Congenital Pulmonary Vein Stenosis

Encouraging Mid-term Outcome

Pradeepkumar Charlagorla, David Becerra, Parth M. Patel, Mark Hoyer, Robert K. Darragh

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

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 %.

Original languageEnglish (US)
Pages (from-to)125-130
Number of pages6
JournalPediatric Cardiology
Volume37
Issue number1
DOIs
StatePublished - Jan 1 2016

Fingerprint

Survival
Mortality
Balloon Angioplasty
Catheterization
Dilatation
Survival Rate
Retrospective Studies
Pulmonary Vein Stenosis
Therapeutics

Keywords

  • Balloon angioplasty
  • Congenital pulmonary vein stenosis
  • Marsupialization
  • Pulmonary hypertension
  • Surgical dilation

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Pediatrics, Perinatology, and Child Health

Cite this

Congenital Pulmonary Vein Stenosis : Encouraging Mid-term Outcome. / Charlagorla, Pradeepkumar; Becerra, David; Patel, Parth M.; Hoyer, Mark; Darragh, Robert K.

In: Pediatric Cardiology, Vol. 37, No. 1, 01.01.2016, p. 125-130.

Research output: Contribution to journalArticle

Charlagorla, P, Becerra, D, Patel, PM, Hoyer, M & Darragh, RK 2016, 'Congenital Pulmonary Vein Stenosis: Encouraging Mid-term Outcome', Pediatric Cardiology, vol. 37, no. 1, pp. 125-130. https://doi.org/10.1007/s00246-015-1249-7
Charlagorla, Pradeepkumar ; Becerra, David ; Patel, Parth M. ; Hoyer, Mark ; Darragh, Robert K. / Congenital Pulmonary Vein Stenosis : Encouraging Mid-term Outcome. In: Pediatric Cardiology. 2016 ; Vol. 37, No. 1. pp. 125-130.
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