Morphology of vascular ring arch anomalies influences prognosis and management

Abstract

ObjectiveThis study aimed to explore the anatomical features of aortic arch anomalies associated with vascular rings, hoping to identify those which may increase the risk of symptomatic presentation and surgical intervention.MethodsThis was a retrospective observational study at a single cardiac unit. Individuals diagnosed with an aortic arch anomaly, either isolated or non-isolated, between June 2014 and September 2018 were included. The morphology of the aortic arch was established via analysis of postnatal echocardiography, CT or MRI scans. CT and magnetic resonance studies were evaluated for the presence of a Kommerell diverticulum in those with aberrant vessels. Case notes were reviewed for relevant clinical data.ResultsOf those with aberrant subclavian arteries, 24/79 (30.4%) were shown to have a Kommerell diverticulum. Additional forms of congenital heart disease were present in 133/227 (58.6%) individuals. Surgical division of the vascular ring was performed in 30/227 (13.2%), most commonly in the setting of a double aortic arch (70.8%). In those with aberrant subclavian arteries, no children without a Kommerell diverticulum were referred for surgery. In those with a Kommerell diverticulum confirmed on imaging, 11/24 underwent surgery.ConclusionIndividuals with a double aortic arch, or an aberrant subclavian artery arising from a Kommerell diverticulum, have the highest requirement for surgical intervention, especially in isolated anomalies. These individuals should remain under monitoring. The subjective nature of symptoms remains problematic. Longitudinal research is required further to understand the natural history of vascular rings and how it links to morphology.

Impact on the trachea in children with prenatally diagnosed vascular ring formed by a right aortic arch

“…The reason for this variance may relate to caseload, for example the study by Young et al 12 reported only 10 cases of isolated RAA‐LD. Whereas other postnatal studies which were undertaken in a pediatric cardiology center 13 and in specialist tracheal unit 14 note similar rates of symptoms as described in this current study.…”

“…Whereas other postnatal studies which were undertaken in a pediatric cardiology center 13 and in specialist tracheal unit 14 note similar rates of symptoms as described in this current study.…”

Management of prenatally detected vascular rings: a United Kingdom national survey

“…This routinely acquired, additional view of the upper mediastinum has also led to an increase in the detection of right and double aortic arches (RAA/DAA) prenatally. [2][3][4] These anomalies can be associated with chromosomal and genetic syndromes and extracardiac anomalies. A DAA and the subtype of a RAA with left ductal ligament encircle the trachea and oesophagus which can lead to symptoms/signs of tracheoesophageal compression, 5 but there are no population-based studies to help understand how frequently this develops and how expectant families should be counselled or managed after birth.…”

Fetal echocardiographic markers to differentiate between a right and double aortic arch

“…The challenge for management of vascular rings lies in those that are isolated without other forms of congenital heart disease. It has been shown that children with a DAA tend to present earlier with symptoms/signs of trache‐oesophageal compression and are more likely to be associated with residual respiratory post‐operative morbidity compared to RAA 2,4‐13 . with some surgical centres advocating universal early surgery 13‐16 .…”

“…It has been shown that children with a DAA tend to present earlier with symptoms/signs of tracheoesophageal compression and are more likely to be associated with residual respiratory post-operative morbidity compared to RAA. 2,[4][5][6][7][8][9][10][11][12][13] with some surgical centres advocating universal early surgery. [13][14][15][16] Accurate prenatal diagnosis of vascular rings assists with perinatal management by facilitating parental education such that possible clinical features can be identified early, leading to timely assessment and treatment.…”