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Objective: Neonates with tetralogy of Fallot and pulmonary atresia (TOF/PA) but no major aorta-pulmonary collaterals are dependent on the arterial duct for pulmonary blood flow and require early intervention, either by primary (PR) or staged repair (SR) with initial palliation (IP) followed by complete repair (CR). The optimal approach has not been established.
Methods: Neonates with TOF/PA who underwent PR or SR were retrospectively reviewed from the Congenital Cardiac Research Collaborative. Outcomes were compared between PR and SR (IP + CR) strategies. Propensity scoring was used to adjust for baseline differences. The primary outcome was mortality. Secondary outcomes included complications, length of stay, cardiopulmonary bypass and anesthesia times, reintervention (RI), and pulmonary artery (PA) growth.
Results: Of 282 neonates, 106 underwent PR and 176 underwent SR (IP: 144 surgical, 32 transcatheter). Patients who underwent SR were more likely to have DiGeorge syndrome and greater rates of mechanical ventilation before the initial intervention. Mortality was not significantly different. Duration of mechanical ventilation, inotrope use, and complication rates were similar. Cumulative length of stay, cardiopulmonary bypass, and anesthesia times favored PR (P ≤ .001). Early RI was more common in patients who underwent SR (rate ratio, 1.42; P = .003) but was similar after CR (P = .837). Conduit size at the time of CR was larger with SR. Right PA growth was greater with PR.
Conclusions: In neonates with TOF/PA, SR is more common in greater-risk patients. Accounting for this, SR and PR strategies have similar mortality. Perioperative morbidities, RI, and right PA growth generally favor PR, whereas SR allows for larger initial conduit implantation.
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http://dx.doi.org/10.1016/j.jtcvs.2023.01.008 | DOI Listing |
Heart Lung Circ
September 2025
Queensland Children's Hospital, Brisbane, Qld, Australia. Electronic address:
JACC Case Rep
September 2025
Seattle Children's Hospital, University of Washington, Seattle, Washington, USA.
Objective: To demonstrate a detailed procedural guide for right ventricular outflow tract (RVOT) stent placement for palliation of tetralogy of Fallot and pulmonary stenosis in a patient weighing <2 kg.
Key Steps: Obtain wire position with an 0.018-inch Hi-Torque floppy wire in a distal branch pulmonary artery.
The Spigelian hernia-cryptorchidism syndrome is a rare clinical entity observed in male neonates. It is characterized by a congenital defect in the Spigelian fascia, resulting in a hernia along the semilunar line, often associated with intestinal obstruction. A hallmark feature is the presence of an ectopic testis, typically located within or immediately adjacent to the hernia sac.
View Article and Find Full Text PDFAnn Pediatr Cardiol
July 2025
Postgraduate Program of Public Health, Universitas Muhammadiyah Aceh, Banda Aceh, Indonesia.
Background: Tetralogy of Fallot (TOF), the most prevalent cyanotic congenital heart anomaly, impacts around 3.9 in 10,000 live births. Repair aims to address intracardiac shunting and right ventricular outflow tract obstruction.
View Article and Find Full Text PDFJACC Case Rep
August 2025
Pediatric Cardiology, Children's Hospital of Richmond at VCU, Richmond, Virginia, USA.
Background: The balance of pulmonary and systemic blood flow is the fundamental paradigm that influences decision-making in cardiovascular shunt physiology.
Case Summary: This case discusses a term neonate with a prenatal diagnosis of 22q11.2 deletion syndrome and tetralogy of Fallot with near pulmonary atresia, a confluent but severely hypoplastic main pulmonary artery and right pulmonary artery, an isolated left pulmonary artery from the aorta, and major aortopulmonary collateral arteries.