Asian Cardiovasc Thorac Ann 2000;8:344-346
© 2000 Asia Publishing EXchange Pte Ltd
Surgical Correction of Vascular Ring Anomalies
Sanjeev Sharma, MD,
Jeri L Dobbs, PA,
Adnan Cobanoglu, MD
Oregon Health Sciences University
Portland, Oregon, USA
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For reprint information contact: Sanjeev Sharma, MD Tel: 1 520 626 6339 Fax: 1 520 626 4042 Department of Cardiothoracic Surgery, The University of Arizona Health Sciences Center, 1501 N. Campbell Ave, Tucson, AZ 85724-5071, USA.
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Abstract
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Between 1981 and 1997, 25 patients underwent operations for relief of tracheoesophageal compression due to vascular rings. Seventeen patients (68%) had a double aortic arch, 6 (24%) had a right aortic arch with an anomalous left subclavian artery and ligamentum arteriosum, and 2 (8%) had a left aortic arch with an anomalous right subclavian artery arising from a Kommerell's diverticulum. Preoperative symptoms consisted of stridor and dysphagia. Four patients (16%) were ventilator-dependent prior to surgery. After division of the vascular ring, wide dissection of the superior mediastinal structures was performed to prevent any compression of the trachea or esophagus. There were no early deaths. Ventilatory support was necessary for a mean of 48 ± 8 hours. One patient required reintubation for 24 hours due to persistent left lower lobe atelectasis. During late follow-up, 2 patients (8%) had recurrent pneumonia, and there were 2 deaths. Surgical repair of vascular rings provided excellent early and late results. The index of suspicion of these aortic arch anomalies should increase if an infant or young child presents with a history of recurrent stridor, dysphagia, or respiratory distress that is not easily explained.
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Introduction
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The first anatomical description of vascular rings by identifying a double aortic arch, is attributed to Hommel in 1737.1 The first clinicopathological description of a vascular ring is attributed to Bayford who in 1794, described a left aortic arch and anomalous right subclavian artery.2 In 1945, Gross3 performed the first division of a vascular ring by dividing the anterior arch in a symptomatic infant with double aortic arch. Much has been written regarding the anomalous pathways of vascular rings, but it is clear that patients' symptoms are caused by compression of the trachea and/or esophagus.4 These "tracheoesophageal compressive syndromes" may be categorized into 4 groups: double aortic arch (DAA); right aortic arch (RAA) with anomalous retroesophageal left subclavian artery; left aortic arch (LAA) with arch vessel anomalies; and pulmonary artery sling.5 These anomalies represent complete and incomplete vascular rings, as described by van Son and colleagues.6 The early and late surgical outcomes of 25 patients who underwent division of vascular rings were reviewed retrospectively.
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Patients and Methods
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From 1981 to 1997, 25 patients underwent surgery for relief of tracheoesophageal compression due to complete and incomplete vascular rings. Pulmonary artery slings were excluded from this study. Seventeen patients (68%) had DAA, 6 (24%) had RAA with an anomalous retroesophageal left subclavian artery and left ligamentum arteriosum, and 2 (8%) had LAA with an anomalous retroesophageal right subclavian artery arising from a Kommerell's diverticulum. Age at operation ranged from 1 week to 36 years (median, 4 ± 18 months). Age at operation was significantly lower in patients with complete versus incomplete rings (Table 1
). Weight at operation ranged from 2 to 74 kg (median, 6 ± 3 kg).
Twenty-four patients (96%) had symptoms of dysphagia, 12 (48%) presented with failure to thrive as evidenced by poor weight gain, 20 (80%) presented with stridor, and 4 (16%) required preoperative intubation. Diagnosis was confirmed by barium swallow in 80%, by aortography in 88%, by computed tomography (CT) in 20%, and by magnetic resonance imaging (MRI) in 20%. Echocardio-graphy was used in all cases to assess intracardiac pathology. Five patients (20%) had significant additional congenital anomalies: tetralogy of Fallot (2); single-ventricle physiology (1); coarctation of the aorta and bi-cuspid aortic valve (1); and ventricular septal defect (1).
The surgical approach used to divide all vascular rings was a left posterolateral thoracotomy. In patients with DAA, the smaller anterior aortic arch was divided and oversewn between the left subclavian artery and the larger posterior aortic arch. The ligamentum arteriosum was divided and oversewn in patients with RAA and anomalous left subclavian artery. Mobilization and division of any mediastinal fibrotic tissue was performed to divide these vascular rings. Blood supply was maintained to all arch vessels in these cases. The 2 patients with incomplete vascular rings, LAA, and an anomalous right subclavian artery arising from a Kommerell's diverticulum, also had aneurysmal dilatation at the origin of the right subclavian artery. They underwent division and oversewing of the descending aorta and right subclavian artery.
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Results
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There were no early deaths. Ventilatory support ranged from 24 to 192 hours (mean, 48 ± 8 hours). One patient required reintubation for an additional 24 hours due to persistent left lower lobe atelectasis. One patient had a superficial wound infection that was treated with antibiotics and dressing changes. Length of hospital stay ranged from 3 to 16 days (mean, 7 ± 2 days). Late follow-up was complete for all patients. Follow-up was achieved through the cardiology clinic or by contacting the patient's parents by telephone. The range of follow-up was 5 months to 17 years (mean, 8 ± 1 years). There were 2 late deaths (8%): one occurred due to myocardial infarction in a patient with congenital bicuspid aortic valve stenosis; and the other was a sudden death in a patient with single-ventricle physiology and pulmonary hypertension. There were no recurrences of symptoms during follow-up. During late follow-up, 2 patients (8%) had recurrent pneumonia due to underlying pulmonary hypertension.
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Discussion
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DAA was the most common anomaly in our patients, followed by RAA with anomalous left subclavian artery, and LAA with anomalous right subclavian artery. The ages at onset of symptoms and operation were significantly earlier in patients with complete vascular rings (DAA and RAA) versus incomplete vascular rings (LAA). These results are similar to other reported surgical series.6–8
There is ongoing controversy in the treatment of vascular rings regarding the choice of imaging modality. Excellent schematic drawings and diagnostic barium esophagograms have been presented elsewhere.5–7 Invasive (aortography) and expensive (CT and MRI scans) studies were performed in these patients prior to surgical consultation, which may have been helpful in preparing an operative plan. However, we believe that a chest radiograph and a barium esopha-gogram are extremely reliable diagnostic studies for vascular rings. We agree with the diagnostic evaluation algorithm presented by Backer and colleagues.7 Diagnostic evaluation should begin with a chest radiograph, followed by a barium esophagogram. No further study need be pursued if a vascular ring is clearly identified. CT, MRI, or aortography should be carried out if the barium esophagogram is nondiagnostic or presents an unusual anatomic configuration. We would add transthoracic echocardiography to other noninvasive tests (CT and MRI) to identify a vascular ring and any intracardiac pathology.
Low morbidity and mortality were seen in our patients as well as previously reported surgical series.6–10 Early and late outcomes were directly related to the presence of associated congenital heart disease. There were no early deaths in this series and postoperative morbidity was due mainly to pneumonia, secondary to tracheomalacia.6,7 To avoid postoperative pneumonia, a strict regimen of pulmonary toilet was instituted with frequent nasotracheal suctioning and chest percussive therapy following extubation. By following this protocol, there were no episodes of early postoperative pneumonia, and only one patient required reintubation for persistent left lower lobe atelectasis and tachypnea.
The left posterolateral thoracotomy provided excellent exposure of the aorta, arch vessels, left pulmonary artery, and superior mediastinal structures. Blood flow was maintained to all arch vessels, except in the 2 patients with LAA and anomalous right subclavian artery. Both of these patients had aneurysmal dilatation of the origin of the right subclavian artery, due to the presence of a Kommerell's diverticulum that is not an actual diverticu-lum but the remnant of the distal end of the right aortic arch.11 These patients underwent division and oversewing of the descending aorta and the right subclavian artery. We believe that reimplantation of the right subclavian artery is unnecessary since ligation of a subclavian artery is well tolerated.12,13
This experience confirms that the surgical management of vascular rings is effective and associated with low morbidity and mortality.6–10 The challenge is to make the correct diagnosis in cases of this rare congenital aortic arch anomaly. There should be a high index of suspicion on the part of primary care physicians who see infants or young children with recurrent stridor, dysphagia, or respiratory distress.
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References
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Turner WH. On the irregularities of the pulmonary artery, arch of the aorta, and the primary branches of the arch, with an attempt to illustrate their mode of origin by a reference to development. Br For Med Chir Rev 1862; 30:173–89.
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Turner WH. On the irregularities of the pulmonary artery, arch of the aorta, and the primary branches of the arch, with an attempt to illustrate their mode of origin by a reference to development. Br For Med Chir Rev 1862; 30:462–83.
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Gross RE. Surgical relief for tracheal obstruction from a vascular ring. N Engl J Med 1945;233:586–90.
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van Son JAM, Julsrud PR, Hagler DJ, Sim EKW, Pairolero PC, Puga FJ, et al. Surgical treatment of vascular rings: the Mayo Clinic experience. Mayo Clin Proc 1993; 68:1056–63.[Medline]
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Abstract
Introduction
