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Complete cartilage-ring tracheal stenosis associated with anomalous left pulmonary artery: the ring-sling complex
Insights
Infants with pulmonary artery sling often have high mortality due to coexisting long-segment tracheal stenosis. This "ring-sling complex" highlights the critical tracheal malformation requiring further research for effective treatment.
Area of Science:
- Pediatric Cardiology
- Thoracic Surgery
- Medical Imaging
Background:
- Pulmonary artery sling, a rare congenital anomaly, is associated with significant infant mortality.
- High mortality rates in these infants are often linked to concurrent airway malformations.
Observation:
- This study reports on five infants with pulmonary artery sling and long-segment tracheal stenosis.
- Airway imaging revealed low carina, horizontal mainstem bronchi, and extensive tracheal stenosis unrelated to the sling's length.
Findings:
- The primary cause of mortality is the coexisting long-segment tracheal stenosis, not solely the pulmonary artery sling.
- A new term, "ring-sling complex," is proposed to emphasize the tracheal defect.
Implications:
- Early and accurate diagnosis of the tracheal malformation is crucial for managing infants with pulmonary artery sling.
- The "ring-sling complex" underscores the need for improved diagnostic and therapeutic strategies for this condition.
Abstract:
The persistent high mortality in infants with pulmonary artery "sling" (retrotracheal anomalous left pulmonary artery) is primarily due to the coexistence in such patients of long-segment tracheal stenosis due to complete cartilage rings. Five such patients are reported. Airway studies (by filtered high-kV radiography, bronchography, and/or CT) showed low carina, horizontal equal-length right and left mainstem bronchi, and long-segment tracheal stenosis. The length of the stenosis far exceeded the contact with the pulmonary sling. The suggested term "ring-sling complex" for such patients correctly places emphasis on detection of the tracheal malformation, which currently has no satisfactory surgical treatment.