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Percutaneous treatment of aortic isthmus atresia with begraft covered stent in a 25 kg children: a case report

Published online by Cambridge University Press:  14 April 2025

G. GiustiOpen the ORCID record for G. Giusti[Opens in a new window],
A. BustiOpen the ORCID record for A. Busti[Opens in a new window] and
M. Papa
G. Giusti*
Affiliation:
Pediatric Cardiology Unit, Niguarda Hospital, Milan, Italy
A. Busti
Affiliation:
Pediatric Cardiology Unit, Niguarda Hospital, Milan, ItalySchool of Medicine and Surgery, Milano-Bicocca University, Milan, Italy
M. Papa
Affiliation:
Pediatric Cardiology Unit, Niguarda Hospital, Milan, Italy
*
Corresponding author: Giuliano Giusti; E-mail: giuliano.giusti@ospedaleniguarda.it
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Abstract

We report the case of a 25 kg male with aortic isthmus atresia and small femoral arterial access. A BeGraft covered stent was percutaneously implanted after perforating the atretic segment. This case demonstrates the feasibility and safety of transcatheter treatment using the Bentley stent, which enables smaller femoral access compared to other balloon-expandable stent platforms.

Keywords

atresic aortic isthmustranscatheter interventioncovered stentaortic coartation

Information

Type
Brief Report
Information
Cardiology in the Young , Volume 35 , Issue 5 , May 2025, pp. 1070 - 1072
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Copyright
© The Author(s), 2025. Published by Cambridge University Press

Introduction

Case presentation

The patient is an 11-year-old male, 139 cm x 25 kg, presenting with severe hypertension and reduced exercise tolerance. The patient came to our attention with no significant surgical history. Upon examination, the patient exhibited severe hypertension (BP 160/100 mmHg) with a significant pressure gradient between the upper and lower extremities (40 mmHg). Cardiac auscultation revealed a systolic–diastolic murmur graded 2–3/6. The baseline ECG showed signs of left atrial enlargement and left ventricular hypertrophy. The baseline echocardiogram indicated a mildly dilated left ventricle with increased wall thickness and preserved global contractility, a bicuspid aortic valve with mild stenosis, and a dysplastic mitral valve with a single papillary muscle presenting moderate stenosis. The aortic arch appeared gothic and showed severe reduction in calibre at the isthmus.

A thoracic CT angiography was performed, which showed a left aortic arch with an acute-angled appearance and the usual origin of the epi-aortic vessels. The presence of aortic isthmus atresia was confirmed. Marked and hypertrophic collateral circulation at the level of the thoracic and mammary arteries was seen (Figure 1a–b).

The case was discussed in our multidisciplinary team, and we decided to perform cardiac catheterisation to attempt to recanalise the aortic isthmus.

The procedure was performed in general anaesthesia and endotracheal intubation, on standard anticoagulation with Unfractioned heparin administrate to mantain an activated clotting time of 250–300 s.

Using the echoguided Seldinger technique, the right femoral artery was cannulated with a 6 Fr introducer (Radifocus, Terumo), and the left radial artery was cannulated with a 4 Fr hydrophilic introducer (Radifocus, Terumo). A Perclose ProStyle closure system (Abbott) was pre-implanted in the femoral artery. An arteriography was performed from the femoral introducer: the diameter of the common femoral artery was measured to be 4 mm.

An aortogram was performed with contrast injection (via GlideCath Angled 4 Fr, Terumo ) from radial access, and no direct anterograde flow was observed, with the presence of a well-defined tapered proximal cap.

A simultaneous injection at the level of the descending thoracic aorta via retrograde femoral access (using a SUPERTORQUE Plus Diagnostic Catheter 5.2 Fr, Cordis) demonstrated proximity between the aortic arch and the descending thoracic aorta (Figure 2a–b). The haemodynamic assessment in the descending thoracic aorta showed a flattened pressure curve without significant pulsatility, with a significant pressure gradient between the aortic arch and the descending thoracic aorta (35 mmHg). The diagnosis of aortic isthmus atresia was confirmed.

The minimal diameter of the distal transverse arch measured 10 mm on the preoperative CT scan, which corresponds to a Z-score of −2.8.

We attempted to recanalise the aortic isthmus from above using coronary guidewires and anterograde guidewire escalation technique. The first attempt of perforation with a workhorse 0.014″ straight Sion Blue (ASASHI Intecc) guidewire failed. Then, a coronary chronic total occlusion guidewire, a 0.014″ Fielder XT-A (Asahi Intecc), was employed, successfully perforating the aortic isthmus, crossing into the descending aorta (Figure 2c).

Then, an arterial-arterial loop was established by externalising the guidewire from the femoral access using a snare technique (Amplatzer GooseNeck Snare Kit, Medtronic) (Figure 2d). To facilitate the smooth passage of materials, a pre-dilatation of the recanalised aortic isthmus was performed using a 2x15 mm semi-compliant Ryurei (Terumo) coronary balloon on the 0.014″ guidewire (Figure 2e).

A 6 Fr multipurpose catheter was advanced from the femoral vascular access to the level of the aortic arch. The arterial–arterial loop was then resolved, and a supportive 0.035″ guidewire (Amplatzer, Medtronic) was placed in the ascending aorta.

A 10 Fr GoreDrySeal Flex long sheath (Gore) was advanced via the femoral access to the level of the transverse aortic arch (Figure 2f).

Under the guidance of multiple angiographies (performed either through the radial and femoral access), a covered BeGraft stent 12 x 29 mm (Bentley) was implanted at the level of the aortic isthmus, reconstructing aortic continuity (Figure 2f). The stent was post-dilated with the same balloon used for the implantation up to 8 atmospheres of pressure, with complete resolution of the waist on the stent:

The final angiography demonstrated properly positioned stent, with no angiographically detectable complications (Figure 2g). The pressure gradient at the catheter pullback dropped to 4 mmHg, localised at the level of the transverse arch, with no gradient at the site of the implanted stent.

The femoral access was closed with the pre-implanted Perclose ProStyle closure system (Abbott). The radial access was closed using a TrBand closure system (Terumo).

The patient was discharged home 3 days after the procedure.

Discussion

Aortic isthmus atresia, or atretic coarctation, is defined as a severe form of aortic coarctation characterised by a complete luminal obstruction of the aortic isthmus despite the anatomical continuity between the aortic isthmus and the descending thoracic aorta. Reference Joseph, Mandalay and Rajendiran1

Figure 1.

( a ) Multiplanar reconstruction image of pre-intervention CT angiography of the chest in a parasagittal plane showing complete vascular lumen occlusion at the aortic isthmus level. ( b ) 3D rendering reconstruction image of the pre-intervention CT angiography of the chest demonstrating the aortic arch with usual origin of the epi-aortic vessels and complete obstruction at the aortic isthmus, with anatomical continuity between the aortic isthmus and the descending thoracic aorta. These findings are consistent with aortic isthmus atresia. ( c ) Multiplanar reconstruction image of the post-intervention CT angiography of the chest in a parasagittal plane C: Multiplanar reconstruction image of the post-intervention CT angiography of the chest in a parasagittal plane shows the stent well-positioned at the isthmic level with no evidence of residual narrowing. A minimal protrusion of the most proximal portion at the origin of the left subclavian artery can be seen, although it does not cause a significant reduction in caliber ( d ) 3D rendering reconstruction image of the post-intervention CT angiography of the chest shows the stent positioned at the desired location at the aortic isthmus, effectively resolving the obstruction at the isthmic site.

Figure 2.

Figure ( a - h ): The images depict the different steps of the transcatheter recanalization procedure and stent implantation in the context of aortic isthmus atresia. ( a ) Through a left radial vascular access, a 4Fr Terumo Glidecath hydrophilic angled catheter was used to perform an angiography from above, which did not show anterograde flow but revealed the presence of a well-defined tapered proximal cap. ( b ) A simultaneous angiography from below through the femoral vascular access using a 5.2Fr Cordis MP catheter showed anatomical continuity between the aortic arch, the aortic isthmus, and the descending thoracic aorta. The image also revealed a dilated and tortuous segment of the left internal mammary artery. ( c ) From the left radial access, using the same 4Fr Terumo Glidecath catheter, several attempts were made to recanalize the complete occlusion at the  level of the aortic isthmus using an anterograde guidewire escalation approach. A coronary chronic total occlusion guidewire, a 0.014’’ Fielder XT-A (Asahi Intecc), was employed, successfully perforating the aortic isthmus and allowing the wire to pass into the descending aorta. ( d ) An arterial-arterial loop was then established by externalizing the Fielder XT-A from the femoral access using a snare technique (with the Amplatzer GooseNeck Snare Kit, Medtronic). ( e ) To facilitate the smooth passage of materials, a pre-dilatation of the recanalized aortic isthmus was performed using a 2x15mm semi-compliant Ruyrei coronary balloon on a 0.014’’ guidewire. ( f ) Subsequently, it was possible to advance a 6Fr MP catheter from below, through the femoral vascular access, over the 0.014’’ guidewire. The arterial-arterial loop was then resolved, and a supportive 0.035’’ guidewire (Amplatzer, Medtronic) was placed in the ascending aorta. A 10 Fr GoreDrySeal Flex long sheath (Gore) was advanced via the femoral access over the supportive 0.035’’ guidewire to the level of the transverse aortic arch ( g ) Under the guidance of multiple angiographies performed through the radial access and the femoral long introducer, a covered Bentley stent (12 x 29 mm) was successfully implanted at the level of the aortic isthmus. ( h ) Final angiography demonstrates the stent implated in the intended position with no angiographically detectable complication and  restored aortic continuity.

Multiple case reports have demonstrated the feasibility and safety of transcatheter treatment of aortic isthmus atresia using covered stent implantation in the adult population. Reference Chessa, Favoccia and Jha2,Reference Butera, Heles and Carminati3

However, such treatment for this congenital defect may be limited in the paediatric population due to the need for large femoral arterial vascular sheaths, which require adequate vessel. It is well known that a high ratio between the size of the arterial vascular access and the vessel is associated with increased risk of vascular complications. Reference Mehta, Lee, Chaturvedi and Benson4

The Bentley BeGraft is a cobalt-chromium aortic stent graft system covered with an expanded polytetrafluoroethylene (ePTFE) tube, indicated for the treatment of native coarctation of the aorta in adolescent or adult patients.

The BeGraft stent, with a diameter of 12 mm, is compatible with a 9 Fr introducer sheath and offers the option for post-dilatation up to a maximum diameter of 20 mm.

Compared to other balloon-expandable covered stents, such as the NUMED Mounted Covered CP Stent, which requires a 12 Fr introducer for the same diameter, the BeGraft stent requires a smaller introducer size. We deliver it using a 10 Fr Gore DrySeal Flex introducer sheath, which, due to its hydrophilic coating and flexibility, allows easy access to challenging anatomies. However, the braided nature of the introducer results in an outer diameter of 4 mm, and this must be kept in mind in children with small vessels. We recognise that a backup vascular surgeon team is mandatory for these high-risk procedures.

In our opinion, this characteristic of the Bentley stent enhances the feasibility of transcatheter treatment for aortic isthmus coarctation in paediatric patients, who generally have smaller femoral artery access.

Previous case series have demonstrated the safety and feasibility of the Bentley stent in coarctation and near-atretic aortic coarctation, and our manuscript describes the use of this stent in patients with aortic isthmus atresia. Reference Al Balushi, Pascall and Jones5,Reference Rebonato, Pilati and Milani6

Our case supports the feasibility and safety of transcatheter treatment of atretic aortic isthmus with a covered stent in a paediatric patient (25 kg, 12 years old) with small femoral arterial vascular access (Figure 1c–d). The use of the Bentley stent has allowed for the use of smaller femoral arterial vascular access compared to other balloon-expandable stent platforms.

Financial support

This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.

Competing interests

None.

References

Joseph, G, Mandalay, A, Rajendiran, G. Percutaneous recanalization and balloon angioplasty of congenital isolated local atresia of the aortic isthmus in adults. Catheter Cardio Inte 2001; 53: 535–541.Google ScholarPubMed
Chessa, M, Favoccia, C, Jha, NK, et al. Long - term follow - up after recanalisation of aortic arch atresia. EuroIntervention 2021; 16 ( 15 ): e1274–e1280.CrossRefGoogle ScholarPubMed
Butera, G, Heles, M, Carminati, M. Percutaneous treatment of aortic isthmus atresia. Catheter Cardio Inte 2011; 78: 933–939.Google ScholarPubMed
Mehta, R, Lee, KJ, Chaturvedi, R, Benson, L. Complications of pediatric cardiac catheterization : a review in the current era. Catheter Cardio Inte 2008; 72: 278–285.Google ScholarPubMed
Al Balushi, A, Pascall, E, Jones, MI, et al. Initial experience with a novel ePTFE - covered balloon expandable stent in patients with near - atretic or severe aortic coarctation and small femoral arterial access. Cardiol Young 2021; 31 : 224–228.CrossRefGoogle ScholarPubMed
Rebonato, M, Pilati, M, Milani, SM, et al. BeGraft aortic stents: a european multi - centre experience reporting acute safety and efficacy outcomes for the treatment of vessel stenosis in congenital heart diseases. J Cardiovasc Dev Dis 2024; 11: 192.Google ScholarPubMed
Figure 0

Figure 1.(a) Multiplanar reconstruction image of pre-intervention CT angiography of the chest in a parasagittal plane showing complete vascular lumen occlusion at the aortic isthmus level. (b) 3D rendering reconstruction image of the pre-intervention CT angiography of the chest demonstrating the aortic arch with usual origin of the epi-aortic vessels and complete obstruction at the aortic isthmus, with anatomical continuity between the aortic isthmus and the descending thoracic aorta. These findings are consistent with aortic isthmus atresia. (c) Multiplanar reconstruction image of the post-intervention CT angiography of the chest in a parasagittal plane C: Multiplanar reconstruction image of the post-intervention CT angiography of the chest in a parasagittal plane shows the stent well-positioned at the isthmic level with no evidence of residual narrowing. A minimal protrusion of the most proximal portion at the origin of the left subclavian artery can be seen, although it does not cause a significant reduction in caliber (d) 3D rendering reconstruction image of the post-intervention CT angiography of the chest shows the stent positioned at the desired location at the aortic isthmus, effectively resolving the obstruction at the isthmic site.

Figure 1

Figure 2.Figure (a-h): The images depict the different steps of the transcatheter recanalization procedure and stent implantation in the context of aortic isthmus atresia. (a) Through a left radial vascular access, a 4Fr Terumo Glidecath hydrophilic angled catheter was used to perform an angiography from above, which did not show anterograde flow but revealed the presence of a well-defined tapered proximal cap. (b) A simultaneous angiography from below through the femoral vascular access using a 5.2Fr Cordis MP catheter showed anatomical continuity between the aortic arch, the aortic isthmus, and the descending thoracic aorta. The image also revealed a dilated and tortuous segment of the left internal mammary artery. (c) From the left radial access, using the same 4Fr Terumo Glidecath catheter, several attempts were made to recanalize the complete occlusion at the  level of the aortic isthmus using an anterograde guidewire escalation approach. A coronary chronic total occlusion guidewire, a 0.014’’ Fielder XT-A (Asahi Intecc), was employed, successfully perforating the aortic isthmus and allowing the wire to pass into the descending aorta. (d) An arterial-arterial loop was then established by externalizing the Fielder XT-A from the femoral access using a snare technique (with the Amplatzer GooseNeck Snare Kit, Medtronic). (e) To facilitate the smooth passage of materials, a pre-dilatation of the recanalized aortic isthmus was performed using a 2x15mm semi-compliant Ruyrei coronary balloon on a 0.014’’ guidewire. (f) Subsequently, it was possible to advance a 6Fr MP catheter from below, through the femoral vascular access, over the 0.014’’ guidewire. The arterial-arterial loop was then resolved, and a supportive 0.035’’ guidewire (Amplatzer, Medtronic) was placed in the ascending aorta. A 10 Fr GoreDrySeal Flex long sheath (Gore) was advanced via the femoral access over the supportive 0.035’’ guidewire to the level of the transverse aortic arch (g) Under the guidance of multiple angiographies performed through the radial access and the femoral long introducer, a covered Bentley stent (12 x 29 mm) was successfully implanted at the level of the aortic isthmus. (h) Final angiography demonstrates the stent implated in the intended position with no angiographically detectable complication and  restored aortic continuity.