Objective To describe acute and mid-term outcomes following presentation with, and treatment for, life-threatening airway bleeding (hemoptysis) in palliated single ventricle congenital heart disease (SV-CHD).

Methods Case series of patients with SV-CHD who presented to a large congenital heart centre with hemoptysis between 2004 and 2015.

Results Twenty-one episodes of hemoptysis occurred in 12 patients (58% female, median 10.5 (IQR 7.2, 16.4) years). First hemoptysis episode occurred after Fontan completion (n=8), after superior cavopulmonary anastomosis (SCPA, n=3) and in one shunt-dependent patient. Bronchoscopy was performed in conjunction with catheterisation in 14/21 (67%) initial catheterisations. A specific anatomic source of airway bleeding was identified in 95% of bronchoscopy cases and was uniformly distributed in all lobar segments. Transcatheter intervention with systemic-to-pulmonary collateral artery (SPC) occlusion was performed in 28/30 catheterisations. Apart from increased airway bleeding during interventional bronchoscopy (37%), there were no procedural complications. Median hospital length of stay was 9.0 (3.5, 14.5) days with patients undergoing 1.0 (1.0,2.0) catheterisations per episode of hemoptysis. Two SCPA patients did not survive to discharge. During a median follow-up of 32.5 (12.5, 87.5) months, freedom from mortality was 75%, with all three deaths occurring in the SCPA group by 4 months posthemoptysis. Recurrent hemoptysis occurred in 60% of patients.

Conclusions Despite the potentially life-threatening nature of hemoptysis in patients with SV-CHD, a policy of bronchoscopic evaluation and transcatheter treatment is safe and may contribute to low mortality at mid-term follow-up in Fontan patients. Hemoptysis in SCPA patients may portend a poor prognosis. Recurrent hemoptysis is common.