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

Abstract

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%).

Keywords: hemoptysis; single ventricle circulation.

Figure 1. Cohort screening and validation.

Flow diagram of the cohort identification process.

Figure 2. Kaplan–Meier analysis showing overall survival after first episode of spontaneous hemoptysis.

Overall survival of 79.4 (61.2–92.9)% at 10 years after the first episode of hemoptysis. Dotted lines represent 95% CI.

Figure 3. Kaplan–Meier analysis comparing overall survival and transplant‐free survival after Glenn palliation in patients with or without history of spontaneous hemoptysis.

A, Comparison of overall survival and (B) transplant‐free survival. There was no difference in overall survival (P=0.882) or transplant‐free survival (P=0.920) between the cohorts with and without hemoptysis. Dotted lines represent 95% CI.

Figure 4. Kaplan–Meier analysis showing freedom from hemoptysis after Fontan palliation.

Patients were included in the post‐Fontan analysis if their first episode of hemoptysis occurred with Fontan circulation (n=21). Patients were excluded if they had a previous episode of spontaneous hemoptysis post Glenn (n=6). Of those with hemoptysis post Fontan (n=21), hemoptysis occurred at a median of 8.1 years after Fontan (range 0.1–18.2 years). Dotted lines represent 95% CI.

Figure 5. Kaplan–Meier analysis showing freedom from recurrence of hemoptysis in the hemoptysis cohort.

Of patients with hemoptysis (n=27), 51.9% (14/27) had multiple episodes of hemoptysis and recurrence occurred at a median of 0.6 year (range 1 d–8.5 years) after the first episode. At 5 years after the first episode of hemoptysis, 50.1% (29.7%–70.5%) of the hemoptysis cohort remained free from recurrence. Dotted lines represent 95% CI.