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Pediatric Pulmonology Dec 2022Vascular rings are congenital anomalies of the aortic arch that compress the trachea and esophagus and may require corrective surgery. Data about the long-term effects...
BACKGROUND
Vascular rings are congenital anomalies of the aortic arch that compress the trachea and esophagus and may require corrective surgery. Data about the long-term effects of vascular rings are scarce. We aimed to evaluate the long-term cardiorespiratory, exercise capacity, and quality of life of vascular ring patients.
METHODS
A single center prospective study evaluating spirometry, echocardiography, six-minute walk test (6MWT), cardiopulmonary exercise testing (CPET), and quality of life questionnaire (SF36) in patients with a diagnosis of vascular ring, with or without corrective surgery.
RESULTS
Twenty-seven patients participated (11.9 ± 6 years, 52% males). The most common diagnosis was double aortic arch (16 patients, 59%). Nineteen patients had corrective surgery (O) and 8 did not (NO). Pulmonary function tests were within normal range in both groups (FEV % predicted O = 87.6 ± 16.5, NO = 83 ± 10.8%). However, 11/27 had abnormal FEV , 5 had abnormal FVC, and 13 (48%) had flattening of the expiratory curve. 6MWD and oxygen uptake were similarly mildly reduced in both groups; (6MWD O = 80.1 ± 10.7% predicted, NO = 74.1 ± 10.9% and oxygen uptake O = 78.5 ± 23.2% predicted, NO = 73.4 ± 14.3%). Peak O pulse (V̇O /HR% predicted) was mildly reduced in the NO group (O = 88.4 ± 17.3%, NO = 75.8 ± 16.2%). Echocardiogram and SF36 scores were normal in all patients.
CONCLUSIONS
Long-term evaluation of patients born with vascular rings revealed mild pulmonary impairment, reduction in 6MWD, and oxygen uptake. The NO group had also mild reduced peak O pulse. Larger, long-term studies assessing functional parameters in operated and non-operated patients are needed to assess disease/surgery limitation in patients with vascular rings. Clinical trial registration number: NCT04781738.
Topics: Male; Humans; Female; Vascular Ring; Quality of Life; Exercise Tolerance; Forced Expiratory Volume; Prospective Studies; Exercise Test; Oxygen; Oxygen Consumption
PubMed: 35971243
DOI: 10.1002/ppul.26112 -
Maedica Dec 2021Double aortic arch represents a congenital vascular malformation that is characterized by the development of a complete vascular ring around the esophagus and trachea...
Double aortic arch represents a congenital vascular malformation that is characterized by the development of a complete vascular ring around the esophagus and trachea due to an anomaly in the development of branchial arteries. We present the case of a 31-year-old gravida that was referred for fetal ultrasound anomalies screening at 22 weeks and six days of gestation. Routine ultrasound scanning of the fetus revealed a structural aortic arch anomaly consistent with a double aortic arch, with no other cardiac and diextracardiac congenital structural malformations. Knowledge of embryology and imaging spectrum of aortic arch anomalies that are able to form vascular rings around the trachea and esophagus are essential for an accurate antenatal diagnosis and therefore, for a correct clinical management.
PubMed: 35261677
DOI: 10.26574/maedica.2020.16.4.717 -
Archivos de Cardiologia de Mexico 2017The right-side aortic arch may be associated with aberrant left subclavian artery, in some cases this artery originates from an aneurismal dilation of the aorta called... (Review)
Review
The right-side aortic arch may be associated with aberrant left subclavian artery, in some cases this artery originates from an aneurismal dilation of the aorta called Kommerell's diverticulum. A report is presented on 2 cases of vascular ring formed by a right-side aortic arch, anomalous left subclavian artery, Kommerell's diverticulum and left patent ductus arteriosus. A review the literature was also performed as regards the embryological development and the imaging methods used to help in the diagnosis of this rare vascular anomaly.
Topics: Abnormalities, Multiple; Aneurysm; Aorta, Thoracic; Cardiovascular Abnormalities; Diverticulum; Humans; Subclavian Artery; Vascular Ring
PubMed: 28625795
DOI: 10.1016/j.acmx.2017.03.006 -
BMJ Case Reports Jun 2014Vascular rings can present with non-specific respiratory and/or oesophageal symptoms. Early diagnosis requires a high index of suspicion. This case report describes an...
Vascular rings can present with non-specific respiratory and/or oesophageal symptoms. Early diagnosis requires a high index of suspicion. This case report describes an uncommon acute presentation of a vascular ring. We report a thriving 14-month-old child with a long history of recurrent wheeze and 'noisy breathing'. He presented acutely with food bolus impaction in the oesophagus which led to a respiratory arrest. Oesophagoscopy and bronchoscopy suggested vascular ring anomaly. A contrast-enhanced CT scan demonstrated a right-sided aortic arch with left ligamentum arteriosum encircling the oesophagus and airway. The ligament was ligated and divided. At follow-up 6 months later, the infant had mild persistent stridor but was otherwise well.
Topics: Aorta, Thoracic; Aortic Arch Syndromes; Bronchoscopy; Diagnosis, Differential; Follow-Up Studies; Humans; Infant; Male; Respiratory Insufficiency; Tomography, X-Ray Computed
PubMed: 24895385
DOI: 10.1136/bcr-2013-202164 -
Journal of Veterinary Science Jul 2023This paper reports the clinical findings and surgical treatment of feline right patent ductus arteriosus (RPDA) with a left aortic arch. A two-month-old female Maine...
This paper reports the clinical findings and surgical treatment of feline right patent ductus arteriosus (RPDA) with a left aortic arch. A two-month-old female Maine Coon was referred for an investigation of regurgitation after weaning. RPDA with a left aortic arch was diagnosed based on the echocardiographic and computed tomography (CT) findings. A right-fourth intercostal thoracotomy was found to be an appropriate approach to the duct. Preoperative diagnosis is crucial and diagnostic imaging, including radiography, echocardiography, and cardiac CT examination, is essential for determining if the aortic arch is right or left.
Topics: Animals; Cats; Female; Ductus Arteriosus, Patent; Vascular Ring; Aorta, Thoracic; Tomography, X-Ray Computed; Catalase; Cat Diseases
PubMed: 37532295
DOI: 10.4142/jvs.22290 -
The Journal of Thoracic and... Sep 2020
Topics: Aorta; Humans; Vascular Ring
PubMed: 32616348
DOI: 10.1016/j.jtcvs.2020.03.160 -
Journal of the American Society of... Aug 2018
Topics: Algorithms; Child, Preschool; Echocardiography; Female; Humans; Infant; Magnetic Resonance Imaging; Male; Retrospective Studies; Sensitivity and Specificity; Vascular Ring
PubMed: 30078432
DOI: 10.1016/j.echo.2018.05.004 -
The Journal of Thoracic and... Aug 2017
Topics: Aorta, Thoracic; Child; Humans; Muscle, Smooth, Vascular; Thoracotomy; Vascular Ring; Young Adult
PubMed: 28412114
DOI: 10.1016/j.jtcvs.2017.03.024 -
Journal of Cardiac Surgery Jun 2022We hereby present a case of tetralogy of Fallot with duplicated brachiocephalic vein having a preaortic and a subaortic component. We aim to highlight the role of...
We hereby present a case of tetralogy of Fallot with duplicated brachiocephalic vein having a preaortic and a subaortic component. We aim to highlight the role of computed tomography angiography in identifying these rare associations of tetralogy of Fallot.
Topics: Angiography; Brachiocephalic Veins; Cardiovascular Abnormalities; Humans; Tetralogy of Fallot; Vascular Ring
PubMed: 35362156
DOI: 10.1111/jocs.16450 -
Journal of Pediatric Surgery Oct 2021Kommerell diverticulum (KD) in children with right aortic arch and aberrant left subclavian artery can be treated surgically via left subclavian artery (LSCA)...
BACKGROUND/PURPOSE
Kommerell diverticulum (KD) in children with right aortic arch and aberrant left subclavian artery can be treated surgically via left subclavian artery (LSCA) translocation or aortopexy. These procedures remain controversial. We compared the outcomes of both procedures.
METHODS
This retrospective study included 48 patients who underwent LSCA translocation (n = 26) or aortopexy (n = 22). Lateral thoracotomy and sternotomy were performed in 38 and 10 patients with severe intracardiac malformations, respectively.
RESULTS
The median age of the aortopexy group was 10 months, median follow-up was 14 months, and mean length of hospitalization was 11.41 ± 6.8 days. The median age of the LSCA translocation group was 12 months, median follow-up was 22 months, and mean length of hospitalization was 14.96 ± 7.74 days. Two patients (LSCA translocation group) required secondary intubations and none suffered from recurrent laryngeal nerve injury or complications related to LSCA translocation. Breathing and swallowing symptoms were relieved in all patients. No additional thoracotomies due to respiratory symptoms were required.
CONCLUSIONS
These two procedures relieve the pressure on the trachea and esophagus with similar short-term outcomes. The long-term outcomes are unknown. LSCA translocation may be the best strategy to prevent symptom recurrence and late complications in these patients.
LEVEL OF EVIDENCE
Level III.
Topics: Aorta, Thoracic; Cardiovascular Abnormalities; Child; Diverticulum; Heart Defects, Congenital; Humans; Infant; Neoplasm Recurrence, Local; Retrospective Studies; Subclavian Artery; Vascular Ring
PubMed: 33678405
DOI: 10.1016/j.jpedsurg.2021.02.009