PubMed · 16181891
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Eugenio Neri. 2005. Invited commentary.. https://doi.org/10.1016/j.athoracsur.2004.12.016
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OBJECTIVE: To review the presentation and natural history of children with vascular rings and present management guidelines. METHODS: Retrospective study of tertiary care pediatric medical center charts from 1991 to 2002. RESULTS: There were 37 males and 27 females with a diagnosis of vascular rings. At presentation, 91% of patients had airway symptoms and 47% had esophageal symptoms. Airway symptoms included stridor (63%), recurrent respiratory infections (47%), respiratory distress (19%), and cough (17%). The most common esophageal symptom was dysphagia (27%). Pre-operative studies included: echocardiography (96%), chest X-ray (93%), barium swallow (75%), magnetic resonance imaging (MRI) (60%), and computerized tomography (CT) scan of the chest (59%). Surgical management included open (n=25) and thoracoscopic (n=39) approach. Complications included recurrent laryngeal nerve injury in five patients (8%). CONCLUSION: Children with vascular rings present with respiratory and/or feeding difficulty. The evaluation should include chest X-ray, echocardiography, and barium swallow. Direct laryngoscopy and bronchoscopy are recommended to assess the degree of compression of the airway and/or esophagus, tracheomalacia, and vocal fold motion prior to intervention. Indication for surgical release is given when the diagnosis is made and can be assisted by advanced radiology studies. Surgical options include minimally invasive techniques involving either thoracoscopic or robotic-assisted repairs, as well as open procedures involving thoracotomy.
OBJECTIVES: Double aortic arch is a common form of complete vascular ring, encircling both the trachea and esophagus, resulting in noncardiac morbidity. We sought to describe management and outcomes in a large single institution clinical series. PATIENTS AND METHODS: We reviewed the medical charts of all of the patients diagnosed with double aortic arch at age <18 years. Data regarding clinical presentation, anatomy, management, and outcomes were abstracted. RESULTS: We identified 81 patients (67% males) born between 1964 and 2005 and presenting at a median age of 5 months (range: birth to 10.3 years). Respiratory symptoms were present in 91%, including stridor in 77%. Gastrointestinal symptoms were present in 40%, with choking with feeds being most common. The dominant branch of the double aortic arch was right in 72%. Associated cardiac anomalies were present in 18%, with noncardiac anomalies in 7%. Repair was performed in 79 patients at a median age of 6 months. There were 2 deaths after surgical repair with no late deaths, with Kaplan-Meier survival estimates of 96% at 5 years. Postoperative complications included chylothorax in 9%. Only 1 patient required reoperation. The most common symptoms at most recent follow-up were respiratory (54%) followed by gastrointestinal symptoms (6%). Postoperative tracheal stenosis was documented in 14%, with tracheomalacia in 7%. There were no late reoperations and no evidence of aortic arch obstruction. CONCLUSIONS: Outcomes are excellent after repair of double aortic arch, although persistent respiratory symptoms are frequent and probably associated with previous compression-related maldevelopment of the trachea and major airways.
New generation multislice CT technology has changed the approach to non-invasive assessment of congenital heart disease, in both paediatric and adult patients. This is mainly because of rapid advances in spatial and temporal resolution and in post-processing capability. At Hôpital Necker-Enfants Malades, CT with multiplanar and three-dimensional reconstruction has become a routine examination in the evaluation of congenital heart disease planning surgery, complex interventional catheterisations and for follow-up. It has proved to be an invaluable diagnostic and decision-aiding methodology in these situations, as a complement to echocardiography and, increasingly, as a substitute for diagnostic angiography (which is usually associated with higher-dose radiation and longer sedation times, as well as occasional morbidity). This review illustrates the current status of 64-slice CT in congenital heart diseases, including assessment of the aorta, the coronary arteries, the pulmonary arteries, the systemic and pulmonary veins, and other intra- and extracardiac malformations.