Weeda Jesse A, Te Pas Arjan B, Haak Monique C, Blom Nico A, van der Palen Roel L F
Division of Paediatric Cardiology, Department of Paediatrics, Willem-Alexander Children's Hospital, Leiden University Medical Centre (LUMC), Leiden, The Netherlands.
Division of Neonatology, Department of Paediatrics, Willem-Alexander Children's Hospital, Leiden University Medical Centre (LUMC), Leiden, The Netherlands.
Biomed Hub. 2024 Dec 2;10(1):1-7. doi: 10.1159/000542723. eCollection 2025 Jan-Dec.
Transposition of the great arteries (TGA), especially with intact ventricular septum (TGA-IVS), presents unique challenges during fetal-to-neonatal transition, which can contribute to developing persistent pulmonary hypertension of the newborn (PPHN).
A male newborn with TGA-IVS, delivered via caesarean section, presented with hypoxemia and tachycardia immediately after birth (preductal SpO: 50-60%, post-ductal SpO: 70-75%). Echocardiography revealed a floppy interatrial septum and two interatrial connections with bidirectional shunting. Ductal flow showed systolic right-to-left shunting, suggesting high pulmonary vascular resistance. Immediate post-birth management included non-invasive respiratory support with continuous positive airway pressure at 100% oxygen and administration of prostaglandin E2 to maintain ductal patency. Despite initial low oxygen saturation levels, escalation of intensive treatments was deferred based on continuous trend monitoring of vital signs and echocardiographic indicators. Oxygenation and circulation gradually improved within the first 2 h after birth to normal values, obviating escalation of intensive interventions like intubation, nitric oxide and/or balloon atrial septostomy. Arterial switch operation at day 3 post-birth was successful.
This case highlights the possible contribution of fetal-to-neonatal transition in TGA-IVS to developing PPHN, which may subside after transition. Moreover, this case highlights the potential for providing a gentle hemodynamic transition without invariably needing early invasive interventions after birth.
大动脉转位(TGA),尤其是室间隔完整的大动脉转位(TGA-IVS),在胎儿至新生儿过渡期间带来独特挑战,这可能导致新生儿持续性肺动脉高压(PPHN)的发生。
一名患有TGA-IVS的男性新生儿通过剖宫产出生,出生后立即出现低氧血症和心动过速(导管前血氧饱和度:50%-60%,导管后血氧饱和度:70%-75%)。超声心动图显示房间隔松弛,存在两个房内连接并伴有双向分流。导管血流显示收缩期右向左分流,提示肺血管阻力高。出生后立即进行的管理包括采用100%氧气持续气道正压通气的无创呼吸支持,并给予前列腺素E2以维持动脉导管通畅。尽管最初血氧饱和度水平较低,但基于对生命体征和超声心动图指标的持续趋势监测,暂未升级强化治疗。出生后最初2小时内氧合和循环逐渐改善至正常水平,从而避免了如插管、一氧化氮和/或球囊房间隔造口术等强化干预措施的升级。出生后第3天进行的动脉调转手术成功。
该病例突出了TGA-IVS中胎儿至新生儿过渡对PPHN发生的可能作用,这种作用可能在过渡后消退。此外,该病例突出了在出生后不一定需要早期侵入性干预即可实现温和血流动力学过渡的可能性。