Tetralogy of Fallot: The Burden of Pulmonary Atresia in the NICU Set-Up: Two Case Reports and a Literature Review

Tetralogy of Fallot: The Burden of Pulmonary Atresia in the NICU Set-Up: Two Case Reports and a Literature Review

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 out...

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Main Authors: Ion Dragomir, Diana Iulia Vasilescu, Adriana Mihaela Dan, Diana Voicu, Sorin Liviu Vasilescu, Laura Andreea Stefan, Alin Nicolescu, Monica Mihaela Cîrstoiu
Format: Article
Language:English
Published: MDPI AG 2025-06-01
Series:Children
Subjects:
tetralogy of Fallot
pulmonary atresia
right ventricular outflow tract obstruction
congenital heart disease
mechanical ventilation
hemodynamics
Online Access:https://www.mdpi.com/2227-9067/12/6/780
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Summary: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.
ISSN:2227-9067

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