Bronchial Compression due to Dilated Branch Pulmonary Artery in Tetralogy of Fallot with Absent Pulmonary Valve

Tetralogy of Fallot with absent pulmonary valve syndrome (TOF-APVS) is a rare variant characterized by severe pulmonary regurgitation and aneurysmal dilatation of pulmonary arteries (PAs), often causing tracheobronchial compression. Airway compromise may dominate the clinical presentation, particularly in late-presenting patients. A 9-year-3-month-old girl with TOF-APVS presented with failure to thrive and recurrent respiratory infections due to severe left main bronchial compression from massively dilated PAs (main PA 55 mm). Complete repair included Nunn’s pulmonary valve reconstruction and reduction arterioplasty of the dilated PAs. Intraoperative bronchoscopy confirmed persistent narrowing of the left main bronchus, prompting reliance on the right lung for ventilation. Serial post-operative chest radiographs showed progressive left lung re-expansion, and echocardiography disclosed no residual stenosis, a right ventricular outflow tract gradient of 20 mmHg, and mild pulmonary regurgitation, with preserved biventricular function. Late-presenting TOF-APVS may be dominated by chronic airway compression, emphasizing the need for pre-operative computed tomography, intraoperative bronchoscopy, and structured post-operative respiratory follow-up.