Tetralogy of Fallot With Pulmonary Atresia/MAPCAs Complicated by Bronchial Ischemia and Pneumonectomy.

BACKGROUND: Bronchial ischemia after unifocalization in patients with tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (ToF/PA/MAPCAs) is a rare and serious postoperative complication. Progression to carinal dehiscence requiring pneumonectomy, and subsequent survival with a single lung, has not been previously reported. CASE SUMMARY: We describe a 4-month-old girl with 22q11.2 deletion and ToF/PA/MAPCAs who developed bronchial ischemia with carinal dehiscence after unifocalization surgery, ultimately requiring left pneumonectomy and slide tracheoplasty. Despite this, she underwent successful biventricular repair at 1 year of age and was discharged 17 months after her initial procedure. CONCLUSIONS: This case demonstrates that single-lung physiology can sustain adequate oxygenation and ventilation after biventricular repair in patients with ToF/PA/MAPCAs, while highlighting the critical importance of recognizing bronchial ischemia as a postoperative complication of unifocalization. TAKE-HOME MESSAGES: Bronchial ischemia should be recognized as a rare but critical postoperative complication of unifocalization surgery in patients with ToF/PA/MAPCAs with potential to progress to carinal dehiscence requiring pneumonectomy. The key considerations for managing single-lung physiology include permissive hypercapnia and protective lung strategies in the postoperative period and close surveillance assessing the need for pulmonary vasodilators due to the risk of pulmonary hypertension. Biventricular repair remains a feasible option in selected patients with ToF/PA/MAPCAs after pneumonectomy.