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Table of Contents   
Year : 2019  |  Volume : 12  |  Issue : 2  |  Page : 163-168
Left subclavian artery stenosis treated with transcatheter stent placement in pediatric patients

1 Department of Pediatric Cardiology, NH RTIICS, Kolkata, India
2 Department of Pediatric Cardiology, NSH, Howrah, West Bengal, India

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Date of Web Publication30-Apr-2019


Left subclavian artery (LSA) stenosis causing limb length shortening or vascular compromise or significant pulse volume and blood pressure differences between two upper limbs are rarely described in pediatric patients. Here, we are presenting three such cases, two of them were congenital and another one acquired in origin. All of them were successfully treated with transcatheter implantation of stent in LSA. Normalization of limb length was detected on follow-up.

Keywords: Left subclavian artery stenosis, left subclavian artery stenting, limb length shortening, vascular compromise

How to cite this article:
Roy M, Chattopadhyay A, Bandhyopadhyay B, Gangopadhyay D. Left subclavian artery stenosis treated with transcatheter stent placement in pediatric patients. Ann Pediatr Card 2019;12:163-8

How to cite this URL:
Roy M, Chattopadhyay A, Bandhyopadhyay B, Gangopadhyay D. Left subclavian artery stenosis treated with transcatheter stent placement in pediatric patients. Ann Pediatr Card [serial online] 2019 [cited 2020 Sep 19];12:163-8. Available from:

   Introduction Top

Subclavian artery stenosis is well described in the adult population commonly associated with atherosclerosis. Other etiologies are arteritis, inflammation due to radiation exposure, compression syndromes, fibromuslar dysplasia, and fibromatosis. Congenital origin is rarely described in literature.

Here, we are reporting three patients, one infant and two toddlers with clinically significant LSA stenosis. The infant and one of the toddlers presented with obvious left upper limb shortening due to severe congenital LSA stenosis. The third patient, a 2.5-year-old child presented with pulse volume and blood pressure (BP) differences between two upper limbs due to severe LSA stenosis as a rare postoperative complication.

   Case Reports Top

Case report 1

A 28-day-old female newborn was referred for the evaluation of incidental detection of murmur. History revealed a second gravida mother having nonconsanguineous marriage with an uneventful gestation and perinatal period. The baby weighed 2.8 kg at birth. There was a history of death of a previous baby with stigmata of multiple congenital anomalies.

The patient was nonsyndromic without external skeletal deformity and weighing 3.6 kg, length was 53 cm. Pulses were well palpable and saturation in room air was 100% in all four limbs. Except faint systolic murmur (2/6) at left supraclavicular region, clinical examinations were noncontributory.

Chest X-ray and 12-lead electrocardiogram were within normal limit. Transthoracic echocardiography detected structurally normal heart and right aortic arch (RAA) with aberrant LSA with flow turbulence across the vessel. Stenosis of LSA was suspected and multi-slice computerized tomographic (MSCT) angiography was advised. Due to the presence of RAA possibility of 22q11 micro deletion was also considered and genetic study was planned. However, the family denied any further investigation at that point of time.

Two months later, the baby was again brought to the hospital for recognizable limb length discrepancy noted by her mother [Figure 1]. She was apparently healthy baby with adequate weight gain (5 kg at 3 months). Anthropometry revealed the left upper limb, 21.4 cm (measured from tip of acromion to tip of middle finger), 8 mm shorter than the right upper limb which measured 22.2 cm. The mid arm circumference of left arm (11.5 cm) was 10 mm lesser than the right mid arm circumference (12.5 cm).
Figure 1: Left upper limb shortening (pre procedure), Case 1

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Clinically there was no obvious color or saturation differences between the two upper limbs. The only noticeable finding in the left upper limb was slightly cooler skin, low volume radial pulse, and difference in mean BP of 10 mmHg.

Urgent MSCT was done which revealed severe focal stenosis of LSA origin [Figure 2]. It was RAA and first branch being left common carotid artery, second one right common carotid artery, third one right subclavian artery (RSA) and the last branch was aberrant LSA. The absence of obvious collateral supply to the left arm was noted. The presence of Kommerell's diverticulum (KD) and vascular ring were excluded. There was no external bony compression. Normal right kidney with ectopic left kidney found as an incidental finding.
Figure 2: Computed tomography angiogram detected right aortic arch with critical narrowing of left subclavian artery just distal to its origin in Case 1

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As there was subtle sign of early ischemic changes and visible shortening of the left upper limb caused by severe focal stenosis of LSA with the absence of collateral formation, LSA stenting as limb salvage strategy and minimally invasive technique was discussed with parents and team of cardio thoracic surgeon.

After taking proper written consent from parents baby was taken into the catheterization laboratory. She was intubated and ventilated and right femoral artery (RFA) access was obtained with 4(F) short sheath (Terumo medical corporation, somerset, New Jersey), full heparinization (100 U/kg) was done and arch angiograms were obtained in PA and shallow left anterior view (LAO) which detected RAA with aberrant LSA with significant stenosis [Figure 3]. The most stenotic segment was 2 mm in width and it was 5 mm distal to the origin of LSA. 4(F) femoral artery sheath was exchange with 5(F) sheath. LSA was threaded with a 0.014 cm × 180 cm stabilizer super soft wire (Lake Region Medical, 340 lake Hazeltine Drive, chaska, USA) and 5(F) Guiding right Judkin's catheter (Cordis de Mexico S. A de C. V) was negotiated over the wire. A 3.5 mm × 16 mm (3V Krome) stent was introduced over the wire, which kept in LSA such a way that it fully covered the stenotic segment. The position of wire-stent assembly was confirmed by fluoroscopy and angiogram. After confirming satisfactory stent position it was inflated [Figure 4]. The post procedure angiogram confirmed perfect position of stent; spanning across the stenotic segment with good flow in the distal part of LSA and left axillary artery. The whole procedure was done aseptically and it was uneventful. Baby was extubated after shifting to paediatric intensive care unit and was kept on heparin (20 U/kg/h) infusion over the night. She was discharged 48 h with oral aspirin 5 mg/kg daily for the next 6 months. Most encouraging sign was normalization of temperature, pulses and BP difference in both limbs at the time of discharge.
Figure 3: Arch angiogram revealed critical narrowing of left subclavian artery just distal to its origin in Case 1

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Figure 4: Therapeutic stenting done in critical narrowing of left subclavian artery Case 1

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On 3 months follow-up, there was no noticeable temperature, pulse volume and BP differences between two upper limbs. Anthropometrically, the left upper limb (22.9 cm) is 6 mm shorter (pre-procedure was 8 mm shorter) than the right upper limb (23.5 cm) and mid upper arm circumference of the left arm (12.7 cm) is 5 mm (pre-procedure was 10 mm lesser) lesser than the right upper arm mid circumference (13.2 cm) [Figure 5]. ultrasonography (USG) of limb was done which confirmed a laminar flow pattern in LSA. The baby is being followed up for the last 4 years with satisfactory result that is characterized by the equalization of both upper limb lengths. Till date, no further therapeutic procedure has been needed.
Figure 5: Almost equalization of both limbs 3 months after procedure Case 1

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Case report 2

A 4-year-old boy presented with incidental detection of murmur. Clinical examination revealed a low volume radial pulse in the left upper limb and lower BP in same limb. The right radial pulse was normal. BP in the right arm was 92/58/68 mmHg, whereas in the left arm measured 72/55/60 mmHg. He also had 2/6 systolic murmur in the left supra-clavicular region. Anthropometrically, his height was 100 cm and left upper limb was 10 mm shorter than the right upper limb.

Echocardiography revealed structurally normal heart with RAA and aberrant LSA with flow turbulence. Computerized tomographic (CT) angiogram had done which confirmed the diagnosis. There was 70%–80% stenosis just beyond the origin of LSA. The presence of limb length discrepancy justified treatment and transcatheter placement of stent was decided.

That child was also electively intubated and ventilated after taking written consent. RFA access was obtained by 5(F) short sheath and arch angiogram was done which confirmed the diagnosis. The stenosed segment was measured 13 mm in length and 2 mm width. The stenosed segment was crossed with 5(F) Judkin right catheter and 0.025 × 260 angulated glide wire (Terumo medical corporation, somerset, New Jersey). Then, the 5(F) short sheath was upgraded to 6(F) short sheath and the 5(F) JR catheter was exchange with 6(F) guiding JR (Cordis de Mexico S. A de C. V) then 0.014 × 180 stabilizer coronary wire was parked in distal part of LSA over which racer 5 mm × 18 mm stent was positioned across the defect. The position of the stent was confirmed by check angiogram and then the stent was deployed under fluoroscopic guidance. Post procedure angiogram showed stent in situ with good flow in LSA and left axillary artery. Patient tolerated the procedure very well and was extubated on table. He was also kept on overnight heparin infusion and discharged with oral aspirin.

Follow-up visit on 3 and 6 months showed near normalization of radial pulse and BP in the left upper limb and significant improvement of limb length. USG of left upper limb was done which confirmed a laminar flow pattern in the LSA. The boy is being followed up for the last 4 years. At present, there is no remaining limb length discrepancy.

Case report 3

Two years 5 months old post cardiac surgery patient on routine follow-up was detected to have BP and pulse volume discrepancy in upper limb. She had already undergone left-sided modified Blalock Taussing shunt (BTs) followed by bidirectional Glenn (BDG) shunt as single ventricular route of palliation.

BT shunt was done at the age of 3 months followed by BDG and takedown of BTs at the age of 9 months. Her immediate and late post-operative period was uneventful. She was on regular follow-up without any significant new findings. However, at the age of 2.5 years pulse volume and BP differences between two upper limbs were documented. Length, mid arm circumference, color, saturation, temperature, and capillary refill time were same in both upper limbs.

Well-functioning BDG was detected with flow turbulence across LSA on echocardiography. LSA origin stenosis was suspected and CT angiogram was done which confirmed the diagnosis. Transcatheter placement of stent was decided as minimally invasive procedure after discussion with family and surgical team.

As like our previous patients she was also electively intubated and ventilated for the procedure. Arch angiogram confirmed severe LSA origin stenosis. The width of the stenosed segment was 3 mm and length was 18 mm.

An Abbot vascular RX herculink Elite 5 mm × 18 mm stent was deployed with satisfactory result [Figure 6] and [Figure 7].
Figure 6: Therapeutic stenting of left subclavian artery Case 3

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Figure 7: Deployment of stent in left subclavain artery Case 3

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That patient is also under follow-up for the past 4 years with clinical and USG evaluation of her left limb. At the age of 4.3 years, she also underwent Fontan completion. Till date, she is doing well both the Fontan circuit and limb perspective.

   Discussion Top

Subclavian artery stenosis is mostly described in adult population which is commonly associated with atherosclerotic changes in subclavian artery. Other etiologies are arteritis, inflammation due to radiation exposure, compression syndromes, fibromuslar dysplasia, and fibromatosis.

Ischemic changes and limb length shortening is described in newborn and early infancy as a complication of arterial cannulation induced thrombosis, with reduced pulses and temperature of concerned limb. That occurs in 2%–33% of children after arterial cannulation.[1],[2] Impaired growth has been recognised for 30 years.[3],[4] Other described aetiologies of limb length shortening includes compound fracture, disseminated sepsis, and purpura fulminance. Compound fractures can directly damage epiphysis of growth plate and may hamper limb growth in later part of life. Like our patient early chances of vascular compromise and limb length shortening due LSA stenosis congenital in origin is rarely described in literature.

A RAA represents a variant of the thoracic vascular anatomy and found in approximately 0.1% of population.[5],[6] About half of the patients have an aberrant LSA which may arise either directly from the aorta or from KD. Although an aberrant LSA may be an isolated finding, it is commonly associated with RAA. Symptoms if any are due to compression of esophagus or trachea characterized by dysphagia, airway compression which can occur in infancy or adulthood.[7],[8],[9] Such obstructive complications are rarely described in neonatal period. Management of LSA with feature of compression of oesophagus or trachea mostly requires surgical reconstruction. However, the presenting features of our cases were unique, there were no features of any obstruction of nearby structure characterized by stridor, feeding difficulties, or chocking episodes. The only symptoms were limb length shortening in two patients and temperature differences in one patient additional to limb length shortening.

Compression of the esophagus may occur when both the ligamentum arteriosum and a KD are present. The ligamentum completes the vascular ring that connects the diverticulum posteriorly behind the esophagus to the left pulmonary artery anteriorly. KD, a remnant of the left dorsal arch, is located where the aberrant LSA originates, causing external posterior compression of the esophagus.[10] In our all three cases, CT angiogram had done before intervention which also excluded KD and vascular ring. If LSA stenosis is associated with KD and vascular ring then surgical correction should be considered rather than only stenting.

Tschirch, et al. reported similar case of RAA with stenosis of aberrant LAS which was diagnosed antenatally by fetal echocardiography. That was treated with two multi-link pixel stent (8 mm × 2.5 mm) on the 18th day of life. They have electively put stent in the stenosed LSA before appearances of any clinical symptoms. Follow-up study by USG revealed significant residual stenosis and that baby needed repeat therapeutic intervention in the form of balloon dilatation of stent with success.[11]

In our patient's major stent-related complications such as thrombosis, rupture, stent migration, fracture, stent block fortunately was absent. BP discrepancies were reduced within 48 h of putting stent in LSA. Follow-up on 3, 6, and 12 months showed almost equalization of both upper limb lengths and mid arm circumference and no features of re-stenosis clinically or ultrasonographycally. There were other alternative management options such as surgical correction or balloon dilatation of stenotic segment. Only balloon dilatation might not have given satisfactory result and a chance of re-stenosis is also very high. However, percutaneous stent implantation into the stenotic subclavian artery represents a less invasive therapeutic option than surgical intervention and more rewording option than only balloon dilatation of stenotic artery.[12]

We had used non bio-absorbable stent in an infant, which may not be sufficient to her limb growth till adolescent period. In that case different surgical options like by-passing the carotid to subclavian or to the axillary artery may be considered electively in future. Literature has shown transposition of subclavian artery on to carotid artery is suitable for congenital aberrant RSA who has presented with subclavian steal syndrome in later part of life.[13]

Loss of pulse as a postcardiac surgical complication is described in literature. Known surgeries are classical BT shunt or aortic coarctation repair where subclavian artery had been used. Limb length shortening due to severe LSA stenosis after taking down of BT shunt which required stenting is exceptional in the world literature. As per the institutional policy surgeons do extra-cardiac Fontan surgery after 4 years of age with minimum body weight of 15 kg. That was the reason we had chosen stenting of LSA rather than completion of Fontan circuit and surgical correction of stenosis in same setting.

   Conclusion Top

Our case series is the first report in the world literature of successful trans-catheter treatment of severe congenital and postoperative critical LSA stenosis leading to vascular compromise and limb length shortening in pediatric age group.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Vlad P, Hohn A, Lambert EC. Retrograde arterial catheterization of the left heart. Experience with 500 infants and children. Circulation 1964;29:787-93.  Back to cited text no. 1
Desilets DT, Ruttenberg HD, Hoffman RB. Percutaneous catheterization in children. Radiology 1966;87:119-22.  Back to cited text no. 2
White JJ, Talbert JL, Haller JA Jr. Peripheral artial injuries in infants and children. Ann Surg 1968;167:757-66.  Back to cited text no. 3
Rudolph AM. Cooperative study on cardiac catheterization. Complications occurring in infants and children. Circulation 1968;37:III59-66.  Back to cited text no. 4
Victorica BE, Van Mierop LH, Elliott LP. Right aortic arch associated with contralateral congenital subclavian steal syndrome. Am J Roentgenol Radium Ther Nucl Med 1970;108:582-90.  Back to cited text no. 5
McElhinney DB, Hoydu AK, Gaynor JW, Spray TL, Goldmuntz E, Weinberg PM, et al. Patterns of right aortic arch and mirror-image branching of the brachiocephalic vessels without associated anomalies. Pediatr Cardiol 2001;22:285-91.  Back to cited text no. 6
Cinà CS, Arena GO, Bruin G, Clase CM. Kommerell's diverticulum and aneurysmal right-sided aortic arch: A case report and review of the literature. J Vasc Surg 2000;32:1208-14.  Back to cited text no. 7
Donnelly LF, Fleck RJ, Pacharn P, Ziegler MA, Fricke BL, Cotton RT, et al. Aberrant subclavian arteries: Cross-sectional imaging findings in infants and children referred for evaluation of extrinsic airway compression. AJR Am J Roentgenol 2002;178:1269-74.  Back to cited text no. 8
Bové T, Demanet H, Casimir G, Viart P, Goldstein JP, Deuvaert FE, et al. Tracheobronchial compression of vascular origin. Review of experience in infants and children. J Cardiovasc Surg (Torino) 2001;42:663-6.  Back to cited text no. 9
Zhao J, Liao Y, Gao S. Right aortic arch with retroesophageal left ligamentum arteriosum. Tex Heart Inst J 2006;33:218-21.  Back to cited text no. 10
Tschirch E, Chaoui R, Wauer RR, Schneider M, Rüdiger M. Perinatal management of right aortic arch with aberrant left subclavian artery associated with critical stenosis of the subclavian artery in a newborn. Ultrasound Obstet Gynecol 2005;25:296-8.  Back to cited text no. 11
Queral LA, Criado FJ. The treatment of focal aortic arch branch lesions with Palmaz stents. J Vasc Surg 1996;23:368-75.  Back to cited text no. 12
De Vleeschauwer P, Horsch S. Subclavian steal syndrome in a congenitaly anomalous subclavian artery: A case report. Ann Vasc Surg 1986;1:389-91.  Back to cited text no. 13

Correspondence Address:
Dr. Mahua Roy
931, Jawpur Road, Kolkata - 700 074, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/apc.APC_128_18

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]