Thirty-Year Prevalence of Spontaneous Hemoptysis in Palliated Single Ventricle Circulation.

BACKGROUND

Spontaneous hemoptysis is a well-recognized risk of palliated single ventricle circulation, yet published literature is limited to case reports and small case series. In this study, we sought to determine the long-term prevalence of spontaneous hemoptysis among patients with palliated single ventricle circulation at a single institution. Secondarily, we sought to characterize the clinical outcomes after spontaneous hemoptysis.

METHODS

We conducted a retrospective study of patients with a history of Glenn or Fontan palliation seen from January 1, 1990 to January 21, 2023. Episodes of spontaneous hemoptysis were identified through 2 independent database screens that queried International Classification of Diseases, Ninth Revision (ICD-9) and Tenth Revision (ICD-10) codes and electronic medical records for "pulmonary hemorrhage" and "hemoptysis." Positive screens were subsequently confirmed or reclassified by manual chart review.

RESULTS

Of 799 patients with palliated single ventricle circulation, 10.9% (87/799) screened positive for hemoptysis. Following verification with manual chart review, 3.4% (27/799) of patients with palliated single ventricle circulation had spontaneous hemoptysis. A total of 61 episodes of spontaneous hemoptysis occurred in 27 patients. Of all hemoptysis episodes, 83.6% (51/61) occurred after Fontan palliation. Of patients with hemoptysis, 51.9% (14/27) had multiple episodes of hemoptysis with recurrence at a median of 0.6 years (range 1 day-8.5 years) after the first episode.

CONCLUSIONS

To our knowledge, this is the first study that quantifies prevalence of hemoptysis in a large cohort of patients with palliated single ventricle circulation. Overall, prevalence of spontaneous hemoptysis was low (3.4%) among patients with palliated single ventricle circulation and there was a moderate rate of recurrence (51.9%).