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Percutaneous closure of a left atrio-ventricular valve paravalvular leak with the Occlutech Paravalvular Leak Device in a 5-year-old child

Published online by Cambridge University Press:  01 December 2025

Natalie SoszynOpen the ORCID record for Natalie Soszyn[Opens in a new window],
Jenny E. Zablah and
Gareth J. Morgan
Natalie Soszyn*
Affiliation:
Division of Cardiology, Department of Pediatrics, Heart Institute, Children’s Hospital Colorado, University of Colorado Denver | Anschutz Medical Campus, Aurora, CO, USA
Jenny E. Zablah
Affiliation:
Division of Cardiology, Department of Pediatrics, Heart Institute, Children’s Hospital Colorado, University of Colorado Denver | Anschutz Medical Campus, Aurora, CO, USA
Gareth J. Morgan
Affiliation:
Division of Cardiology, Department of Pediatrics, Heart Institute, Children’s Hospital Colorado, University of Colorado Denver | Anschutz Medical Campus, Aurora, CO, USA
*
Corresponding author: Natalie Soszyn; Email: nsoszyn@hotmail.com
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Abstract

Since 2014, transcatheter paravalvular leak closure with the Occlutech Paravalvular Leak Device has been successfully accomplished in adults with high technical success. We describe the first successful use of the Occlutech Paravalvular Leak Device in the left atrio-ventricular valve in the United States in a 5-year-old child with a history of previously repaired atrio-ventricular septal defect.

Keywords

Percutaneous closureparavalvular leakmulti-modality imaging

Information

Type
Case Report
Information
Cardiology in the Young , Volume 35 , Issue 11 , November 2025, pp. 2406 - 2409
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Copyright
© The Author(s), 2025. Published by Cambridge University Press

Introduction

Paravalvular leak is a known complication following prosthetic valve implantation, with an incidence of 5–17% in mechanical valve recipients.Reference Calvert, Northridge and Malik1–Reference Onorato, Alamanni and Muratori4 While many patients with paravalvular leaks remain asymptomatic, clinically significant leaks can lead to heart failure and haemolysis necessitating re-intervention. Surgical repair has been the standard approach for clinically significant paravalvular leak.Reference Calvert, Northridge and Malik1,Reference Onorato, Alamanni and Muratori4,Reference Goktekin, Vatankulu and Ozhan5 However, redo-surgery for paravalvular leaks carries significant mortality and morbidity. With the development of implantable devices combined with advances in imaging and the accumulation of experience, percutaneous paravalvular leak closure, first performed in 1992, has emerged as an attractive, less invasive alternative in those at high risk for surgery.Reference Calvert, Northridge and Malik1,Reference Hourihan, Perry, Mandell, Keane, Rome and Bittl6

In paediatric patients, particularly those with congenital cardiac problems, managing paravalvular leak presents unique challenges due to smaller anatomy and morphological complexity, combined with already limited device options. Device selection remains critical to ensure procedural success while minimising potential complications such as embolisation or residual shunting propagating haemolysis. Since 2014, transcatheter paravalvular leak closure with the Paravalvular Leak Device (Occlutech, GmbH, Jena, Germany) has been successfully accomplished with high technical success, mirrored by significant clinical improvement following Paravalvular Leak Device (PLD) implantation.Reference Onorato, Alamanni and Muratori4,Reference Goktekin, Vatankulu and Ozhan5,Reference Jiménez-Valverde, Escutia-Cuevas, Aceves-Millán, Fuentes-Espinoza, Pérez-Siller and Espinoza-Rueda7–Reference Onorato, Alamanni and Muratori9 The creation of an international expert group, on-going elaboration of recommendations, and the set-up of an international registry have driven transcatheter paravalvular leak closure with this device in adults; however, literature on its use in paediatrics is absent.

We describe the first successful use of the Occlutech Paravalvular Leak Device in the left atrio-ventricular valve in the United States, and the first reported use in a child -a 5-year-old with a history of repaired atrio-ventricular septal defect followed by mechanical valve replacement.

Case report

A 5-year-old, 16.6-kilogram girl presented with severe left atrio-ventricular valve regurgitation and atrial septal patch dehiscence requiring replacement with a 23 mm St Jude mechanical valve and re-do atrial septal defect patch repair at 15 months. She had a history of a repaired atrio-ventricular septal defect at 1 year of age in India. Her post-operative course was complicated by endocarditis treated conservatively with antibiotics, mechanical haemolysis, complete heart block requiring pacemaker insertion, and fundal ulceration secondary to oesophagitis due to antiplatelet use.

At discharge, her echocardiogram demonstrated an increased gradient, suggesting stenosis of the mechanical left atrio-ventricular valve (mean gradient 12 mmHg). This was in the setting of severe paravalvular leak at the antero-lateral aspect of the left atrio-ventricular valve annulus. Over the following years, despite optimal medical therapy, she deteriorated clinically with poor growth and reduced exercise capacity. Her echocardiogram demonstrated progressive left ventricular dilatation secondary to left atrio-ventricular valve stenosis and severe paravalvular leak. As she was a high-risk surgical candidate following multiple previous sternotomies and endocarditis, the decision was made following a multi-disciplinary discussion for transcatheter closure of her paravalvular leak.

The procedure was performed with trans-oesophageal and fluoroscopic guidance (Figure 1A). Access was obtained in the right femoral vein with an 8 Fr sheath. A right heart catheterisation was initially performed in standard fashion, which demonstrated a mean left atrial pressure (via pulmonary venous wedge) of 17 mmHg. Her trans-oesophageal echocardiogram (TOE) demonstrated a crescentic paravalvular leak approximately 10 mm long with a maximal width of approximately 3 mm. It appeared to be bounded by the edge of the valve sewing ring. Transeptal puncture to the left atrium was performed with a BRK needle via an 8 Fr SL1 sheath under TOE guidance. A 4 Fr JR catheter was used to advance a 0.035” Glide wire across the paravalvular defect. This was then exchanged for a 0.035” Ampltazer SuperStiff wire over which both the catheter and SL1 sheath were removed and replaced with an 8 Fr Abbott TorqVue sheath, which was positioned in the left ventricle. A 10 mm × 4 mm “waisted” Occlutech Paravalvular Leak Device was selected. The rationale for this size was to fill as much of the crescentic section as possible without risking too large a disc, which could compromise prosthetic leaflet motion. Using three-dimensional TOE guidance, the device was positioned across the defect and deployed using trans-oesophageal and fluoroscopic guidance to ensure that the long axis orientation of the device lay along the long axis of the crescentic paravalvular leak (Figures 1B and C). A repeat right heart catheterisation was performed at the end of the case, demonstrating an improvement in mean left atrial pressure to 10 mmHg. The post-procedural TOE showed only a trivial residual leak and no interference with the prosthetic valve leaflets.

Figure 1.

Three-dimensional (3D) trans-esophageal echocardiography (TOE) demonstrating A. location of the paravalvular defect (yellow) which is anterior and lateral to the mechanical (23 mm St Jude) valve (red) B. PLD (blue) in relation to the mechanical valve after implantation. C. Fluoroscopy demonstrating stable device position across the paravalvular defect post-release.

Following the procedure, the patient was monitored for 24 hours and then discharged on her chronic anticoagulation (warfarin). At 6-month follow up, she had improved exercise tolerance and growth with no residual paravalvular leak, improved left atrio-ventricular valve gradient (5 mmHg) (due presumably to normalisation of the antegrade mitral valve flow volume), and mild left ventricular dilatation with low normal systolic function.

Discussion

Paravalvular leaks occur around surgically or percutaneously implanted valves because of an incomplete seal between the sewing ring of the prosthetic valve and the native annulus or valve dehiscence, which can be a sequela of endocarditis. Occurring in 6–32% of cases, paravalvular leaks more commonly occur around the mitral valve and when a mechanical valve is placed.Reference García, Arzamendi and Jimenez-Quevedo2,Reference Onorato, Muratori, Smolka, Malczewska, Zorinas and Zakarkaite8 Symptomatic heart failure or refractory mechanical haemolytic anaemia subsequently develops in 1–5% of patients with paravalvular leak.Reference García, Arzamendi and Jimenez-Quevedo2,Reference Onorato, Alamanni and Muratori9 The high rate of mortality and morbidity following conventional re-do surgery, the burden of complicated post-operative care, and the rapid development of catheter-based procedures have motivated medical professionals, together with industry partners, to introduce a less invasive treatment strategy into clinical practice.

Self-expanding devices, such as the Ampltazer vascular plug (AVP II/III/IV), duct occluders (ADO I/II), atrial septal occluders, and muscular ventricular septal defect occluders, are often re-purposed and used off-label depending on the patient’s anatomy.Reference Onorato, Alamanni and Muratori4,Reference Onorato, Muratori, Smolka, Malczewska, Zorinas and Zakarkaite8 The Occlutech Paravalvular Leak Device (PLD) is the first device to be designed specifically for paravalvular leak closure. Although widely used in EuropeReference Calvert, Northridge and Malik1, the application of the Occlutech PLD in the United States remains limited to compassionate use cases. The PLD obtained European Conformité Européene mark approval in 2014 but does not have US Food and Drug Administration Pre-market Approval. To date, only a few studies of mitral paravalvular leak closure using this device have been published; none have reported its use in children.Reference Bedair, Morgan and Bapat3–Reference Goktekin, Vatankulu and Ozhan5,Reference Jiménez-Valverde, Escutia-Cuevas, Aceves-Millán, Fuentes-Espinoza, Pérez-Siller and Espinoza-Rueda7–Reference Onorato, Alamanni and Muratori9 It is a two-disc device, with each disc containing a polyethylene terephthalate patch, linked by a nitinol connection in either a waist (broad) or twist (narrow) configuration (Figure 2). The twist design provides greater conformability to defect shape and is preferred for smaller defects, while the waist design is more suitable for large defects. Considering the heterogenicity and complexity of paravalvular leak geometries, the availability of a range of Paravalvular Leak Device sizes, shapes, and waists is important for achieving procedural success. The device’s flexible nitinol structure and adaptive design make it particularly suited for irregularly shaped paravalvular leaks, which are common in post-surgical patients. In this case, the waisted Occlutech PLD was selected over the twisted design. This allowed us to use the expanded waist as the primary closure point while minimising the disc sizes which was important in this smaller anatomy to decrease the risk of interference with the valve leaflets. Our successful implantation demonstrates that the Occlutech PLD can be effectively used in a small child with a mechanical left atrio-ventricular valve, despite anatomical and procedural challenges.

Figure 2.

Illustrations of the technical characteristics of the square shaped and rectangular shaped Occlutech paravalvular leak device (PLD) in the two different disc connections.

One of the primary concerns in transcatheter paravalvular leak closure is ensuring a secure seal while minimising interference with valve function. In this case, pre-procedural imaging was crucial for selecting an appropriately sized device and determining the optimal approach. The use of three-dimensional echocardiography in combination with fluoroscopy facilitated precise positioning, reducing the risk of embolisation or residual shunting.

Despite the successful outcome, there are still several future considerations. The long-term durability of transcatheter paravalvular leak closure in a paediatric patient has not been established. Somatic and cardiac growth may have an effect on the performance of the device and its interaction with the valve leaflets. Future studies are required to evaluate the long-term durability and outcomes of this device in paediatric patients over time.

Conclusion

We demonstrate the case of a 5-year-old girl with a previous history of a repaired atrio-ventricular septal defect followed by mechanical valve replacement, who underwent successful paravalvular leak closure of a left atrio-ventricular valve paravalvular leak with an Occlutech Paravalvular Leak Device. This case highlights the feasibility, challenges, and outcomes associated with the use of the Occlutech Paravalvular Leak Device in a paediatric patient, providing insights into its potential role in managing paravalvular leaks in younger populations.

Acknowledgements

None.

Financial support

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

Competing interests

We have adhered to a strict confidentiality policy regarding this case report. No identifying information is present within this case. Dr Gareth J Morgan and Dr Jenny E Zablah are both consultants for Occlutech.

References

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Figure 0

Figure 1.Three-dimensional (3D) trans-esophageal echocardiography (TOE) demonstrating A. location of the paravalvular defect (yellow) which is anterior and lateral to the mechanical (23 mm St Jude) valve (red) B. PLD (blue) in relation to the mechanical valve after implantation. C. Fluoroscopy demonstrating stable device position across the paravalvular defect post-release.

Figure 1

Figure 2.Illustrations of the technical characteristics of the square shaped and rectangular shaped Occlutech paravalvular leak device (PLD) in the two different disc connections.