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Vascular rings and slings: long-term follow-up of pulmonary function
Insights
Surgical repair of vascular rings and slings effectively resolves severe respiratory and swallowing issues in children. However, some patients may experience persistent airway obstruction, requiring further evaluation.
Area of Science:
- Pediatric Surgery
- Cardiovascular Surgery
- Thoracic Surgery
Background:
- Vascular anomalies of the aortic arch and pulmonary artery can cause significant respiratory and swallowing symptoms in children.
- These anomalies, including double aortic arch, right aortic arch with left ligamentum arteriosum, anomalous innominate artery, and pulmonary artery sling, require surgical intervention.
Purpose of the Study:
- To evaluate the safety and efficacy of surgical repair for symptomatic aortic arch and pulmonary artery anomalies in pediatric patients.
- To assess the long-term respiratory outcomes and the presence of central airway obstruction after surgical correction.
Main Methods:
- A retrospective review of 54 pediatric patients who underwent surgery for symptomatic vascular anomalies between 1968 and 1983.
- Diagnosis was confirmed using chest roentgenogram, bronchoscopy, barium esophagram, and arteriography.
- Follow-up included clinical assessment and pulmonary function testing in a subset of patients.
Main Results:
- Surgical repair demonstrated a low mortality rate (one postoperative death) and immediate symptomatic relief in all survivors.
- Long-term follow-up revealed complete resolution of severe symptoms in most patients, although mild respiratory issues persisted in some.
- Pulmonary function tests in asymptomatic patients indicated central airway obstruction in 9 out of 17 individuals.
Conclusions:
- Surgical repair of vascular rings and slings is a safe and effective treatment for symptomatic pediatric patients.
- Despite successful symptom resolution, a significant proportion of patients may have persistent anatomic tracheal or bronchial distortion, as evidenced by pulmonary function studies.
Abstract:
Between 1968 and 1983, 54 patients underwent surgery for symptomatic aortic arch and pulmonary artery anomalies at St Christopher's Hospital for Children. Presenting symptoms included stridor, wheeze, apnea, recurrent pulmonary infections, or dysphagia. Diagnosis was established with chest roentgenogram, bronchoscopy, barium esophagram, and arteriography. Four types of vascular anomalies were encountered; double aortic arch (24 patients), right aortic arch with left ligamentum arteriosum (17 patients), anomalous innominate artery (10 patients), and pulmonary artery sling (three patients). There were no intraoperative deaths and only one postoperative death. All surviving patients had immediate relief of their severe respiratory or swallowing symptoms. Mild respiratory symptoms persisted postoperatively from 3 months to 4 years and included frequent or severe upper respiratory infections, persistent cough, stridor and pneumonia. Five of the 53 surviving patients were lost to follow up. The remaining 48 patients were followed from 6 months to 14 years and all but one patient noted complete resolution of all respiratory symptoms. Twenty-nine patients at follow up were old enough to undergo pulmonary function testing including vital capacity, functional residual capacity, and inspiratory and expiratory flow volume loops. Seventeen of these 29 asymptomatic patients consented to these studies, and nine of these patients had abnormal flow volume loops indicative of significant central airway obstruction; the other eight studies were normal. We conclude that surgical repair for vascular rings and slings is safe and symptomatically efficacious. However, anatomic tracheal or bronchial distortion persists in a significant number of these patients as evaluated by pulmonary function studies.