Dragomir Ion, Vasilescu Diana Iulia, Dan Adriana Mihaela, Voicu Diana, Vasilescu Sorin Liviu, Stefan Laura Andreea, Nicolescu Alin, Cîrstoiu Monica Mihaela
Department of Neonatology, Emergency University Hospital Bucharest, 050098 Bucharest, Romania.
"Carol Davila" University of Medicine and Pharmacy Bucharest, 050474 Bucharest, Romania.
Children (Basel). 2025 Jun 14;12(6):780. doi: 10.3390/children12060780.
Tetralogy of Fallot (ToF) with pulmonary atresia (PA) and complete right ventricular outflow tract obstruction (RVOTO) represents one of the most critical forms of congenital heart disease in neonates. These cases require complex and timely interventions to ensure survival and optimize long-term outcomes. While surgical correction offers a favorable prognosis, the period from birth to surgery is often marked by significant hemodynamic, respiratory and nutritional challenges, particularly in neonatal intensive care units (NICUs). This study aims to outline a structured, physiology-guided approach to the preoperative management of neonates with ToF and complete RVOTO, emphasizing stabilization strategies, hemodynamic support, ventilatory management and nutritional optimization. We performed a focused literature review of practices in neonatal ToF management and illustrated our experience through two case reports highlighting divergent outcomes in infants with the same anatomical diagnosis. The management strategies covered include delivery room stabilization, the use of prostaglandins, mechanical ventilation techniques, nutritional interventions and the timing of surgical intervention. A phased, physiology-guided management strategy is the key to the successful preoperative treatment of ToF with pulmonary atresia. Optimizing hemodynamics, ensuring adequate pulmonary blood flow and supporting nutritional needs are the main drivers for growth and may reduce the time from diagnosis to surgical correction.
法洛四联症（ToF）合并肺动脉闭锁（PA）及完全性右心室流出道梗阻（RVOTO）是新生儿先天性心脏病中最严重的形式之一。这些病例需要复杂且及时的干预措施以确保存活并优化长期预后。虽然手术矫正可带来良好的预后，但从出生到手术这段时间通常面临显著的血流动力学、呼吸和营养方面的挑战，尤其是在新生儿重症监护病房（NICU）。本研究旨在概述一种结构化的、以生理学为导向的方法，用于管理患有ToF和完全性RVOTO的新生儿术前情况，重点强调稳定策略、血流动力学支持、通气管理和营养优化。我们对新生儿ToF管理实践进行了重点文献综述，并通过两个病例报告阐述了我们的经验，这两个病例报告突出了具有相同解剖诊断的婴儿的不同结局。所涵盖的管理策略包括产房稳定、前列腺素的使用、机械通气技术、营养干预以及手术干预的时机。分阶段的、以生理学为导向的管理策略是成功术前治疗合并肺动脉闭锁的ToF的关键。优化血流动力学、确保充足的肺血流量并满足营养需求是生长的主要驱动因素，并且可能缩短从诊断到手术矫正的时间。
