Diagnosis of the Double Aortic Arch and Its Differentiation from the Conotruncal Malformations

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Abstract

Purpose

The clinical and radiological characteristics of the double aortic arch (DAA) and its differentiation from conotruncal malformations (CTM) were reported in order to familiarize pediatric practitioners with these congenital heart diseases.

Materials and Methods

From July 1994 to December 2006, a total of 6 patients (4 male and 2 female, aged 16 days to 6.5 years) with DAA were enrolled in this retrospective study. The study modalities included chart recordings, plain chest radiographs, barium esophagograms, echocardiograms, cardiac catheterization, cardiac angiograms, surgery, magnetic resonance imaging, and chromosome analysis. Patients with incomplete vascular rings or with right aortic arches and left ligamentum were excluded. In addition, the clinical and radiological profiles of 38 patients with CTM, including dextro-transposition of the great arteries (d-TGA) (n = 28), hemitruncus arteriosus (HTA) (n = 3), type I truncus arteriosus (TA) (n = 4), and the aortopulmonary window (APW) (n = 3), were comparatively reviewed.

Results

All 6 patients with DAA presented with postprandial choking and respiratory distress that prompted their initial visit to the hospital. One of the 6 patients presented with congestive heart failure, and none with cyanosis. Esophagograms showed indentations in 5 patients with DAA. All patients with d-TGA presented with cyanosis and heart failure, while patients with HTA, type I TA, and APW manifested overt heart failure. Suprasternal and subcostal approaches of the echocardiography may offer diagnositic windows for DAA. As for CTM, parasternal and subcostal approaches could always determine the causality. Cardiac catheterization with angiography comprehensively delineated the pathology.

Conclusion

In case of postprandial choking and respiratory distress in neonates and infants, barium esophagograms can indicate the presence of DAA. Diagnosis of DAA and its differentiation from the CTM can be achieved by echocardiography, angiography, or magnetic resonance imaging.

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Most cited references 30

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Trends in vascular ring surgery.

Constantine Mavroudis, Cynthia Rigsby, Lauren D. Holinger … (2005)

We sought to review our experience with infants and children with anatomically complete vascular rings (ie, double aortic arch and right aortic arch with left ligamentum) and define perioperative trends in diagnostic imaging, operative techniques, and clinical outcomes. From 1946 through 2003, 209 patients (113 with double aortic arch and 96 with right aortic arch) underwent surgical repair. Mean and median ages at the time of the operation were as follows: double aortic arch, 1.4 +/- 2.4 years and 0.75 years, respectively; right aortic arch, 2.7 +/- 3.9 years and 0.9 years, respectively. Fourteen (14.6%) patients with right aortic arch had an associated Kommerell diverticulum. Cardiac diagnoses were present in 26 (12.4%) of 209 patients. There has been no operative mortality since 1959. In the past 30 years, mean hospital stay decreased from 8 to 3 days. Primary means of diagnosis has shifted from barium swallow and angiography to computed tomographic scanning or magnetic resonance imaging. In the past 10 years, 73% of patients had preoperative or intraoperative bronchoscopy. The technique of operation has shifted to a muscle-sparing left thoracotomy without routine chest drainage. In 7 recent patients with right aortic arch and a Kommerell diverticulum, the diverticulum was resected, and the left subclavian artery was transferred to the left carotid artery as a primary procedure. At our institution, computed tomographic scanning has replaced barium swallow as the diagnostic procedure of choice for vascular ring evaluation. We recommend both preoperative bronchoscopy and echocardiography. Use of a muscle-sparing thoracotomy without routine chest drainage has decreased mean hospital stay. For patients with a right aortic arch and associated Kommerell diverticulum, we recommend diverticulum resection with left subclavian artery transfer to the left carotid artery.

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Management and outcomes of double aortic arch in 81 patients.

Khalfan Alsenaidi, Rebecca Gurofsky, Tara Karamlou … (2006)

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. 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. 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. 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.

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Vascular anomalies causing tracheoesophageal compression. Review of experience in children.

Salim F Idriss, Lorraine Backer, S DeLeon … (1989)

Two hundred four infants and children (mean age 13 months) have undergone operation for the relief of tracheoesophageal obstruction resulting from vascular anomalies. One hundred thirteen patients had complete vascular rings (group I), 61 with double aortic arch and 52 with right aortic arch with a left ligamentum. Nine patients had a pulmonary artery sling (group II), 71 had innominate artery compression (group III), and 11 had miscellaneous anomalies (group IV). Patients were admitted with respiratory distress, stridor, apnea, dysphagia, or recurrent respiratory infections. Diagnosis was established by barium esophagogram in group I; barium esophagogram, bronchoscopy, and computed tomography or angiography in group II; bronchoscopy in group III; and barium esophagogram or angiography in group IV. The operative approach was through a left thoracotomy in group I, II and IV (93% of these patients) and through a right thoracotomy for group III (96% of these patients). The operative mortality rate was 4.9% and there were seven late deaths (3.4%). There have been no operative deaths in patients with isolated vascular anomalies in the past 28 years. Follow-up data from 1 month to 20 years (mean 8.5 months) were available on 159 patients; 141 (92%) were essentially free of symptoms, and 12 (8%) had residual respiratory problems. Five of six patients in group II having a lung scan postoperatively had a patent left pulmonary artery. A strong index of suspicion is necessary to avoid the complications of vascular rings in children. Barium swallow is the best single diagnostic technique for patients with complete vascular rings. A bronchoscopic study is required to diagnose innominate artery compression. Angiograms or computed tomographic scans are used to confirm the diagnosis of pulmonary artery sling. Left thoracotomy provides excellent exposure for all vascular rings except the displaced innominate artery, for which a right thoracotomy is the best approach.

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Author and article information

Journal

Journal ID (nlm-ta): Yonsei Med J

Journal ID (publisher-id): YMJ

Title: Yonsei Medical Journal

Publisher: Yonsei University College of Medicine

ISSN (Print): 0513-5796

ISSN (Electronic): 1976-2437

Publication date (Print): 31 October 2007

Publication date (Electronic): 31 October 2007

Volume: 48

Issue: 5

Pages: 818-826

Affiliations

Department of Pediatrics, Division of Pediatric Cardiology and Director of Pediatric Intensive Care Unit, Changhua Christian Hospital, Changhua 50050, Taiwan.

Author notes

Reprint address: requests to Dr. Meng-Luen Lee, Department of Pediatrics, Division of Pediatric Cardiology, Changhua Christian Hospital, No. 135, Nanhsiao St., Changhua 50050, Taiwan. Tel: 886-4-7238595 (1902), Fax: 886-4-7238847, ferdielee@ 123456yahoo.com

Article

DOI: 10.3349/ymj.2007.48.5.818

PMC ID: 2628149

PubMed ID: 17963340

SO-VID: 467a4904-460d-4286-825b-f35fef45ef49

License:

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Diagnosis of the Double Aortic Arch and Its Differentiation from the Conotruncal Malformations

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Abstract

Purpose

Materials and Methods

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Jaypee UN SDG 03

Most cited references 30

Trends in vascular ring surgery.

Management and outcomes of double aortic arch in 81 patients.

Vascular anomalies causing tracheoesophageal compression. Review of experience in children.

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