Pulmonary Artery Sling with Tracheal Stenosis Combined with A Large Ventricular Septal Defect in A Two-Month-Old Boy: A Case Report

Pulmonary artery sling (PAS) is a rare congenital cardiovascular abnormality. In typical PAS patients, the left pulmonary artery (LPA) arises from the right pulmonary artery and passes between the trachea and esophagus, which possibly causes tracheal stenosis and some respiratory symptoms. PAS typically associates with other cardiovascular anomalies, which may cause difficulties to the treatment of patients. This report described a rare case of a two-month-old boy with PAS, VSD, and tracheal stenosis simultaneously and underwent procedures without tracheoplasty.

A Delayed Anatomic Diagnosis and Management Challenge in an Initially Asymptomatic Infant With Type II Pulmonary Artery Sling: A Case Report

Pulmonary artery sling (PAS) is a rare but fatal malformation. Patients with PAS tend to develop obstructive symptoms in few weeks of life. Conversely, some patients may be otherwise mild or asymptomatic in their early life. Currently, no consensus on the intervention timing and treatment strategy for asymptomatic and mild cases has been reached. Moreover, the extent of tracheal stenosis is another determining factor for the choice of intervention timing since clinical symptoms might not correspond well with the degree of stenosis. Lack of comprehensive assessment of entire airways confer underestimation of disease severity and in turn improper choice of treatment regimens and poor outcomes. Herein, we described an infantile case of PAS, who was scheduled initially for periodic outpatient follow-up on account of the absence of symptoms and inadequate imaging assessment at diagnosis. The patient developed recurrent wheezing and progressive respiratory distress at 7 months of age. After left pulmonary artery (LPA) reimplantation without tracheal intervention, bronchoscopy was performed due to failure to wean from mechanical ventilation, which demonstrated complete tracheal cartilage rings, a long segment tracheal stenosis, a low tracheal bifurcation at T6, and the absence of a separate right middle lobe bronchus. The patient was finally diagnosed with type IIb PAS and extubated successfully following conservative treatment. Miserably, neurological sequelae were devastating, leading to poor outcomes. Comprehensive airway evaluation using bronchoscopy is substantial to early identification of all components responsible for airway compromise in PAS anatomic subtypes. Considering severe concomitant maldevelopment of the bronchial tree in children with type IIb PAS, early and complete correction by surgery might decrease perioperative morbidities and mortalities of these patients.

Cardiovascular causes of tracheobronchial compression: a decade experience in a Paediatric Congenital Heart Centre

Background: Vascular compression of the airway often complicates CHD management. This study evaluated the use of CT in determining cardiovascular causes, clinical manifestations, and outcome of tracheobronchial compression among children with CHD. Methods: A retrospective review of clinical records of all patients with CT scan evidence of tracheobronchial compression from January 2007 to December 2017 at National Heart Institute. Cardiovascular causes of tracheobronchial compression were divided into three groups; group I: vascular ring/pulmonary artery sling, II: abnormally enlarged or malposition cardiovascular structure due to CHD, III: post-CHD surgery. Results: Vascular tracheobronchial compression was found in 81 out of 810 (10%) patients who underwent CT scan. Group I lesions were the leading causes of vascular tracheobronchial compression (55.5%), followed by group II (34.6%) and group III (9.9%). The median age of diagnosis in groups I, II, and III were 16.8 months, 3 months, and 15.6 months, respectively. Half of group I patients are manifested with stridor and one-third with recurrent chest infections. Persistent respiratory symptoms, lung atelectasis, or prolonged respiratory support requirement were clues in groups II and III. Higher morbidity and mortality in younger infants with severe obstructive airway symptoms, associated airway abnormalities, and underlying complex cyanotic CHD. Conclusions: Vascular ring/pulmonary artery sling and abnormally enlarged or malposition cardiovascular structure were the leading causes of cardiovascular airway compression. A high index of suspicion is needed for early detection due to its non-specific presentation. The outcome often depends on the severity of airway obstruction and complexity of cardiac lesions.