Kawasaki Yuki, Murakami Yosuke, Ehara Eiji, Oshitani Tomoaki, Nakamura Kae, Yoshida Yoko, Suzuki Tsugutoshi, Nishigaki Kyoichi
Department of Pediatric Cardiology, Osaka City General Hospital, Osaka, Japan.
Department of Pediatric Arrhythmia, Osaka City General Hospital, Osaka, Japan.
J Cardiol Cases. 2019 May 23;20(1):30-34. doi: 10.1016/j.jccase.2019.03.009. eCollection 2019 Jul.
Truncus arteriosus (TrA) type A3, according to the Van Praagh (VP) classification, cannot be classified under the Collett and Edwards (C-E) system. In this rare anomaly, postnatal closure of the ductus arteriosus can cause unilateral pulmonary artery obstruction; hence, prenatal diagnosis and early confirmatory postnatal diagnosis are important. This case was referred to our hospital for suspected fetal heart disease at 29 weeks' gestation. TrA C-E type 1 was diagnosed by fetal echocardiography, with a right-sided aortic arch, absent inferior vena cava, and azygos continuation. The neonate was delivered vaginally at 41 weeks' gestation. Postnatal echocardiography showed a right-sided aortic arch with the right pulmonary artery originating from the common arterial trunk and the left pulmonary artery originating from the brachiocephalic artery. The diagnosis was TrA VP type A3, with a right-sided aortic arch and left-sided ductus arteriosus. Patency of the left-sided ductus arteriosus was maintained with prostaglandin E1.alpha-cyclodextrin. Right pulmonary artery banding was performed 3 days after birth. The Rastelli procedure was performed when the patient was 2 months old and weighed 4.2 kg. Delayed diagnosis of VP type A3 can cause unilateral pulmonary artery disconnection; hence, timely and accurate diagnosis is warranted to ensure stable disease management. < Delayed diagnosis of truncus arteriosus (TrA) Van Praagh (VP) type A3 can cause unilateral pulmonary artery disconnection; thus, we should consider both Collett and Edwards and VP classifications, including a ductus arteriosus contralateral to the aortic arch, during TrA diagnosis. Furthermore, to prevent the risks associated with an open-heart surgery in the neonatal period in such cases, pulmonary blood flow can be controlled by maintaining the ductus arteriosus with prostaglandin E1.alpha-cyclodextrin and unilateral pulmonary artery banding.>.
根据范普拉格（VP）分类，A型3型动脉干（TrA）无法归类于科利特和爱德华兹（C-E）系统。在这种罕见的异常情况中，动脉导管出生后闭合可导致单侧肺动脉梗阻；因此，产前诊断和出生后早期确诊很重要。该病例在妊娠29周时因疑似胎儿心脏病转诊至我院。经胎儿超声心动图诊断为C-E 1型TrA，合并右侧主动脉弓、下腔静脉缺如及奇静脉延续。新生儿在妊娠41周时经阴道分娩。出生后超声心动图显示右侧主动脉弓，右肺动脉发自共同动脉干，左肺动脉发自头臂动脉。诊断为VP A3型TrA，合并右侧主动脉弓和左侧动脉导管。通过前列腺素E1α-环糊精维持左侧动脉导管通畅。出生后3天进行了右肺动脉束带术。患儿2个月大、体重4.2 kg时进行了罗斯蒂利手术。VP A3型延迟诊断可导致单侧肺动脉离断；因此，需要及时准确的诊断以确保疾病的稳定管理。<动脉干（TrA）范普拉格（VP）A3型延迟诊断可导致单侧肺动脉离断；因此，在TrA诊断过程中，我们应同时考虑科利特和爱德华兹分类以及VP分类，包括与主动脉弓对侧的动脉导管。此外，为防止此类病例新生儿期心脏直视手术相关风险，可通过使用前列腺素E1α-环糊精维持动脉导管通畅和单侧肺动脉束带来控制肺血流量。>
