From the Research
Management of hemoptysis in patients with Tetralogy of Fallot (TOF) and pulmonary atresia with Major Aortopulmonary Collateral Arteries (MAPCAs) requires a multidisciplinary approach focusing on both immediate stabilization and definitive treatment. Initial management should include airway protection, hemodynamic stabilization, and correction of any coagulopathy. For acute episodes, administer tranexamic acid (1g IV followed by 1g over 8 hours) to help control bleeding, as supported by general medical principles 1. Oxygen supplementation should be provided to maintain saturation above 92%. If bleeding is severe, consider selective bronchial intubation to protect the non-bleeding lung. Diagnostic bronchoscopy can help localize the bleeding source.
Definitive Treatment
For definitive treatment, cardiac catheterization with selective angiography is essential to identify the culprit MAPCAs, which can then be embolized using coils or vascular plugs. Surgical options include unifocalization of MAPCAs with repair of TOF if the patient is a candidate, as discussed in 2 and 1. Recurrent hemoptysis may require pulmonary artery rehabilitation with balloon dilation and stenting of stenotic segments.
Preventive Measures
Preventive measures include avoiding anticoagulants when possible, treating pulmonary infections promptly, and maintaining adequate hydration. These patients require lifelong cardiology follow-up with regular imaging to monitor MAPCA development and pulmonary vascular status, as hemoptysis in this population typically results from high-pressure collateral vessels causing pulmonary hemorrhage or from bronchial artery hypertrophy secondary to chronic cyanosis, as noted in 3 and 1.
Key Considerations
Key considerations in managing these patients involve understanding the complex anatomy and physiology of TOF with pulmonary atresia and MAPCAs, as outlined in 3 and 4, and tailoring the treatment approach to the individual patient's needs, taking into account the most recent and highest quality evidence available, such as the study from 1.