Congenital heart disease

Systemic Sirolimus Therapy Is Associated With Reduced Intervention Frequency in Pulmonary Vein Stenosis

JACC AdvPublished Retrospective cohort study

Authors' conclusion

SST was associated with a reduction in frequency of PVS interventions.

Study context

Population
Patients with moderate to severe pediatric pulmonary vein stenosis (PVS) treated with systemic sirolimus therapy (SST) for ≥1 month, 2015–2022
Participants
45 patients
Exposure
On-SST vs pre-SST intervals
Outcome
PVS intervention rate (interventions/year)
Methodological detailspaired pre-SST vs on-SST intervals, generalized Poisson mixed models
  • Paired pre-SST vs on-SST intervals
  • Generalized Poisson mixed models

Main finding

As reported in the abstract

Median per-patient PVS intervention rate (interventions/year)

P < 0.001

Observational data: associations, not effects.

Further findings

OutcomeP value
Cumulative number of interventions over time (mean cumulative function)Increased cumulative number pre-SSTP < 0.001

Duration of SST and follow-up

Years

Median duration of SST
1.7
Median follow-up from SST initiation
2.7

Mortality · median 1.1 years (range, 4.4 months-6.5 years) after SST initiation

Deaths
6

Original publication

Systemic Sirolimus Therapy Is Associated With Reduced Intervention Frequency in Pulmonary Vein Stenosis. JACC Adv. Published 15 November 2024. doi:10.1016/j.jacadv.2024.101401

Composed by AI from the published abstract and checked against it. How Briefs are made →

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