Abstract Introduction Recurrent pulmonary hemorrhage in patients with complex congenital heart disease and pulmonary hypertension can be a treatment refractory condition with increased morbidity and mortality. Pulmonary hemorrhage is frequently driven by aortopulmonary collaterals. Although inhaled tranexamic acid (TXA) has been used successfully for short-term inpatient treatment of hemotypsis, there is a lack of data regarding outpatient use. We present a case of a patient with complex congenital heart disease and recurrent aorticopulmonary collaterals with limited surgical treatment options who benefited from TXA use at home. Case Presentation The patient is an 18-year-old male with double outlet right ventricle, ventricular septal defect, status post pulmonary artery band and Blalock-Taussig-Thomas shunt, pulmonary hypertension and recurrent pulmonary hemorrhage secondary to aortopulmonary collaterals despite multiple embolization attempts. Pulmonary hypertension is treated with macitentan, selexipag, and sildenafil. He also has chronic lung disease and chronic respiratory failure with tracheostomy and ventilator dependence. Home ventilator settings were PIP 19 cmH2O, PEEP 8 cmH2O, rate 20, inspiratory time 1.2 seconds, and 5 L/min oxygen bleed-in, targeting saturations of 75-85%.During his recent admission, he had bilateral opacities increased on the right (Figure 1) concerning for pulmonary hemorrhage and/or infection. Empiric antibiotics were initiated, and inhaled TXA was started, resulting in prompt resolution of bleeding. PEEP was increased to 10cmH2O. Attempts to wean TXA led to recurrent hemoptysis. He was not a candidate for further aorticopulmonary collateral embolization due to high aorticopulmonary collateral burden. Furthermore, previous coiling attempts did not offer symptom resolution. He was sent home on inhaled TXA every 6 hours as a palliative measure. He was ultimately weaned at home to once a day. He remains clinically stable without recurrent bleeding.There have been anecdotal communications with other pulmonary centers regarding outpatient TXA use for recurrent hemoptysis, but no cases have been reported in the literature, especially in pediatric patients. Discussion Our case demonstrates that inhaled TXA can be used in the outpatient setting for recurrent hemoptysis due to aorticopulmonary collaterals especially when standard interventions are no longer feasible. More studies need to be done on home TXA use to evaluate the long-term efficacy and safety. Figure 1 Conclusions Long-term outpatient inhaled TXA may represent a viable option for palliative control of recurrent pulmonary hemorrhage in patients with advanced cardiopulmonary disease. This case highlights the need for more research regarding the practicality, tolerability, and sustained benefit of outpatient nebulized TXA administration in such patients. This abstract is funded by: none
Tayliakh et al. (Fri,) studied this question.