Congenital Pulmonary Vein Stenosis: Encouraging Mid-term Outcome

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• 作者：Pradeepkumar Charlagorla ; David Becerra ; Parth M. Patel ; Mark Hoyer…
• 关键词：Congenital pulmonary vein stenosis ; Balloon angioplasty ; Surgical dilation ; Marsupialization ; Pulmonary hypertension
• 刊名：Pediatric Cardiology
• 出版年：2016
• 出版时间：January 2016
• 年：2016
• 卷：37
• 期：1
• 页码：125-130
• 全文大小：578 KB
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• 作者单位：Pradeepkumar Charlagorla (1)
  David Becerra (2)
  Parth M. Patel (2)
  Mark Hoyer (1)
  Robert K. Darragh (1)
  
  1. Division of Pediatric Cardiology, Riley Hospital for Children, Indiana University, 705, Riley Hospital Drive, Indianapolis, IN, 46032, USA
  2. Indiana University School of Medicine, Indianapolis, IN, 46032, USA
  
• 刊物类别：Medicine
• 刊物主题：Medicine & Public Health
  Cardiology
  Cardiac Surgery
  Vascular Surgery
  
• 出版者：Springer New York
• ISSN：1432-1971

文摘

Congenital pulmonary vein stenosis (PVS) is a rare entity with limited outcome literature. Multiple interventional approaches have evolved including surgical and catheterization techniques. Our objective is to report our center experience and to compare short-term and mid-term outcomes among these therapeutic modalities. Retrospective study on 23 patients (n = 23) with PVS that required intervention over the last 13 years (2000–2013). Patients were divided into three groups based on type of initial intervention. Of these, 10 (43.5 %) had balloon angioplasty, 3 (13.0 %) had surgical dilation, and 10 (43.5 %) had surgical marsupialization. Mortality and number of re-interventions were our primary outcomes. Mean age at diagnosis was 10.9 ± 18.4 months. Mean age at initial intervention was 14.5 ± 18.0 months. Mean pre- and post-initial intervention PVS gradients were 9.2 ± 3.4 and 3.4 ± 2.2 mmHg, respectively. Mean survival time and re-intervention-free survival time were 4.8 ± 4.0 and 2.8 ± 3.4 years. No statistical significance was found between the interventions with respect to survival time (p = 0.52) and re-intervention free time (p = 0.78). High initial pre- and post-intervention gradients were significantly associated with re-intervention-free survival (p = 0.01 and p = 0.03, respectively). Patients with bilateral disease have increased mortality (p = 0.01) and decreased 5-year survival (p = 0.009) compared to patients with unilateral disease irrespective of type of intervention. No statistically significant difference in mortality or re-intervention rate was present among these different therapeutic modalities. This study has the longest follow-up so far reported in the current literature (58 months) with overall survival of 78 %. Keywords Congenital pulmonary vein stenosis Balloon angioplasty Surgical dilation Marsupialization Pulmonary hypertension