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Background <p>Pulmonary vein stenosis (PVS) is a rare but serious condition in children, often requiring surgical or catheter-based interventions. The initial optimal treatment strategy remains unclear due to disease complexity, progression, and high rates of recurrence.</p> Methods <p>In this retrospective, single-center study, we identified children with primary or secondary PVS from a cardiac catheterization and surgical database between 2015 and 2023. Patients with single ventricle physiology were excluded. Demographics and outcomes were compared between patients who underwent catheter-based intervention only and those who underwent at least one surgical pulmonary vein repair, with or without subsequent catheter-based reintervention. Reintervention following surgical repair was assessed using Kaplan-Meier analysis.</p> Results <p>Among 56 children with biventricular physiology and PVS (33 males, 59%), 16 (29%) underwent at least one surgical repair at a median age of 9 months (IQR 4–20), while 40 (71%) were managed with catheter-based interventions alone. Surgical repair was more frequently performed in patients with bilateral or complex disease, particularly those without prematurity or with coexisting congenital heart defects requiring open-heart surgery. Over time, catheter-based approaches became increasingly preferred. Overall, 92% of surgical patients required reintervention, most within the first year. Mortality did not significantly differ between groups (<i>p</i> = 0.294). In the surgical group, elevated right ventricular/systemic systolic pressure ratio (HR: 1.25, <i>p</i> = 0.048) and the presence of scimitar syndrome (HR: 8.25, <i>p</i> = 0.011) were associated with increased mortality.</p> Conclusions <p>Surgical pulmonary vein repair remains an important option, particularly in cases where catheter-based intervention is not feasible due to anatomical challenges or when multiple pulmonary veins are severely affected. However, recurrent pulmonary vein re-intervention is common, regardless of whether the initial approach was surgical or catheter-based.</p>

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Surgical and Catheter-Based Intervention in Pediatric Pulmonary Vein Stenosis

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年4月17日

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  • Daiji Takajo,
  • Paul J. Critser,
  • Awais Ashfaq,
  • Russel Hirsch

摘要

Background

Pulmonary vein stenosis (PVS) is a rare but serious condition in children, often requiring surgical or catheter-based interventions. The initial optimal treatment strategy remains unclear due to disease complexity, progression, and high rates of recurrence.

Methods

In this retrospective, single-center study, we identified children with primary or secondary PVS from a cardiac catheterization and surgical database between 2015 and 2023. Patients with single ventricle physiology were excluded. Demographics and outcomes were compared between patients who underwent catheter-based intervention only and those who underwent at least one surgical pulmonary vein repair, with or without subsequent catheter-based reintervention. Reintervention following surgical repair was assessed using Kaplan-Meier analysis.

Results

Among 56 children with biventricular physiology and PVS (33 males, 59%), 16 (29%) underwent at least one surgical repair at a median age of 9 months (IQR 4–20), while 40 (71%) were managed with catheter-based interventions alone. Surgical repair was more frequently performed in patients with bilateral or complex disease, particularly those without prematurity or with coexisting congenital heart defects requiring open-heart surgery. Over time, catheter-based approaches became increasingly preferred. Overall, 92% of surgical patients required reintervention, most within the first year. Mortality did not significantly differ between groups (p = 0.294). In the surgical group, elevated right ventricular/systemic systolic pressure ratio (HR: 1.25, p = 0.048) and the presence of scimitar syndrome (HR: 8.25, p = 0.011) were associated with increased mortality.

Conclusions

Surgical pulmonary vein repair remains an important option, particularly in cases where catheter-based intervention is not feasible due to anatomical challenges or when multiple pulmonary veins are severely affected. However, recurrent pulmonary vein re-intervention is common, regardless of whether the initial approach was surgical or catheter-based.