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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| Format: | Article |
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MDPI AG
2025-06-01
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| Series: | Children |
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| Online Access: | https://www.mdpi.com/2227-9067/12/6/780 |
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| author | Ion Dragomir Diana Iulia Vasilescu Adriana Mihaela Dan Diana Voicu Sorin Liviu Vasilescu Laura Andreea Stefan Alin Nicolescu Monica Mihaela Cîrstoiu |
| author_facet | Ion Dragomir Diana Iulia Vasilescu Adriana Mihaela Dan Diana Voicu Sorin Liviu Vasilescu Laura Andreea Stefan Alin Nicolescu Monica Mihaela Cîrstoiu |
| author_sort | Ion Dragomir |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-9848b7731a8a4efc86fca8941d8ea357 |
| institution | Kabale University |
| issn | 2227-9067 |
| language | English |
| publishDate | 2025-06-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Children |
| spelling | doaj-art-9848b7731a8a4efc86fca8941d8ea3572025-08-20T03:26:21ZengMDPI AGChildren2227-90672025-06-0112678010.3390/children12060780Tetralogy of Fallot: The Burden of Pulmonary Atresia in the NICU Set-Up: Two Case Reports and a Literature ReviewIon Dragomir0Diana Iulia Vasilescu1Adriana Mihaela Dan2Diana Voicu3Sorin Liviu Vasilescu4Laura Andreea Stefan5Alin Nicolescu6Monica Mihaela Cîrstoiu7Department of Neonatology, Emergency University Hospital Bucharest, 050098 Bucharest, RomaniaDepartment of Neonatology, Emergency University Hospital Bucharest, 050098 Bucharest, RomaniaDepartment of Neonatology, Emergency University Hospital Bucharest, 050098 Bucharest, Romania“Carol Davila” University of Medicine and Pharmacy Bucharest, 050474 Bucharest, RomaniaDepartment of Obstetrics and Gynecology, Emergency University Hospital Bucharest, 050098 Bucharest, Romania“Carol Davila” University of Medicine and Pharmacy Bucharest, 050474 Bucharest, RomaniaDepartment of Pediatric Cardiology, Marie Curie Children’s Hospital, 050098 Bucharest, Romania“Carol Davila” University of Medicine and Pharmacy Bucharest, 050474 Bucharest, RomaniaTetralogy 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.https://www.mdpi.com/2227-9067/12/6/780tetralogy of Fallotpulmonary atresiaright ventricular outflow tract obstructioncongenital heart diseasemechanical ventilationhemodynamics |
| spellingShingle | Ion Dragomir Diana Iulia Vasilescu Adriana Mihaela Dan Diana Voicu Sorin Liviu Vasilescu Laura Andreea Stefan Alin Nicolescu Monica Mihaela Cîrstoiu Tetralogy of Fallot: The Burden of Pulmonary Atresia in the NICU Set-Up: Two Case Reports and a Literature Review Children tetralogy of Fallot pulmonary atresia right ventricular outflow tract obstruction congenital heart disease mechanical ventilation hemodynamics |
| title | Tetralogy of Fallot: The Burden of Pulmonary Atresia in the NICU Set-Up: Two Case Reports and a Literature Review |
| title_full | Tetralogy of Fallot: The Burden of Pulmonary Atresia in the NICU Set-Up: Two Case Reports and a Literature Review |
| title_fullStr | Tetralogy of Fallot: The Burden of Pulmonary Atresia in the NICU Set-Up: Two Case Reports and a Literature Review |
| title_full_unstemmed | Tetralogy of Fallot: The Burden of Pulmonary Atresia in the NICU Set-Up: Two Case Reports and a Literature Review |
| title_short | Tetralogy of Fallot: The Burden of Pulmonary Atresia in the NICU Set-Up: Two Case Reports and a Literature Review |
| title_sort | tetralogy of fallot the burden of pulmonary atresia in the nicu set up two case reports and a literature review |
| topic | tetralogy of Fallot pulmonary atresia right ventricular outflow tract obstruction congenital heart disease mechanical ventilation hemodynamics |
| url | https://www.mdpi.com/2227-9067/12/6/780 |
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