Surgical repair of a child with tetralogy of Fallot with absent left pulmonary artery: a case report

Introduction and importance: Tetralogy of Fallot (TOF) with unilateral absence of a pulmonary artery is a rare anomaly with challenging surgical management. Key risks include postoperative pulmonary hypertension due to a reduced pulmonary vascular bed. This report presents a case of TOF with absent left pulmonary artery (ALPA) that resulted in a fatal postoperative complication, highlighting a potentially underreported mechanism. Case presentation: A 5-year-old female with Tetralogy of Fallot with absent left pulmonary artery (TOF-ALPA) underwent successful surgical repair, including ventricular septal defect closure, right ventricular outflow tract augmentation, and patent ductus arteriosus (PDA) ligation with metallic clips. Her initial recovery was uneventful. However, on postoperative Day 4, she developed sudden massive hemoptysis and cardiac arrest, resulting in death despite prolonged resuscitation. Clinical discussion: The fatal hemoptysis was likely caused by a rupture of the solitary right pulmonary artery or a bronchovascular fistula. This is attributed to increased hemodynamic stress on the single artery post-repair and potential erosion from metallic PDA ligation clips. The event occurred despite the absence of pulmonary hypertension or coagulopathy, pointing towards structural or surgical factors. Conclusion: This case highlights the significant risk of lethal vascular complications in TOF-ALPA patients, even after technically successful surgery. It uniquely underscores the potential for catastrophic vascular erosion from metallic ligation clips in the context of a solitary pulmonary artery. This outcome calls for caution in the surgical approach, especially regarding materials used near fragile vasculature, and emphasizes the need for vigilant postoperative monitoring for delayed complications in this high-risk population.