Congenital Heart Surgery Nomenclature and Database Project: vascular rings, tracheal stenosis, pectus excavatum

doi:10.1016/S0003-4975(99)01279-5

The Annals of Thoracic Surgery

Volume 69, Issue 3, Supplement 1, March 2000, Pages 308-318

Presented at the International Nomenclature and Database Conferences for Pediatric Cardiac Surgery, 1998–1999.

https://doi.org/10.1016/S0003-4975(99)01279-5 Get rights and content

Abstract

The extant nomenclature for vascular rings, tracheal stenosis, and pectus deformities is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. Vascular rings are subclassified as double aortic arch, right arch/left ligamentum, pulmonary artery sling, and innominate compression. Tracheal stenosis is subclassified as congenital complete tracheal rings (localized or long-segment) or acquired postintubation types. Pectus deformities are subclassified as pectus excavatum and carinatum (mild, moderate, severe). A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Section snippets

Background

Vascular rings are congenital anomalies of the aortic arch system where vascular structures encircle and compress the trachea and esophagus. In anatomic terms, there are two types of vascular rings; those that are complete and have vascular structures completely encircling the trachea and esophagus, and rings that are incomplete and only compress a portion of the esophagus or trachea without encircling it as a complete ring. These two types of tracheoesophageal compression anomalies present

Analysis: vascular rings

The phrase vascular ring was first used by Robert Gross in his report describing the first successful division of a double aortic arch in 1945 [1]. Since that time, the phrase vascular ring has been used to refer to a group of congenital vascular anomalies that encircle and compress the esophagus and trachea. In that original article by Gross, he described the two classic true anatomic vascular rings, double aortic arch and right aortic arch with a left ligamentum. The other two

Vascular ring hierarchy level 2

Vascular ring, NOS

Vascular ring, Double aortic arch
Vascular ring, Right aortic arch/left ligamentum
Vascular ring, Innominate artery compression
Vascular ring, PA sling
Vascular ring, Other

Double aortic arch

There are two distinct aortic arches (right and left) encircling the trachea and esophagus. They may be patent or atretic.

Right arch/left ligamentum

The aortic arch is to the right of the trachea. The ligamentum extends from the MPA to the descending thoracic aorta completing the vascular ring.

Innominate compression

The innominate artery compresses the trachea anteriorly by more than 75% of the original tracheal lumen.

PA sling

The LPA originates from the RPA. The LPA courses posteriorly between the trachea and esophagus in its route to the left lung

Vascular ring hierarchy level 3

Vascular ring, NOS
Vascular ring, Double aortic arch, NOS

Vascular ring, Double aortic arch, Balanced arches
Vascular ring, Double aortic arch, Left arch dominant
Vascular ring, Double aortic arch, Right arch dominant

Vascular ring, Right aortic arch/left ligamentum, NOS

Vascular ring, Right aortic arch/left ligamentum, Mirror image branching
Vascular ring, Right aortic arch/left ligamentum, Retroesophageal left subclavian artery (aberrant left subclavian artery)
Vascular ring, Right aortic arch/left

Vascular ring hierarchy level 3 definitions

Left arch = apex of arch to left of trachea.
Right arch = apex of arch to right of trachea.
Dominant arch = larger vessel of the two arches.
Balanced arches = arches of equal size.

Vascular ring hierarchy level 4

Vascular ring, NOS
Vascular ring, Double aortic arch, NOS
Vascular ring, Double aortic arch, Balanced arches
Vascular ring, Double aortic arch, Left arch dominant, NOS

Vascular ring, Double aortic arch, Left arch dominant, Right arch atretic
Vascular ring, Double aortic arch, Left arch dominant, Right arch patent

Vascular ring, Double aortic arch, Right arch dominant, NOS

Vascular ring, Double aortic arch, Right arch dominant, Left arch atretic
Vascular ring, Double aortic arch, Right arch dominant,

Vascular ring modifiers

Vascular ring modifier, Double aortic arch, Coarctation left arch
Vascular ring modifier, Double aortic arch, Coarctation right arch
Vascular ring modifier, Double aortic arch, Left ligamentum
Vascular ring modifier, Double aortic arch, Right ligamentum
Vascular ring modifier, Double aortic arch, Left patent ductus arteriosus
Vascular ring modifier, Double aortic arch, Right patent ductus arteriosus
Vascular ring modifier, Right aortic arch/left ligamentum, Kommerell’s diverticulum
Vascular ring

Additional vascular ring modifier comments

Oftentimes a double aortic arch will be associated with a ligamentum arteriosum, which in some rare cases can be a patent ductus arteriosus. The double aortic arch modifiers therefore include left ligamentum, right ligamentum, left patent ductus arteriosus, and right patent ductus arteriosus. Alternatively, the patent ductus arteriosus can be included in the database as a second diagnosis. In a similar fashion, the ligamentum in a patient with a right aortic arch and left ligamentum may in some

Rare vascular rings

Vascular ring, Left aortic arch/aberrant right subclavian artery 11, 17
Vascular ring, Left aortic arch/aberrant right subclavian artery-aneurysm base right subclavian artery
Vascular ring, Left cervical aortic arch [18]
Vascular ring, Right cervical aortic arch
Vascular ring, Left aortic arch/right descending aorta, right ligamentum [19]
Vascular ring, Left aortic arch/right descending aorta, right patent ductus arteriosus [20]
Vascular ring, Ductus arteriosus sling [21]
Vascular ring, Hemitruncal

Vascular ring treatment hierarchy level 1

Vascular ring repair

Vascular ring treatment hierarchy level 2

Vascular ring repair, NOS

Vascular ring repair, Double aortic arch
Vascular ring repair, Right aortic arch/left ligamentum
Vascular ring repair, Innominate artery compression
Vascular ring repair, PA sling
Vascular ring repair, Other

Vascular ring treatment hierarchy level 3

Vascular ring repair, NOS
Vascular ring repair, Double aortic arch, NOS

Vascular ring repair, Double aortic arch, Right arch ligation and division
Vascular ring repair, Double aortic arch, Right arch division and oversewing
Vascular ring repair, Double

Vascular ring repair modifiers

Vascular ring repair modifier, Location of arch division, Between ascending aorta/left arch
Vascular ring repair modifier, Location of arch division, Between ascending aorta/right arch
Vascular ring repair modifier, Location of arch division, Between left carotid/left subclavian
Vascular ring repair modifier, Location of arch division, Between left subclavian/descending aorta
Vascular ring repair modifier, Location of arch division, Between right carotid/right subclavian
Vascular ring repair

Analysis tracheal stenosis

Tracheal stenosis in infants and children is most often secondary to a congenital anomaly commonly known as complete tracheal rings. When a child has a complete tracheal ring there is absence of the normal posterior membranous trachea and the cartilage ring is circumferential and complete. The number of complete rings in any one patient can range from 1 to over 20 (essentially, the entire trachea from cricoid to carina). The rings can also extend into the right or left main bronchus. In

Tracheal stenosis hierarchy level 2

Tracheal stenosis, NOS

Tracheal stenosis, Congenital-complete tracheal rings
Tracheal stenosis, Post intubation
Tracheal stenosis, Traumatic
Tracheal stenosis, Congenital web
Tracheal stenosis, Other

Tracheal stenosis hierarchy level 3

Tracheal stenosis, NOS
Tracheal stenosis, Congenital-complete tracheal rings, NOS

Tracheal stenosis, Congenital-complete tracheal rings, Localized (less than 50% tracheal length)
Tracheal stenosis, Congenital-complete tracheal rings, Long-segment (more than 50% of tracheal length)

Tracheal stenosis, Post intubation, NOS

Tracheal stenosis, Post intubation, Localized (less than 50% tracheal length)
Tracheal stenosis, Post intubation, Long-segment (more than 50% of tracheal length)

Tracheal stenosis,

Tracheal stenosis modifiers

Tracheal right upper lobe (RUL)
PA sling
Intracardiac anomaly
Tracheomalacia
Bridging bronchus [34]
Number of complete rings
Subglottic stenosis
Bronchial stenosis
Bronchomalacia

Tracheal stenosis treatment hierarchy level 1

Tracheal procedure

Tracheal stenosis treatment hierarchy level 2

Tracheal procedure, NOS

Tracheal procedure, Resection with end-to-end anastomosis
Tracheal procedure, Pericardial tracheoplasty
Tracheal procedure, Rib cartilage tracheoplasty
Tracheal procedure, Slide tracheoplasty
Tracheal procedure, Tracheal autograft reconstruction
Tracheal procedure, Tracheal homograft reconstruction
Tracheal procedure, Homograft (aortic or PA) reconstruction
Tracheal procedure, Tracheoplasty (Other)
Tracheal procedure, Balloon dilatation
Tracheal procedure, Stent

Potential diagnostic related risk factors for tracheal stenosis

Granulation tissue

Residual/recurrent tracheal stenosis

Tracheo/bronchomalacia

Air leak → pneumomediastinum

Mediastinitis

Pulmonary hypertension

Respiratory failure requiring prolonged ventilation

Analysis pectus excavatum

Pectus excavatum is a relatively commonly occurring chestwall deformity in children. It is a depression of the sternum that commonly starts at the angle of Louis, and is deepest at the xiphisternal junction. Pectus carinatum is a protrusion of the sternum, and is approximately one-tenth as common as pectus excavatum. A severe pectus excavatum deformity can cause cardiopulmonary insufficiency from the compression of the right atrium and right ventricle and diminished vital capacity of the lungs.

Pectus treatment hierarchy level 1

Pectus excavatum repair
Pectus carinatum repair

Pectus treatment hierarchy level 2

Pectus excavatum repair, NOS

Pectus excavatum repair, Ravitch repair, with metal strut
Pectus excavatum repair, Ravitch repair, without metal strut
Pectus excavatum repair, Sternal turnover
Pectus excavatum repair, Costoplasty (unilateral)
Pectus excavatum repair, Nuss minimally invasive technique
Pectus excavatum repair, Silicone implant reconstruction

Pectus carinatum repair, NOS

Pectus carinatum repair, Ravitch repair, with metal strut
Pectus carinatum

Potential diagnostic related risk factors for pectus

Mediastinitis

Bar migration

Cardiac injury

Pleural effusion

Subcutaneous fluid accumulation

Pectus recurrence

Unstable sternum

Pectus reoperation

Diagnosis and procedure short list

Diagnosis Short List
Vascular ring
PA sling
Tracheal stenosis
Tracheal disease, Other
Pectus

Procedure Short List
Vascular ring repair
PA sling repair
Tracheal procedure
Pectus repair

Vascular rings

Incidence of the types of vascular rings by year, by age at presentation and symptoms.
Types of operations that are performed for each subset of vascular ring by year which include double aortic arch, right arch/left ligamentum, innominate compression, PA sling, and other categories.
Types of double aortic arch by year. Operative approach and type of operation by year. Mortality and complication rate by year. Length of hospital stay by year. Long-term outcome defined as the length of time that

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Cited by (116)

Incidental finding of double aortic arch and tract from noncoronary sinus to left atrium: A case report
2024, Radiology Case Reports
The double aortic arch with vascular ring is a rare but documented aortic arch variant, traditionally presenting with difficulty swallowing or breathing due to extrinsic compression. Tracts from the noncoronary sinus to left atrium are very rare, with limited case reports to compare against. We report an incidental finding of double aortic arch in an elderly woman who underwent a cerebral angiogram for symptoms of a right-sided stroke, with a further anomaly identified on subsequent CT gated aortogram of a possible tract between the non-coronary sinus and left atrium. It is worth noting that the aortic arch abnormality was missed on previous plain radiographs, which can happen even among experienced radiologists. This case illustrates the need for a thorough, systematic approach to interpreting chest radiographs to avoid missing mediastinal lesions, such as aortic abnormalities.
Evaluation of the trachea in fetuses with double aortic arch using prenatal ultrasound: a retrospective cohort study
2023, American Journal of Obstetrics and Gynecology MFM
Double aortic arch is the most common form of complete vascular ring. The trachea and/or esophagus could be compressed by the complete vascular ring, which may lead to early respiratory and/or esophageal symptoms in children with double aortic arch. Accurate prenatal assessment of tracheal compression could provide relevant information for perinatal clinical management of double aortic arch and emergency treatment of infants with double aortic arch. The fetal trachea is filled with amniotic fluid and can be clearly visualized with prenatal ultrasound. Previous studies reported the use of prenatal ultrasound to measure the tracheal internal diameters in normal fetuses and showed a linear correlation between the fetal tracheal internal diameters and gestational age. However, to the best of our knowledge, few studies have quantitatively evaluated tracheal compression in fetuses with double aortic arch using ultrasound.
This study aimed to evaluate the tracheal compression caused by the vascular ring in fetuses with double aortic arch using prenatal ultrasound and to analyze the relationship between tracheal compression and postnatal clinical symptoms.
The data of fetuses with double aortic arch diagnosed with prenatal ultrasound at 2 institutions from January 2011 to April 2021 were retrospectively analyzed. Singleton pregnancies with normal fetuses as the control group were prospectively recruited. The tracheal compression—evaluated by comparing the tracheal internal diameter z scores against the gestational age—was assessed in fetuses with double aortic arch and in normal fetuses. The live-born infants with double aortic arch were divided into symptomatic and asymptomatic groups for the comparison of z scores. The receiver operating characteristic curve for the tracheal internal diameter z score cutoffs and prediction of symptomatic infants with double aortic arch was plotted. Intraobserver and interobserver agreements were investigated.
A total of 26 fetuses with double aortic arch were diagnosed, and 14 fetuses (53.8%) with double aortic arch were delivered alive. Among the 14 live-born infants, 7 (50.0%) were symptomatic, whereas 7 (50.0%) were asymptomatic. The tracheal internal diameter z scores were significantly lower in the double aortic arch group than in the normal groups (−0.62±1.36 vs 0.00±0.78; P<.001). The tracheal internal diameter z scores were significantly lower in the symptomatic group than in the asymptomatic group (−1.42±0.92 vs −0.49±0.96; P=.018). The area under the curve was 0.878 (95% confidence interval, 0.689–1.000). Using a tracheal internal diameter z scores cutoff of −1.21, the sensitivity was 71%, and the specificity was close to 100%. The intraclass correlation coefficients of interobserver and intraobserver agreements were 0.987 (95% confidence interval, 0.980–0.992) and 0.975 (95% confidence interval, 0.955–0.987), respectively.
The clinical symptoms in infants with double aortic arch were associated with prenatal tracheal compression, which can be prenatally evaluated using ultrasound. If fetuses are diagnosed with double aortic arch, prenatal surveillance of the tracheal internal diameters and comparison with z score reference ranges could provide pertinent information that would aid perinatal clinical management.
Don't go barking up the wrong tree: Thinking beyond infection in a toddler with stridor
2022, American Journal of Emergency Medicine
A toddler presented to the emergency department with persistent stridor and barky cough for 4 weeks and progressive dysphagia for 1 week. During this time, he had sought medical attention 6 additional times and had been treated for pneumonia, wheezing and croup, receiving antibiotics and several courses of steroids without improvement. On the final presentation, airway imaging did not reveal a foreign body. However, bedside laryngoscopy demonstrated bilateral vocal cord paralysis. Further imaging revealed an intracranial posterior fossa mass which provided the unifying diagnosis for his persistent symptoms.
Vascular rings
2021, Seminars in Pediatric Surgery
Vascular rings are congenital aortic arch anomalies that lead to compression of the trachea or esophagus. The goal of this review is to summarize our current recommendations for the management of patients with a diagnosis of a vascular ring. We review the history, classification methods, and epidemiology of the various types of vascular rings. We then propose a management strategy for the relatively new paradigm of fetal diagnosis, including the management of asymptomatic vascular rings. Finally, we finish with a review of the operative techniques and outcomes for the four main categories of vascular rings.
You’re surrounded: Vascular ring/sling
2021, Pediatric Imaging for the Emergency Provider
Vascular rings in pediatric patients present in a variety of ways, but pulmonary (cough, wheezing, persistent respiratory infections) and gastrointestinal (dysphagia and gastroesophageal reflux) symptoms are most commonly encountered. Plain radiography is the first-line imaging modality in patients who present with persistent respiratory symptoms and is a first-line imaging modality. Ultrasonography has been utilized to evaluate for the presence of cardiovascular abnormalities, including vascular rings, in the prenatal period and in children, but becomes less helpful in evaluation of vascular anomalies with increasing age. Historically, barium esophagography has been the traditional imaging modality when plain radiography is suspicious for a vascular ring, but advanced imaging techniques, including computed tomography angiography and magnetic resonance imaging angiography, are now utilized for more direct visualization of vascular anatomy.
Great vessel anomalies and their impact on the surgical treatment of tracheobronchomalacia
2020, Journal of Pediatric Surgery
Citation Excerpt :
Fig. 1a–e] Therefore, the classification of a double aortic arch was subdivided into a right dominant arch, a left dominant arch, or balanced arch; a right aortic arch was further separated into patients with a right aortic arch and retroesophageal left subclavian artery, a right arch with mirror-branching pattern, or a right arch with a circumflex aorta. Other great vessel anomalies that were captured included an aberrant right subclavian artery, innominate artery compression, and/or pulmonary sling. [17] Other variations in great vessel anatomy, including an aberrant insertion of the carotid or vertebral arteries, were identified under an “other” category.
Tracheobronchial compression (TBC) from great vessel anomalies (GVA) can contribute to tracheobronchomalacia (TBM) symptoms. The frequency, impact on symptoms and optimal management of GVA in these patients, with or without a history of esophageal atresia (EA), are still unclear.
Patients who underwent surgery for TBM/ TBC between 2001 and 2017 were reviewed. Demographics, type of GVA, and operative interventions were collected. The frequency and treatment modalities of GVA between EA and non-EA patients were compared.
Overall, 209 patients met criteria; 120 (57.4%) patients had at least one GVA, including double aortic arches (n = 4, 1.9%), right aortic arches (n = 14, 6.7%), aberrant right subclavian arteries (n = 15, 7.2%), and innominate artery compression (n = 71, 34.0%). Non-EA patients were more likely to have surgery later in life (29.5 months versus 16 months, p = 0.0002), double aortic arch (p = 0.0174), right aortic arch (p < 0.0001), and undergo vascular reconstruction concurrently with their airway procedure (25% vs 8.4%, p = 0.002). Vessel reconstruction was performed in 25 patients; six required cardiac bypass.
The frequency of GVA in patients with symptomatic airway collapse is substantial. Multidisciplinary evaluation is imperative for operative planning as many require complex reconstruction and collaboration with cardiac surgery, particularly patients without a history of EA.
Level III.

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Mavroudis SupplementCongenital Heart Surgery Nomenclature and Database Project: vascular rings, tracheal stenosis, pectus excavatum

Abstract

Section snippets

Background

Analysis: vascular rings

Vascular ring hierarchy level 2

Double aortic arch

Right arch/left ligamentum

Innominate compression

PA sling

Vascular ring hierarchy level 3

Vascular ring hierarchy level 3 definitions

Vascular ring hierarchy level 4

Vascular ring modifiers

Additional vascular ring modifier comments

Rare vascular rings

Vascular ring treatment hierarchy level 1

Vascular ring treatment hierarchy level 2

Vascular ring treatment hierarchy level 3

Vascular ring repair modifiers

Analysis tracheal stenosis

Tracheal stenosis hierarchy level 2

Tracheal stenosis hierarchy level 3

Tracheal stenosis modifiers

Tracheal stenosis treatment hierarchy level 1

Tracheal stenosis treatment hierarchy level 2

Potential diagnostic related risk factors for tracheal stenosis

Analysis pectus excavatum

Pectus treatment hierarchy level 1

Pectus treatment hierarchy level 2

Potential diagnostic related risk factors for pectus

Diagnosis and procedure short list

Vascular rings

Med Clin North Am

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

J Pediatr Surg

J Thorac Cardiovasc Surg

Ann Thorac Surg

J Thorac Cardiovasc Surg

Am J Surg

J Pediatr Surg

J Thorac Cardiovasc Surg

Ann Thorac Surg

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

J Pediatr Surg

Ann Thorac Surg

J Pediatr Surg

Mavroudis Supplement
Congenital Heart Surgery Nomenclature and Database Project: vascular rings, tracheal stenosis, pectus excavatum