Isolated Left Subclavian Artery, Complete Atrioventricular Block, and Tricuspid Atresia in a Neonate

Chaturvedi, Kanupriya; Prasad, Deepa; Ashwath, Ravi; Strainic, James; Snyder, Christopher S.

doi:10.14503/thij-15-5692

Cited by 5 publications

(5 citation statements)

References 15 publications

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“…In up to 60% of isolated left subclavian cases, there are co-existent congenital heart defects, mostly Conotruncul anomalies such as double outlet right ventricle and tetralogy of Fallot, atrial septal defects and ventricular septal defects (9). Not surprisingly, our patient has multiple ventricular septal defects, the largest of which was a Perimambranous VSD with 6 mm diameter.…”

Section: Discussionmentioning

confidence: 65%

Isolated Left Subclavian Artery, Multiple Ventricular Septal Defects and Pulmonary Hypertension In A Child: A Case Report

Khalilian¹,

Hekmat²,

Sadr³

et al. 2021

Preprint

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solated left subclavian artery is a rare congenital aortic arch anomaly in which the left subclavian artery is connected to the pulmonary artery via a patent Ductus Arteriosus or a remnant of it, instead of the arota. Generally, it is associated with the right aortic arch and other congenital heart defects, mostly tetralogy of Fallot. Isolated left subclavian artery can cause subclavian steal syndrome, pulmonary steal syndrome and size or blood pressure discrepancy between the two upper limbs. We present a 14-months-old infant with isolated left subclavian artery, multiple ventricular septal defects and pulmonary hypertension. To our knowledge, it is a rare anomaly which can influence the surgical planning and outcomes.

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Section: Discussionmentioning

confidence: 65%

Isolated Left Subclavian Artery, Multiple Ventricular Septal Defects and Pulmonary Hypertension In A Child: A Case Report

Khalilian¹,

Hekmat²,

Sadr³

et al. 2021

Preprint

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“…Ablation of a smaller area within the cardiac neural crest, results in the definitive aorta (fourth arch artery) is on the right, other malformations include double outlet right ventricle, tetralogy of Fallot et al 4,5 . Therefore, the above embryonic mechanisms can explain that ILSA is always associated with RAA, and more than half of ILSA is also associated with cardiac abnormalities, the most common being TOF, 6,7 there are also case reports related to the single ventricle, DORV, transposition of the great arteries, type I and II of aortopulmonary window, 8 coarctation, tricuspid atresia, 9 pseudopulmonary artery sling, 10 and other intracardiac abnormalities. All the CHD fetuses in our study were right aortic arch, and the intracardiac malformations were DORV and TOF, which suggested that when RAA was combined with those intracardiac malformations, it is necessary to pay attention to whether there is ILSA.…”

Section: Discussionmentioning

confidence: 99%

Prenatal ultrasound findings, genetic testing, and literature review of Isolated left subclavian artery

et al. 2023

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Background The occurrence of Isolated left subclavian artery (ILSA) is relatively rare, ILSA is caused by the persistence of the dorsal segment of the sixth left arch, with regression of the fourth arch artery and interruption of the left dorsal aorta at the distal end of the seventh intersegmental artery on the left side during embryonic development. The left subclavian artery is connected to the pulmonary artery through an arterial duct, which can be closed or unobstructed. This abnormality can lead to congenital subclavian steal syndrome and vertebrobasilar artery insufficiency. Case presentation We reported three fetuses with ILSA and intracardiac malformation. Among them, one case was suspected to be diagnosed with ILSA by echocardiography, while the other two cases were not diagnosed, but were accidentally discovered during autopsy. We have also conducted a literature review of its prenatal screening, diagnosis, management, and outcomes. Our three cases were tested by WES‐Trio (whole exome sequencing). Worldwide, the ILSA cases reported in English literature have not been detected by WES. And likely pathogenic results were found in our two cases. Although it could not explain the intracardiac malformation we found, it will help to explore the etiology in the future. Conclusions Prenatal echocardiography detection and diagnosis of ILSA is a new challenge, which has different effects on the prognosis of the fetus. When finding intracardiac malformation with right aortic arch, we need to perform an unconventional view of ultrasound scanning and combine with CDFI to find the origin of the left subclavian artery. Although we cannot find the cause of the disease temporarily, but our genetic results can help prenatal genetic counseling.

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“…To our knowledge, only two cases of a concomitant ISA and congenital complete atrioventricular block have been reported. 17,18 These two malformations may be derived from the same genetic defect or may be regulated by the same pathway. However, the family refused further genetic testing of the child after pacemaker implantation.…”

Section: Discussionmentioning

confidence: 99%

“…An early diagnosis in such a case might avoid unnecessary surgery, such as a Blalock-Taussig operation. 17…”

Section: Discussionmentioning

confidence: 99%

Clinical and imaging characteristics of isolated subclavian artery in pediatric patients

Zhu

Zhou

et al. 2021

Clinical Imaging

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To describe clinical and imaging characteristics of an isolated subclavian artery (ISA) in pediatric patients. Background: ISA is a rare congenital aortic arch anomaly defined as a loss of connection between the subclavian artery and aorta. The clinical manifestations and complications of ISA in children are unclear. Methods: This retrospective study included clinical and imaging data of ISA patients younger than 18 years whose data were recorded in the electronic radiology database during January 2006-August 2019. Results: Of 102 enrolled patients, 59 had been diagnosed in the first year of life. The majority of the patients also had congenital heart diseases, of which tetralogy of Fallot was the most common. The vertebral artery and collateral branch of the descending aorta served as blood flow supplies in 94 and 8 patients with ISA, respectively, as confirmed using computed tomography or magnetic resonance imaging. However, the blood supply did not influence the development of ISA. Eleven patients exhibited mild or moderate stenosis of the ISA, although only two exhibited coldness or a blood pressure gradient in the upper extremities. These two symptomatic patients also presented with patent ductus arteriosus, and this association was significant (P = 0.008). Conclusion: ISA management is often determined based on symptoms and associated congenital heart diseases. The ISA is prone to stenosis in patients with ipsilateral patent ductus arteriosus. We recommend early surgical ligation or percutaneous closure of the ductus arteriosus in patients with ISA.

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Isolated Left Subclavian Artery, Complete Atrioventricular Block, and Tricuspid Atresia in a Neonate

Cited by 5 publications

References 15 publications

Isolated Left Subclavian Artery, Multiple Ventricular Septal Defects and Pulmonary Hypertension In A Child: A Case Report

Isolated Left Subclavian Artery, Multiple Ventricular Septal Defects and Pulmonary Hypertension In A Child: A Case Report

Prenatal ultrasound findings, genetic testing, and literature review of Isolated left subclavian artery

Clinical and imaging characteristics of isolated subclavian artery in pediatric patients

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