中度肥大。未见肺动脉发育不全。发现肺充血，气化不良。肺泡内有碎片。检测到含铁血黄素沉积，提示产前止血或出血。严重的PPHN可能发生于胎儿期肺动脉痉挛伴肺充血。TGA的肺血管床可出现广泛的病变。TGA和PPHN肺血管组织的病理形态不一致。学习目的：大动脉转位伴室间隔完整（TGA/IVS）的新生儿可能有严重的肺动脉高压。TGA/IVS肺血管疾病的进展是不可预测的；因此，在诊断为TGA/IVS的胎儿分娩时，应在能够进行导管置入和球囊房间隔造口术的机构进行
The combination of persistent pulmonary hypertension of the newborn (PPHN) and transposition of the great arteries (TGA) has serious impacts on treatment and prognosis, often with adverse outcomes. We report the case of a male full-term newborn with TGA with intact ventricular septum and severe PPHN who died 2 h after birth; further, we examined his vascular histology. On autopsy, lung histology showed mild fibrous hypertrophy in the intima and moderate medial hypertrophy of the minimal pulmonary artery. Hypoplasia of the pulmonary artery was not detected. Pulmonary congestion was detected and pneumatization was poor. Debris was present in the alveoli. Hemosiderin deposition was detected, suggesting prenatal hemostasis or hemorrhage. Severe PPHN may have occurred because of pulmonary arterial spasm accompanying pulmonary congestion which had been in the fetal stage. A wide range of lesions can be present in the pulmonary vascular bed in TGA. The pathologies of pulmonary vascular tissues with TGA and PPHN are not uniform.<Learning objective:Neonates with transposition of the great arteries with intact ventricular septum (TGA/IVS) may have severe pulmonary hypertension. The progression of pulmonary vascular disease in TGA/IVS is unpredictable; therefore, the delivery of a fetus diagnosed with TGA/IVS should be performed at an institution in which catheterization and balloon atrial septostomy can be performed.>