Major haemoptysis in children with cystic fibrosis: a 20-year retrospective study.

BACKGROUND: Major haemoptysis occurs in approximately 1% of children with cystic fibrosis (CF). This report describes management and follow-up of these children at a tertiary centre in Australia.

METHODS: Retrospective review of medical records from 1980-1999.

RESULTS: Fifty-one children (45% female) had major haemoptysis (102 episodes). Mean age at first episode was 15 years (range 7-19) and mean FEV(1) was 56% predicted (range 14-98). Massive life-threatening haemoptysis was not confined to those with severe lung disease (FEV1 < 50% predicted). Bronchial artery embolisation (BAE) was more likely to be the initial treatment for those with massive haemoptysis and chronic recurrent bleeding tended to be treated conservatively (P = 0.01). Overall, 52 BAE were performed in 28 children with an immediate success rate of 98%; 13 children (46%) had repeated BAE. Four patients died as a direct result of severe haemoptysis. Mean follow-up was 54 months (range 0.5-183). Median survival time (Kaplan-Meier estimate) after first haemoptysis was 70 months, with a significantly longer survival for male patients independent of age (RR 3.8; 95% CI 1.7-8.8; P = 0.001). Median survival time following initial treatment with BAE was longer (103 months) compared to conservative treatment (52 months, P = 0.09).

CONCLUSIONS: Massive haemoptysis was unrelated to the severity of lung disease and was more likely to be treated with embolisation. BAE was highly effective, however, 46% of the children required re-embolisation at some time, which is similar to the recurrence risk for major hemoptysis treated conservatively on longer term follow-up.