scholarly journals VP32.03: Aberrant right subclavian artery as only finding in a mosaic Turner syndrome

2020 ◽  
Vol 56 (S1) ◽  
pp. 188-189
Author(s):  
A.S. Silva ◽  
P.A. Costa ◽  
A. Fontoura Oliveira ◽  
S. Azevedo ◽  
M. Torrão ◽  
...  
Keyword(s):  
Turner Syndrome ◽  
Subclavian Artery ◽  
Aberrant Right Subclavian Artery

P2244Anomalies in women of the aortic arch in Turner syndrome as an important risk factor for premature morbidity and mortality

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
E Klaskova ◽  
S Kapralova ◽  
J Zapletalova ◽  
Z Tudos ◽  
K Adamova
Keyword(s):  
Risk Factor ◽  
Aortic Arch ◽  
Cell Line ◽  
Turner Syndrome ◽  
Subclavian Artery ◽  
X Chromosome ◽  
Coarctation Of The Aorta ◽  
Aberrant Right Subclavian Artery ◽  
Structural Abnormality ◽  
Study Group

Abstract Introduction Turner syndrome (TS) represents the most common chromosomal disorder in women being, caused by the absence or structural abnormality of X chromosome. Congenital heart defects affect up to 50% of females with TS.Prevalence of coarctation of the aorta in TS has been estimated 7–18% depending on imaging method. Introduction of cardiac magnetic resonance imaging (MRI) into the routine practice markedly increased the detection rate of anomalies of the aortic arch such as elongated transverse aortic arch with abnormal curvature, i.e.kinking, pseudocoarctation or aberrant right subclavian artery. Aims of study was to estimate prevalence of anomalies of the aortic arch in our study group according to the karyotype. Methods and patients Study group consisted of 67 patients with TS at the age 7.3 yrs (range 0.1 - 16.5 yrs.). Complete cardiovascular examination (echocardiography, MRI of the heart and great vessels) and cytogenetic examination were performed in each of our study patient. Results The prevalence of anomalies of the aortic arch was 15% (10 patients). Four of them had elongated transverse aortic, coarctation of the aorta was found in three cases, aberrant right subclavian artery in two patients and one girl had right aortic arch. 45,X cell line was presented in every patient with anomaly of the aortic arch, none of them had structural abnormality of X chromosome. Conclusions Compared with the general population, the prevalence of CoA and the others anomalies of the aortic arch is significantly higher in women with TS, especially with 45,X cell line. As far as CoA is considered to be one of the major risk factor for aortic dissection detailed cardiovascular screening focused on thoracic aorta anomalies seems to be crucial in order to prevent it. Acknowledgement/Funding Supported by Ministry of Health, Czech Republic - MZ VES 2017 (Reg. No. NV17-29111A).

scholarly journals Study on the views and methods of ultrasonic screening and diagnosis for abnormal aortic arch in infants

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Xinjian He ◽  
Jiaoyang Chen ◽  
Gaoyang Li
Keyword(s):  
Diagnostic Accuracy ◽  
Aortic Arch ◽  
Subclavian Artery ◽  
Coarctation Of The Aorta ◽  
Double Aortic Arch ◽  
Aberrant Right Subclavian Artery ◽  
Coincidence Rate ◽  
Aortic Arch Anomalies ◽  
Screening Sensitivity ◽  
Axis View

Abstract Background The purpose of this study was to explore echocardiographic views and methods of aortic arch anomalies in infants, so as to improve the screening sensitivity and diagnostic accuracy. Methods 140 children with abnormal aortic arch diagnosed by ultrasound in Children’s Hospital of Hebei Province from January 2014 to December 2019 were selected for retrospective analysis. All were confirmed by surgery or/and computerized tomography angiography. Series of views for aortic arch (the three-vessel and tracheal view, aortic arch short axis view, left aortic arch long axis view, aortic arch long axis continuous scan views) were performed in all cases on the basis of the routine views of echocardiography. The screening sensitivity and diagnostic coincidence rate of different echocardiographic views for aortic arch anomalies were analyzed. Results Among the 140 infants, right aortic arch were 21 cases (6/21 were accompanied by mirror branch and 15/21 were with aberrant left subclavian artery). Left aortic arch with aberrant right subclavian artery were 2 cases, and double aortic arch with both arches open were 20 cases. Double aortic arch with left arch atresia were 2 cases, and atresia of the proximal aorta with aortic arch dysplasia was 1 case. Coarctation of the aorta were 67 cases, and interruption of aortic arch were 27 cases. All the patients were correctly diagnosed except that 2 infants with interruption of aortic arch were incorrectly diagnosed as coarctation of the aorta, and 1 infant with coarctation of the aorta was misdiagnosed as interruption of aortic arch by echocardiography. The screening sensitivities of four views and four-view combination for abnormal aortic arch were 99.3, 73.6, 87.1, 99.3, and 100%; the diagnostic coincidence rates were 85.7, 27.1,66.4, 95.0%, and 97.9% respectively. On the basis of traditional left aortic long axis view, other three views had their own advantages. The screening sensitivity and diagnostic coincidence rate of four-view combination were significantly improved. Conclusions The three-vessel trachea view is simple and feasible, which is suitable for screening abnormal aortic arch. The combination of four views conduces to improving screening sensitivity and diagnostic accuracy of aortic arch abnormalities.

scholarly journals Case report: a step-wise management of concurrent presentation of congenital single lung and aberrant right subclavian artery in an infant girl

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Keon Young Park ◽  
Kevin C. Janek ◽  
Joshua L. Hermsen ◽  
Petros V. Anagnostopoulos ◽  
Hau D. Le
Keyword(s):  
Subclavian Artery ◽  
Tissue Expander ◽  
Rare Condition ◽  
Aberrant Right Subclavian Artery ◽  
Cross Sectional ◽  
Unique Challenge ◽  
Surgical Interventions ◽  
Tracheal Compression ◽  
Dysphagia Lusoria ◽  
Infant Girl

Abstract Introduction Congenital single lung (CSL) is a rare condition, and symptomatic patients often present with respiratory distress or recurrent respiratory infection due to mediastinal shift causing vascular or airway compression. Aberrant right subclavian artery (ARSA) is another rare congenital anomality that can lead to tracheal or esophageal compressions. There is only one other case of concurrent presentation of CSL and ARSA reported, which presented unique challenge in surgical management of our patient. Here we present a step-wise, multidisciplinary approach to manage symptomatic CSL and ARSA. Case presentation An infant girl with a prenatal diagnosis of CSL developed worsening stridor and several episodes of respiratory illnesses at 11 months old. Cross-sectional imaging and bronchoscopic evaluation showed moderate to severe distal tracheomalacia with anterior and posterior tracheal compression resulting from severe mediastinal rotation secondary to right-sided CSL. It was determined that her tracheal compression was mainly caused by her aortic arch wrapping around the trachea, with possible additional posterior compression of the esophagus by the ARSA. She first underwent intrathoracic tissue expander placement, which resulted in immediate improvement of tracheal compression. Two days later, she developed symptoms of dysphagia lusoria due to increased posterior compression of her esophagus by the ARSA. She underwent transposition of ARSA to the right common carotid with immediate resolution of dysphagia lusoria. As the patient grew, additional saline was added to the tissue expander due to recurrence in compressive symptoms. Conclusions Concurrent presentation of CSL and ARSA is extremely rare. Asymptomatic CSL and ARSA do not require surgical interventions. However, if symptomatic, it is crucial to involve a multidisciplinary team for surgical planning and to take a step-wise approach as we were able to recognize and address both tracheomalacia and dysphagia lusoria in our patient promptly.

scholarly journals Predictive value of aberrant right subclavian artery for fetal chromosome aneuploidy in women of advanced maternal age

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Li-Ping Chen ◽  
Yong-Feng Lai ◽  
Xiao-Hong Zhong ◽  
Jian-Hong You ◽  
Jiang-Hua Chen ◽  
...  
Keyword(s):  
Subclavian Artery ◽  
Maternal Age ◽  
Chromosomal Abnormalities ◽  
Age Groups ◽  
Advanced Maternal Age ◽  
Aberrant Right Subclavian Artery ◽  
High Prevalence ◽  
Ultrasound Findings ◽  
Increase Risk ◽  
Singleton Pregnancies

Abstract Background In the entire population, an aberrant right subclavian artery (ARSA) is closely associated with chromosomal abnormalities. ARSA with additional ultrasonic findings would increase risk of chromosomal abnormalities. The risk of fetal chromosomal abnormalities increased exponentially with the maternal age. These risks in the advanced maternal age (AMA) group are uncertain. This study aimed to determine the incidence of ARSA in Chinese AMA and non-AMA women and the frequency of aneuploidy among AMA and non-AMA women with ARSA. Methods This retrospective study included 13,690 singleton pregnancies, were divided into AMA and non-AMA groups. Integrated obstetric ultrasonic screening, biochemical screening, noninvasive prenatal screening, and fetal karyotype analysis were analyzed. Results The overall incidence of ARSA was 0.69%, with no difference between age groups. The incidence of chromosomal abnormalities in the AMA group (37 / 2860) was much higher than that of the non-AMA group. The risk of chromosomal abnormalities significantly increased with both ARSA detected and additional ultrasound findings. With combined ARSA and AMA, the likelihood of the incidence of chromosomal abnormalities increased. Chimerism (45X / 46XX) was found with isolated ARSA in AMA pregnancies. Conclusion There is a high prevalence of chromosomal abnormalities in fetuses of AMA women. ARSA increases the risk of chromosomal abnormalities in both age groups, especially combined with ARSA. When ARSA occurs in AMA women, it confers a high likelihood of chromosomal abnormalities.

Isolated aberrant right subclavian artery at 14-16 weeks of gestation and Down syndrome

2017 ◽  
Vol 46 (1) ◽  
pp. 62-63
Author(s):  
Ron Beloosesky ◽  
Alon Shrim ◽  
Maya Steinberg ◽  
Nizar Khatib ◽  
Moshe Bronshtein
Keyword(s):  
Down Syndrome ◽  
Subclavian Artery ◽  
Aberrant Right Subclavian Artery

scholarly journals Less-Invasive Endovascular Treatment of Arch Aneurysm With Aberrant Right Subclavian Artery

2015 ◽  
Vol 100 (3) ◽  
pp. 1089-1091 ◽  
Author(s):  
Yosuke Takahashi ◽  
Yasuyuki Sasaki ◽  
Yasuyuki Kato ◽  
Manabu Motoki ◽  
Yasuyuki Bito ◽  
...  
Keyword(s):  
Endovascular Treatment ◽  
Subclavian Artery ◽  
Aberrant Right Subclavian Artery ◽  
Less Invasive ◽  
Arch Aneurysm
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