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Area of Science:

  • Pediatric surgery
  • Congenital cardiovascular and respiratory anomalies
  • Thoracic surgery

Background:

  • Pulmonary artery sling (PAS) is frequently diagnosed with long-segment congenital tracheal stenosis (LSCTS).
  • This combination may also involve lung hypoplasia, aplasia, or agenesis.
  • The impact of lung hypoplasia on surgical outcomes for combined PAS and LSCTS is not well-defined.

Purpose of the Study:

  • To analyze the association between lung hypoplasia abnormalities and combined PAS and LSCTS.
  • To determine the influence of lung hypoplasia on surgical outcomes in pediatric patients with PAS and LSCTS.

Main Methods:

  • Retrospective analysis of pediatric patients (0-18 years) who underwent surgery for both PAS and LSCTS between 1995 and 2019.
  • Patients were categorized into two groups: those with normal lungs and those with hypoplastic lungs.
  • Outcomes assessed included mortality, ventilation days, and intensive care unit (ICU) days.

Main Results:

  • Of 75 patients, 8 (10.7%) had hypoplastic right lung (hypoplasia, aplasia).
  • Mortality was significantly higher in the hypoplastic lung group (50%) compared to the normal lung group (9.0%; P = .007).
  • No significant differences were observed in median ventilation or ICU days between the groups.

Conclusions:

  • Lung hypoplasia associated with PAS and LSCTS is typically right-sided.
  • Severe airway obstruction and single-lung physiology necessitate significant preoperative cardiorespiratory support.
  • Lung hypoplasia is significantly associated with adverse surgical outcomes in patients with combined PAS and LSCTS.