Lung Hypoplasia Associated With Ring-Sling Complex Is Usually Right-Sided

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.