Cureus. 2025 Jul 28;17(7):e88928. doi: 10.7759/cureus.88928. eCollection 2025 Jul.
ABSTRACT
The concurrent presentation of Pierre-Robin sequence (PRS) and dextro-transposition of the great arteries (d-TGA) is extremely rare and presents distinct management challenges. We describe a term male neonate with the classic PRS triad (severe micrognathia, glossoptosis, and a U-shaped cleft palate) who developed profound cyanosis unresponsive to supplemental oxygen. Echocardiography demonstrated d-TGA with an intact ventricular septum, a restrictive patent foramen ovale, and a patent ductus arteriosus. Prone positioning and a nasopharyngeal airway were unsuccessful in improving oxygenation. On day 7 of life, tongue-lip adhesion relieved glossoptosis and increased arterial oxygen saturation to >94 %. Definitive arterial switch repair was performed on day 10 while the infant received prostaglandin E₁. Post-operative care involved coordinated input from cardiology, nephrology, and infectious-disease teams to manage transient renal dysfunction and ventilator-associated pneumonia, both of which resolved. The infant was discharged hemodynamically stable on day 33 and, at 15-month follow-up, exhibited normal neurodevelopment, a left-ventricular ejection fraction of 60%, and growth at the 75th percentile. This case supports an “airway-first, heart-early” strategy: prompt, reversible airway stabilization followed by timely arterial switch operation can achieve outcomes comparable to isolated d-TGA, even in resource-limited settings, when coupled with structured multidisciplinary care and vigilant postoperative surveillance.
PMID:40881541 | PMC:PMC12388853 | DOI:10.7759/cureus.88928
