Averin K et al. – This study evaluated the acute and mid–term outcomes following presentation with, and treatment for, life–threatening airway bleeding in palliated single ventricle congenital heart disease (SV–CHD). The study demonstrated that bronchoscopic evaluation and transcatheter intervention is safe and may contribute to low mortality at mid–term follow–up in Fontan patients, despite the potentially life–threatening nature of hemoptysis in patients with SV–CHD. Hemoptysis in superior cavopulmonary anastomosis (SCPA) patients may indicate a poor prognosis. Recurrent hemoptysis is common in this patient population.

Methods

• Case series of patients with SV–CHD with episodes of hemoptysis between 2004 and 2015.

Results

• A total of 21 episodes of hemoptysis occurred in 12 patients.
• First episode of hemoptysis occurred after Fontan completion in 8 patients, after SCPA in 3 patients and in 1 shunt–dependent patient.
• Bronchoscopy was performed in conjunction with catheterization in 67% of initial catheterizations.
• In 95% of bronchoscopy cases, a specific anatomic source of airway bleeding was identified and was uniformly distributed in all lobar segments.
• Transcatheter intervention with systemic–to–pulmonary collateral artery occlusion was performed in 28/30 catheterizations.
• No procedural complications other than increased airway bleeding during interventional bronchoscopy (37%) were observed.
• The median length of stay in the hospital was 9.0 (3.5, 14.5) days with patients undergoing 1.0 (1.0, 2.0) catheterizations per episode of hemoptysis.
• In all, 2 SCPA patients did not survive to discharge.
• During a median follow–up of 32.5 months, freedom from mortality was 75%; all 3 deaths occurred in the SCPA group by 4 months post hemoptysis.
• Recurrent hemoptysis was observed in 60% of patients