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成功挽救一名孤立性单侧肺动脉缺如新生儿的左肺动脉。

Successful salvage of the left pulmonary artery in a neonate with isolated unilateral absence of the pulmonary artery.

作者信息

Kawada Kota, Saiki Hirofumi, Kemmochi Manabu, Kuwata Seiko, Takanashi Manabu, Miyaji Kagami, Senzaki Hideaki

机构信息

Department of Neonatology, Kitasato University, Sagamiraha, Japan.

Kawada Pediatric Clinic, Shizuoka, Japan.

出版信息

J Cardiol Cases. 2020 Jan 10;21(5):169-171. doi: 10.1016/j.jccase.2019.12.004. eCollection 2020 May.

Abstract

Isolated unilateral absence of the pulmonary artery (UAPA) is a congenital anomaly where involution of the extrapulmonary PA is insufficient and the intrapulmonary PA is only fed by the ductus arteriosus. Affected lung disorder causes complications years after ductus closure; thus, early diagnosis is of importance to avoid these complications. Here, we present the case of a male infant who was admitted to the neonatal intensive care unit because of transient tachypnea of the newborn and absence of the left PA (LPA) was indicated. Intensive echocardiography could detect neither the LPA nor the aortopulmonary collateral arteries to the left lung. Although the ductus was orthotopic with the right aortic arch, use of prostaglandin (PG) E1 unmasked the diagnosis of UAPA with bilateral ductus arteriosus. After ductal closure, delineation of the anatomy is not necessarily easy even with catheterization, whereas early use of PGE1 facilitates anatomical understanding by echocardiography, particularly early after birth. < While identification of disrupted pulmonary artery is often unsuccessful after ductal closure in the patients with unilateral absence of pulmonary artery, use of prostaglandin in the neonate with right aortic arch and large orthotropic right ductus arteriosus allowed successful recirculation of the disrupted pulmonary artery by reopening closed contralateral ductus arteriosus. Prostaglandin infusion early after birth delineated anatomical diagnosis of unilateral absence of pulmonary artery due to bilateral ductus, and it also made it possible to perform single stage uni-focalization.>.

摘要

孤立性单侧肺动脉缺如(UAPA)是一种先天性异常,即肺外肺动脉的退化不充分,肺内肺动脉仅由动脉导管供血。受影响的肺部疾病在动脉导管闭合数年之后会引发并发症;因此,早期诊断对于避免这些并发症至关重要。在此,我们报告一例男婴,因其新生儿短暂性呼吸急促入住新生儿重症监护病房,检查提示左肺动脉(LPA)缺如。经强化超声心动图检查,既未发现LPA,也未发现至左肺的主肺动脉侧支动脉。尽管动脉导管位置正常,与右位主动脉弓相连,但使用前列腺素(PG)E1后明确诊断为双侧动脉导管的UAPA。动脉导管闭合后,即使通过心导管检查也不一定能轻易明确解剖结构,而早期使用PGE1有助于通过超声心动图了解解剖结构,尤其是在出生后早期。< 在单侧肺动脉缺如的患者中,动脉导管闭合后往往无法成功识别中断的肺动脉,但对于右位主动脉弓和位置正常的较大右位动脉导管的新生儿,使用前列腺素可通过重新开放闭合的对侧动脉导管,成功实现中断肺动脉的再循环。出生后早期输注前列腺素可明确双侧动脉导管所致单侧肺动脉缺如的解剖诊断,还使得单阶段单灶化手术成为可能。>

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