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

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.

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.

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.

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%).