Vascular ring surgery – can we do better?

Abstract Background Vascular anomalies of the Aortic arch can cause respiratory symptoms in children due to tracheal compression. Treatment consists of division of the vascular rings, nevertheless data regarding mid- term results is scarce. The purpose of this study was to evaluate clinical results of vascular ring surgery. Methods Between2007–2014, 85 children underwent vascular ring surgery. 51 had Double Aortic Arch (DAA, 60%), 31 Right Arch with Aberrant Subclavian Artery (RAA & ALSA, 36.5%) and 3 had RAA, mirror image branching & left ductus arteriosus (3.5%). Mean age and weight at operation were 12.4±13months and 8.6±4.1 kg respectively. Mid-term follow-up included clinical follow up by a pulmonologist (38 patients, 44.7%) and a telephone questionnaire (71 patients, 83%) performed 57±25.7 months after surgery. Results In most patients, symptomatic relief occurred in less than 6 months (table 1). Mid –term follow up revealed that although most parents described a significant improvement in their child's respiratory symptoms (95%), a significant number of patients described some residual respiratory symptoms (table 2). We did not find any significant association between age at surgery (under 6 month), or vascular ring anatomy (DAA vs. RAA&ASA) and the presence of residual symptoms at follow up Conclusions Surgical division of vascular rings results in a significant clinical improvement within one year, nevertheless many patients remain symptomatic to some degree. We found no association between the age at surgery or anatomic variant to the presence of symptoms in mid- term follow up. Further evaluation whether a more aggressive surgical approach is warranted in order to decrease the incidence long-term symptoms. Funding Acknowledgement Type of funding source: None

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Mid-term results of vascular ring surgery

Cardiology in the Young ◽

10.1017/s1047951121004224 ◽

2021 ◽

pp. 1-6

Author(s):

Gabriel Amir ◽

Niv Soffair ◽

Elchanan Bruckheimer ◽

Elchanan Nachum ◽

Tommy Shoenfeld ◽

...

Keyword(s):

Respiratory Symptoms ◽

Respiratory Infections ◽

Vascular Ring ◽

Retrospective Chart Review ◽

Residual Symptoms ◽

Vascular Rings ◽

Anatomic Variant ◽

Age At Surgery ◽

Mid Term Results

Abstract Background: Vascular rings cause respiratory symptoms in children. Treatment consists of surgical division; however, data regarding mid-term results are scarce. The purpose of this study was to evaluate clinical results of vascular ring surgery. Methods: Retrospective chart review of consecutive patients who underwent vascular ring surgery. Mid-term follow-up consisted of clinic visits and telephone questionnaire over a 1-year period sampling at five points in time. Results: Follow-up of 85 patients who underwent vascular rings surgery revealed significant symptomatic improvement within 6 months. In total, 50% were symptomatic to some degree at their last documented clinic visit complaining of stridor (36.8%), cough (34.2%), wheezing (10.5%), dyspnoea on exertion (10.5%), or recurrent respiratory infections (23.7%). By telephone questionnaires, 83% described a significant improvement in respiratory symptoms, 63.4% had some residual symptoms, 36.6% stridor, 38.8% chronic cough, 32.4% bronchodilator use,18.3% had at least one episode of pneumonia per year, 31% of children had dyspnoea or respiratory limitation, and 12.7% while doing physical activities. We found no association between the age at surgery or anatomic variant to the presence of symptoms at mid-term follow-up. Conclusions: Surgical division of vascular rings results in significant clinical improvement within 1 year; nevertheless, some patients remain symptomatic to some degree. We found no association between the age at surgery or anatomic variant to the presence of symptoms at mid-term follow-up. Further evaluation is warranted to assess the nature of residual symptoms and explore whether anatomical causes can be identified leading to surgical modifications.

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Vascular ring presenting as dysphagia in an adult woman: a case report

Annals of The Royal College of Surgeons of England ◽

10.1308/rcsann.2016.0232 ◽

2017 ◽

Vol 99 (1) ◽

pp. e3-e5 ◽

Cited By ~ 1

Author(s):

BL Powell

Keyword(s):

Aortic Arch ◽

Vascular Ring ◽

Symptomatic Relief ◽

Barium Swallow ◽

Common Symptom ◽

Kommerell’S Diverticulum ◽

History Of ◽

Swallow Study ◽

Computed Tomography Angiogram

A 48-year-old woman was seen in a surgical outpatient clinic with a 2 year history of progressive dysphagia with occasional regurgitation, partially controlled with a proton pump inhibitor. Primary investigations of pH testing and gastroscopy were normal, although a barium swallow study revealed significant hold-up at the aortic arch impression and a posterior right-sided oesophageal impression suggestive of a right-sided aortic arch. A follow-up computed tomography angiogram discovered a vascular ring encircling the trachea and oesophagus, formed by a right-sided aortic arch with aberrant aortic branches, and a Kommerell’s diverticulum. It was deemed that the patient’s symptoms were related to this vascular ring. The patient underwent stage-one surgery – an extra-anatomic bypass of the double aortic arch and right subclavian artery – and 4 months later a stent graft insertion over the origin of the diverticulum with the aim of complete symptomatic relief. This case presents a common symptom familiar to any clinician (dysphagia), which has been caused by a rare pathology. It is even more unusual that this should present itself in adulthood.

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An unusual case of vascular ring: The right aortic arch with mirror-image branching and aneurysm of the left ductus arteriosus

Pediatric Pulmonology ◽

10.1002/ppul.1950090109 ◽

1990 ◽

Vol 9 (1) ◽

pp. 41-43

Author(s):

H. Arakawa ◽

K. Tokuyama ◽

H. Mochizuki ◽

M. Shigeta ◽

M. Tashiro ◽

...

Keyword(s):

Aortic Arch ◽

Unusual Case ◽

Ductus Arteriosus ◽

Vascular Ring ◽

Mirror Image ◽

Right Aortic Arch ◽

The Right

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Double aortic arch: an elusive diagnosis in a child with CHD

Cardiology in the Young ◽

10.1017/s1047951121004856 ◽

2021 ◽

pp. 1-3

Author(s):

Mariana Lemos ◽

Miguel Fogaça da Mata ◽

Ana Coutinho Santos

Keyword(s):

Ventricular Septal Defect ◽

Pulmonary Artery ◽

Aortic Arch ◽

Ct Angiography ◽

Septal Defect ◽

Vascular Ring ◽

Pulmonary Atresia ◽

Double Aortic Arch ◽

Shunt Surgery

Abstract An 18-month-old male with pulmonary atresia and ventricular septal defect presented with stridor after neonatal systemic-to-pulmonary artery shunt surgery, that persisted on follow-up. CT angiography revealed a vascular ring with balanced double aortic arch.

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Complete Vascular Ring Detected by Barium Esophagography

Ear Nose & Throat Journal ◽

10.1177/014556130208100816 ◽

2002 ◽

Vol 81 (8) ◽

pp. 554-555 ◽

Cited By ~ 3

Author(s):

Liam J. Skinner ◽

Stephanie Ryan ◽

John D. Russell

Keyword(s):

Aortic Arch ◽

Vascular Ring ◽

Double Aortic Arch ◽

Vascular Rings ◽

Barium Esophagography

The diagnosis of a vascular ring can be made on the basis of characteristic findings on barium esophagography. We report a case of a double aortic arch in a 9-month-old girl that was diagnosed in this manner, and we briefly review the anatomic characteristics of vascular rings.

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Video-Assisted Thoracoscopic Division of Vascular Rings in Pediatric Patients

The American Surgeon ◽

10.1177/000313480507100403 ◽

2005 ◽

Vol 71 (4) ◽

pp. 289-291 ◽

Cited By ~ 8

Author(s):

Curt S. Koontz ◽

Amina Bhatia ◽

Joe Forbess ◽

Mark L. Wulkan

Keyword(s):

Aortic Arch ◽

Pediatric Patients ◽

Vascular Ring ◽

Operating Time ◽

Surgical Techniques ◽

Retrospective Chart Review ◽

Ligamentum Arteriosum ◽

Vascular Rings ◽

Computed Tomographic ◽

Video Assisted

Vascular rings are usually repaired via left thoracotomy. We report our series of pediatric patients with vascular rings that were repaired thoracoscopically. From February 2002 to September 2004, 13 patients underwent video-assisted thoracoscopic surgical techniques (VATS) division of their vascular ring. Chest magnetic resonance arterography (MRA) and/or computed tomographic arteriography (CTA) were used to evaluate the vascular ring in most patients. Patients were chosen for VATS repair based on surgeon's choice and type of vascular ring. Data are expressed as mean ± SD. The Children's Healthcare of Atlanta Institutional Review Board approved this retrospective chart review. Age and weight was 1.5 ± 1.8 years (range: 4 months–17 years) and 16.0 ± 12.5 kg (range: 6.0–22.1 kg), respectively (n = 13). Associated diseases included congenital heart disease (n = 2). Symptoms included respiratory complaints (n = 6), dysphagia (n = 2), dysphagia and shortness of breath (n = 1), pneumonia (n = 2), tracheal deviation (n = 1), and one patient was asymptomatic. Vascular ring types included double aortic arch (n = 4) and right aortic arch with an aberrant left subclavian artery and a left ligamentum arteriosum (n = 9). Operating time was 70 ± 20 minutes (range: 46–122 minutes). One patient had to be opened because of a large arch. Length of stay was 1.9 ± 0.9 days (range: 1–3 days). There were no complications, and all patients improved clinically at follow-up. Thoracoscopic repair of certain types of vascular rings seems to be safe and effective in children. More patients, however, need to be studied.

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P725 An unexpected direction: a case report of a double aortic arch in an asymptomatic woman in adulthood

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.397 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

I Campos ◽

C Vieira ◽

N Salome ◽

V H Pereira ◽

A Costeira Pereira ◽

...

Keyword(s):

Case Report ◽

Aortic Arch ◽

Ct Angiography ◽

Vascular Ring ◽

Double Aortic Arch ◽

Posterior Arch ◽

Vascular Rings ◽

Thoracic Ct ◽

Extrinsic Compression ◽

The Right

Abstract Introduction Complete vascular rings represent about 0.5-2% of all congenital cardiovascular malformations, with the double aortic arch (DAA) being the most common of the complete vascular rings, causing tracheoesophageal compression. The right (posterior) arch is usually dominant (70%), although the two arches can have the same size (5%). The left (anterior) arch is dominant in only approximately 25% of cases. In most cases, this anomaly is diagnosed during childhood due to symptoms caused by oesophageal or tracheal compression. For this reason, case reports of adults are rare. This report describes a case of a 61-year-old woman with DAA with dominant left arch, diagnosed accidentallyby thoracic CT angiography. Case Report Description A 61 years old woman with a previous story of hypertension and type 1 diabetes presented to the emergency service with dyspnoea and thoracic pain. She also referred a history of intermittent dysphagia and cough with at least 12 years of progression. All the parameters of the physical examination were within normal limits. The electrocardiogram showed a normal sinus rhythm with no evidence of acute ischemia and her blood analyses did not show any abnormalitie. She also performed a thoracic CT angiography, which excluded signs of pulmonary embolism, but revealed a vascular ring suggesting a double aortic arch with permeability in both right and left arches as well as their collaterals. The Cardiac MRI was performed with the purpose of excluding ischemia, confirming the double aortic arch with left dominance. The right arch, posterior to the oesophagus and trachea, and the left arch, in an anterior position, showed an anatomic compression of the oesophagus as well as the proximal trachea, capable of eliciting the symptoms mentioned. Other congenital anomalies were excluded. The echocardiography did not demonstrate any additional cardiac malformation. Endoscopy shows a pulsatile extrinsic compression of the esophagus (aortic ring). The patient is currently being studied and closely monitored in the Cardiology consultation. Discussion The most common type of complete vascular ring is the double aortic arch, which accounts for 70% of the complete rings. In most cases, there are two permeable arches, usually with right dominance (70% of the cases). Rarely, both arches are symmetrical. Symptoms usually appear in the fifth month of life. In most cases, only supportive treatment is required. Conclusion This case illustrates the atypical features of this congenital malformation, namely the diagnosis during adulthood as well as the left dominance. Abstract P725 Figure. A double aortic arch

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Kommerell’s Diverticulum With a Twist: A Case of Recurrent Wheeze in an 8-Year-Old Boy

Global Pediatric Health ◽

10.1177/2333794x19897506 ◽

2019 ◽

Vol 6 ◽

pp. 2333794X1989750

Author(s):

Tiffany Amber Robles ◽

Aditya Srinivasan ◽

Lynnette Mazur ◽

Anand Gourishankar

Keyword(s):

Aortic Arch ◽

Pediatric Patient ◽

Respiratory Symptoms ◽

Vascular Ring ◽

Anatomical Variant ◽

Vascular Abnormalities ◽

Aortic Arch Anomaly ◽

Asthma Exacerbations ◽

Kommerell’S Diverticulum ◽

Recurrent Wheeze

Kommerell’s diverticulum is a rare, congenital aortic arch anomaly, usually associated with other vascular abnormalities. When present with a concurrent right-sided aortic arch and an aberrant subclavian artery, this triad can form a vascular ring that encompasses the trachea and esophagus. This anatomical variant is usually asymptomatic but can present with respiratory symptoms due to compression of the trachea. In this report, we discuss a case of a Kommerell’s diverticulum, which presented as frequent and recurring asthma exacerbations in a pediatric patient.

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Surgical Management of Right Aortic Arch Obstruction Associated With Rare Form of Vascular Ring

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135119885893 ◽

2020 ◽

Vol 11 (2) ◽

pp. 222-225

Author(s):

Evgeny V. Krivoshchekov ◽

Frank Cetta ◽

Oleg A. Egunov ◽

Evgenii A. Sviazov ◽

Valeriy O. Kiselev ◽

...

Keyword(s):

Aortic Arch ◽

Surgical Management ◽

Rare Case ◽

Vascular Ring ◽

Right Aortic Arch ◽

Rare Form ◽

Descending Aorta ◽

Arch Obstruction ◽

Right Descending Aorta

This clinical case demonstrated surgical management for a rare case of vascular ring associated with an elongated and kinked aortic arch and a right descending aorta in a ten-year-old male using an extra-anatomic bypass grafting method and dividing the vascular ring. Computer tomography performed at six-month follow-up showed a favorable surgical outcome.

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