-
European Journal of Cardio-thoracic... Feb 2022Vascular rings are rare anomalies of congenital heart disease that cause respiratory and gastrointestinal symptoms. This study assessed the long-term outcomes of...
OBJECTIVES
Vascular rings are rare anomalies of congenital heart disease that cause respiratory and gastrointestinal symptoms. This study assessed the long-term outcomes of patients with vascular ring division.
METHODS
A multi-institution retrospective review of 371 patients with vascular rings undergoing surgical division at 3 paediatric cardiac institutions between November 2007 and October 2019 was performed.
RESULTS
The complete vascular rings consisted of a double aortic arch (24.5%), right aortic arch with left ligamentum arteriosum (36.7%) and left aortic arch, with right ligamentum arteriosum (0.5%). The incomplete vascular rings consisted of a pulmonary artery sling (22.9%), left aortic arch with aberrant right subclavian artery (15.1%) and innominate artery compression syndrome (0.3%). Respiratory symptoms included stridor (71.4%), wheezing (49.1%), coughing (31.5%), gastrointestinal symptoms included choking (12.4%), dysphagia (3.2%) and emesis (1.9%). Only one patient died after discharge, yielding a late mortality rate of 0.3% (1/360). The 10-year overall survival rate was 96.8%. Postoperative complications were reported in 51 patients, 15 of whom required reoperation. The 10-year freedom from reoperation rate was 95.9%. Follow-up was completed in 95.4% (354/371) of patients, with a mean follow-up time of 4.3 ± 2.9 years (range from 1 to 13 years). Twenty patients (5.6%) experienced residual symptoms during long-term follow-up.
CONCLUSIONS
The outcomes of vascular ring division are excellent. A Kommerell diverticulum >1.5 times the aberrant left subclavian artery origin is an operative indication for primary resection. Tracheomalacia is a risk factor for reoperation and residual symptoms, and preoperative fibrobronchoscopy is important for evaluation.
Topics: Aorta, Thoracic; Aortic Diseases; Child; Heart Defects, Congenital; Humans; Subclavian Artery; Vascular Ring
PubMed: 34632492
DOI: 10.1093/ejcts/ezab432 -
Pediatric Cardiology Jun 2023Vascular rings are increasingly identified on fetal echocardiography. The purpose of this study is to analyze clinical outcomes and patterns of diagnostic testing in...
Vascular rings are increasingly identified on fetal echocardiography. The purpose of this study is to analyze clinical outcomes and patterns of diagnostic testing in fetuses with vascular rings diagnosed by echocardiography. A retrospective cohort study was performed of fetuses with postnatally confirmed vascular rings from 2017 to 2022. Clinical outcomes included type and timing of symptoms, and timing of surgical intervention. Freedom from symptoms and/or surgery was assessed by Kaplan-Meier analysis. Frequency of genetic and diagnostic testing (barium esophagogram, CT/MRI angiogram, and bronchoscopy) was also assessed. Overall, 46 patients were evaluated (91% with a right aortic arch/left ductus and 4% with a double aortic arch). A vascular ring was isolated in 59%, associated with structural heart lesions in 33%, and associated with noncardiac anomalies in 8%. Prenatal diagnoses increased over time. Symptoms developed in 24% (11/46); 82% (9/11) had respiratory and 45% (5/11) had gastroesophageal complaints. Surgery was performed in 17% (11/46). Symptoms presented bimodally, prior to 100 or after 400 days of life. There was no difference in the type of symptoms for early (< 100 days) or late (> 400 days) presenters. Symptomatic patients received more diagnostic testing. Genetic testing was obtained in 46% and positive in 33%, with 22q11 deletion and Trisomy 21 being identified. Prenatal diagnoses of vascular rings increased over time, with subjects developing symptoms bimodally in early or late infancy. The frequency of genetic testing was suboptimal given the prevalence of genetic abnormalities seen in this population.
PubMed: 37354371
DOI: 10.1007/s00246-023-03219-5 -
Outcomes following thoracotomy or thoracoscopic vascular ring division in children and young adults.The Journal of Thoracic and... Aug 2017To determine the short- and intermediate-term outcomes following vascular ring division by video-assisted thoracoscopic (VATS) or thoracotomy approach.
OBJECTIVE
To determine the short- and intermediate-term outcomes following vascular ring division by video-assisted thoracoscopic (VATS) or thoracotomy approach.
METHODS
This study is a retrospective review of patients with double aortic arch or right aortic arch with aberrant left subclavian artery and left ligamentum (RAA with LSCA/LL) who underwent division via left thoracotomy or VATS approach at a single institution. Outcome measures included operation duration (OR time), hospital length of stay (LOS), postoperative complications, and freedom from reintervention.
RESULTS
A total of 200 patients underwent vascular ring division by VATS (n = 115) or thoracotomy (n = 85). Of 200 patients, 125 (56%) displayed respiratory symptoms at presentation, 29 (11%) displayed digestive tract symptoms, and 41 (32%) displayed both. Median follow-up was 2.1 years (interquartile range: 1 month to 7.1 years). Overall cohort mortality was 1 (0.5%) of 200 at 11 years after surgery. Fifteen (7.5%) experienced immediate postoperative complications. At follow-up visit within 1 month after surgery, 92 (74%) of 125 patients reported improvement in symptoms. Freedom from reintervention was 96% and 90% at 5 years and 10 years, respectively. Among patients with RAA with LSCA/LL (n = 113), VATS was associated with shorter LOS compared with thoracotomy (1.2 vs 3.4 days; P < .001), but rates of complications were not significantly different (9% vs 4%, P = .68).
CONCLUSIONS
Vascular ring division by either thoracotomy or VATS can be performed with low rates of complication and need for reintervention. Compared with thoracotomy, VATS is associated with shorter operative duration and hospital LOS among patients with RAA with LSCA/LL.
Topics: Child; Child, Preschool; Female; Humans; Infant; Length of Stay; Male; Operative Time; Postoperative Complications; Reoperation; Retrospective Studies; Thoracic Surgery, Video-Assisted; Thoracotomy; Time Factors; Vascular Ring
PubMed: 28366551
DOI: 10.1016/j.jtcvs.2017.01.058 -
Echocardiography (Mount Kisco, N.Y.) Dec 2020Vascular ring and sling are congenital anomalies of the vascular structure in the thorax with a prevalence of 2.4/10,000 live births. Double aortic arch (DAA), right...
Vascular ring and sling are congenital anomalies of the vascular structure in the thorax with a prevalence of 2.4/10,000 live births. Double aortic arch (DAA), right aortic arch with left ductus arteriosus and aberrant left subclavian artery (RAA-ALSA), and pulmonary artery sling (PAS) are the three common types of vascular ring and sling. These anomalies can be isolated or accompanied by intracardiac malformation. The presence of both vascular ring and PAS is extremely rare. Here, we report a fetus who was prenatally diagnosed with PAS and RAA-ALS, and developed symptoms due to esophageal and airway compression after birth.
Topics: Aorta, Thoracic; Ductus Arteriosus; Humans; Retrospective Studies; Vascular Ring
PubMed: 33107081
DOI: 10.1111/echo.14907 -
Seminars in Thoracic and Cardiovascular... 2020Our series of vascular ring patients treated at Ann & Robert H. Lurie Children's Hospital of Chicago has now exceeded 450 patients (see Central Picture). The...
Our series of vascular ring patients treated at Ann & Robert H. Lurie Children's Hospital of Chicago has now exceeded 450 patients (see Central Picture). The recommendations from this review are based on our experience with those patients. Since 1990, we have used preoperative cross-sectional imaging in all vascular ring patients. This has led us to refine our operative strategies and carefully tailor the operation to the individual's unique anatomy. In particular for right aortic arch patients, we now address the Kommerell diverticulum in most cases and carefully consider Kommerell diverticulum resection with left subclavian artery transfer. In double aortic arch patients, we also look for a Kommerell diverticulum and resect the diverticulum if present. This will decrease the number of patients presenting for a reoperation after initial vascular ring surgery. Since 1947, we have operated on 217 patients with a right aortic arch. The median age is 1.5 years. Since 2006, 47 out of 111 patients have had Kommerell diverticulum resection and left subclavian artery transfer to the left carotid artery. Mean length of stay is 4.5 days. In the same time period, 187 patients have been treated for a double aortic arch. The median age is 1 year. Twelve of these patients also had resection of a Kommerell diverticulum. The mean length of stay in this group was 3 days. Advanced preoperative cross-sectional imaging leads to precise operative techniques tailored to the unique anatomy of each vascular ring patient.
Topics: Aorta, Thoracic; Deglutition Disorders; Diverticulum; Esophageal Stenosis; Female; Heart Defects, Congenital; Humans; Infant; Male; Tomography, X-Ray Computed; Tracheal Stenosis; Vascular Malformations; Vascular Ring; Vascular Surgical Procedures
PubMed: 32354547
DOI: 10.1053/j.pcsu.2020.02.004 -
Journal of Pediatric Surgery Nov 2020Vascular rings are often diagnosed after evaluation for swallowing and breathing difficulties. Data regarding symptoms following vascular ring repair is sparse. We...
PURPOSE
Vascular rings are often diagnosed after evaluation for swallowing and breathing difficulties. Data regarding symptoms following vascular ring repair is sparse. We sought to determine whether symptoms persist using chart review and a survey.
METHODS
Sixty-three patients underwent open vascular ring repair from July 2007 to May 2018. Data regarding vascular anatomy, demographics, pre- and postoperative symptoms, and chromosomal abnormalities were collected. Freedom from reoperation, 30-day mortality, and complications were assessed. Patient families were contacted for a symptom focused survey.
RESULTS
The median age of surgical intervention was 14.4 months (IQR 5.8-34.7 months) for single aortic arches with an aberrant subclavian artery (SAA), and 5.3 months (IQR 1.3-10.1 months) for double aortic arches (DAA) (Table). Prior to surgery, all but two SAA were symptomatic. There was no operative mortality. Three patients required re-exploration for chylothorax, and three required late aortopexy. At last follow-up, 45% (18/40) SAA and 65% (15/23) DAA had post-operative symptoms. Fourteen patient families completed the symptom survey (10 SAA, 4 DAA). Five SAA had breathing and swallowing symptoms, and 3 SAA and 3 DAA had breathing difficulties.
CONCLUSIONS
Open vascular ring repair remains a safe repair. However, further investigation of the persistent symptoms in these patients is merited.
STUDY TYPE / LEVEL OF EVIDENCE
Retrospective Comparative Study, Level III.
Topics: Aorta, Thoracic; Child, Preschool; Deglutition Disorders; Humans; Infant; Respiration; Respiratory Tract Diseases; Retrospective Studies; Subclavian Artery; Treatment Outcome; Vascular Ring
PubMed: 32005503
DOI: 10.1016/j.jpedsurg.2019.12.022 -
Cardiovascular Pathology : the Official... 2019The traditional classification of congenital aortic arch abnormalities was described by James Stewart and colleagues in 1964. Since that time, advances in diagnostic... (Review)
Review
The traditional classification of congenital aortic arch abnormalities was described by James Stewart and colleagues in 1964. Since that time, advances in diagnostic imaging technology have led to better delineation of the vasculature anatomy and the identification of previously unrecognized and unclassified anomalies. In this manuscript, we review the existing literature and propose a series of modifications to the original Stewart classification of congenital aortic arch abnormalities to incorporate this new knowledge. In brief, we propose the following modifications: (1) In Group I, we further divide subgroup B into left arch atretic and right arch atretic; (2) In Group II, we add three more subgroups, including aberrant right innominate artery, "isolated" right innominate artery (RIA), "isolated" right carotid artery with aberrant right subclavian artery; (3) In Groups I, II, and III, we add a subgroup of absence of both ductus arteriosus; and (4) In Group IV, we add three subgroups, including circumflex retro-esophageal aorta arch, persistent V aortic arch, and anomalous origin of pulmonary artery from ascending aorta.
Topics: Aorta, Thoracic; Clinical Decision-Making; Heart Defects, Congenital; Humans; Predictive Value of Tests; Prognosis; Terminology as Topic; Vascular Malformations
PubMed: 30623879
DOI: 10.1016/j.carpath.2018.11.004 -
Ultrasound in Obstetrics & Gynecology :... Apr 2016Use of recent antenatal screening guidelines for cardiac abnormalities has increased fetal diagnoses of right aortic arch (RAA). We aimed to establish the outcome of... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVES
Use of recent antenatal screening guidelines for cardiac abnormalities has increased fetal diagnoses of right aortic arch (RAA). We aimed to establish the outcome of fetal RAA without intracardiac abnormalities (ICA) to guide postnatal management.
METHODS
In the retrospective cohort part of our study, outcome measures were rates of chromosomal abnormalities, 22q11.2 deletion, fetal extracardiac abnormalities (ECA), postnatal ICA and ECA, and symptoms of and surgery for vascular ring. A systematic review and meta-analysis was also performed; results are reported as proportions. Kaplan-Meier analysis of vascular ring cases with surgery as endpoint was performed.
RESULTS
Our cohort included 86 cases; 41 had a vascular ring. Rates of chromosomal abnormalities, 22q11.2 deletion and fetal ECA were 14.1%, 6.4% and 17.4%, respectively. Sixteen studies including our cohort (312 fetuses) were included in the systematic review. Overall rates of chromosomal abnormalities and 22q11.2 deletion were 9.0% (95% CI, 6.0-12.5%) and 6.1% (95% CI, 3.6-9.3%), whilst the respective rates for cases with no ECA were 4.6% (95% CI, 2.3-7.8%) and 5.1% (95% CI, 2.4-8.6%). ECA were seen in 14.6% (95% CI, 10.6-19.0%) prenatally and in 4.0% (95% CI, 1.5-7.6%) after birth. Postnatal ICA were identified in 5.0% (95% CI, 2.7-7.9%). Rate of symptoms of vascular rings (follow-up ≥ 24 months postpartum) was 25.2% (95% CI, 16.6-35.0%), and 17.1% (95% CI, 9.9-25.7%) had surgery. Two-year freedom from surgery was 83.0% (95% CI, 74.3-90.1%).
CONCLUSIONS
Fetal RAA without ICA is more frequently associated with ECA than with chromosomal abnormalities. Most cases, however, are isolated. Vascular-ring symptoms occur in about 25% of cases. Postnatal surveillance is required mainly in the first 2 years after delivery.
Topics: Abnormalities, Multiple; Aorta, Thoracic; Aortic Arch Syndromes; Chromosome Aberrations; Cohort Studies; DiGeorge Syndrome; Female; Fetal Diseases; Fetal Heart; Heart Defects, Congenital; Humans; Kaplan-Meier Estimate; Pregnancy; Prenatal Diagnosis; Retrospective Studies; Ultrasonography, Prenatal
PubMed: 26643657
DOI: 10.1002/uog.15805 -
Pediatric Radiology Dec 2015Vascular rings can be challenging to diagnose because they can contain atretic portions not detectable with current imaging modalities. In these cases, where the... (Review)
Review
Vascular rings can be challenging to diagnose because they can contain atretic portions not detectable with current imaging modalities. In these cases, where the compressed airway and esophagus are not encircled by patent, opacified vessels, there are useful secondary signs that should be considered and should raise suspicion for the presence of a vascular ring. These signs include a double aortic arch, the four-vessel sign, the distorted subclavian artery sign, a diverticulum of Kommerell, a ductal diverticulum contralateral to the aortic arch, and a descending aorta contralateral to the arch or circumflex aorta. If none of these findings is present, a ring can be excluded with confidence.
Topics: Aorta, Thoracic; Aortic Arch Syndromes; Diagnosis, Differential; Diagnostic Imaging; Humans; Infant, Newborn; Subclavian Artery; Vascular Malformations
PubMed: 26260202
DOI: 10.1007/s00247-015-3424-7 -
Die Pharmazie Jan 2022The relationship between large conductance calcium activated potassium channel (BK) and vascular lesions in type 2 diabetes mellitus (T2DM) was investigated by observing...
The relationship between large conductance calcium activated potassium channel (BK) and vascular lesions in type 2 diabetes mellitus (T2DM) was investigated by observing vascular reactivity of thoracic aorta and mesenteric artery and the current changes of BK in vascular smooth muscle cells (VSMCs). The thoracic aorta and mesenteric artery of T2DM rats were isolated, the whole cell perforated patch clamp experiment and single channel patch clamp experiment of acute enzyme separation of thoracic aorta and mesenteric artery smooth muscle cells were performed to measure the membrane capacitance and the amplitude of macro current. And the vascular ring experiment was performed to observe the change of relaxation percentage. The results showed that the amplitude of BK current in vascular smooth muscle cells of diabetic rats was higher than that of the control group. The channel current had outward rectifying characteristics. BK is related to vascular reactivity and smooth muscle relaxation in T2DM rats. The opening probability of BK in VSMCs of diabetic rats was significantly increased. This study suggests that BK may be a new target for diabetic vascular disease.
Topics: Animals; Aorta, Thoracic; Diabetes Mellitus, Experimental; Diabetes Mellitus, Type 2; Mesenteric Arteries; Muscle, Smooth, Vascular; Myocytes, Smooth Muscle; Rats
PubMed: 35045921
DOI: 10.1691/ph.2022.1740