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Transcatheter patent ductus arteriosus closure in a syndromic patient with interrupted inferior vena cava: overcoming anatomical challenges

Published online by Cambridge University Press:  21 March 2025

Ilker Ufuk Sayici Open the ORCID record for Ilker Ufuk Sayici [Opens in a new window] ,
Pelin Altinbezer Open the ORCID record for Pelin Altinbezer [Opens in a new window]  and
Utku Arman Örün Open the ORCID record for Utku Arman Örün [Opens in a new window]
Ilker Ufuk Sayici*
Affiliation:
Pediatric Cardiology Department, Etlik City Hospital, Ankara, Turkey
Pelin Altinbezer
Affiliation:
Pediatric Cardiology Department, Etlik City Hospital, Ankara, Turkey
Utku Arman Örün
Affiliation:
Pediatric Cardiology Department, Etlik City Hospital, Ankara, Turkey
*
Corresponding author: Ilker Ufuk Sayici; Email: usayici@hotmail.com
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Abstract

This case report discusses a 1-year-old female with severe growth retardation and multiple congenital anomalies, including a large patent ductus arteriosus and interrupted inferior vena cava. Successful percutaneous patent ductus arteriosus closure was achieved via the azygos venous route, overcoming anatomical challenges and highlighting the importance of individualized procedural strategies in complex cases.

Keywords

Patent ductus arteriosusskeletal dsyplasiaİnterrupted inferior vena cava

Information

Type
Brief Report
Information
Cardiology in the Young , Volume 35 , Issue 5 , May 2025 , pp. 1076 - 1078
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Copyright
© The Author(s), 2025. Published by Cambridge University Press

Introduction

Patent ductus arteriosus (PDA) is a common congenital heart defect, accounting for 5–10% of all congenital heart defects. Reference S.C., S.B. and H.W.1 It is characterized by the persistence of a fetal blood vessel that connects the pulmonary artery to the descending aorta, which normally closes soon after birth. The introduction of percutaneous closure techniques has greatly improved outcomes, especially in patients who are not good surgical candidates. Reference Porstmann2 However, the closure of PDA in patients with additional congenital anomalies, such as interrupted inferior vena cava (IVC) and complex skeletal deformities, presents unique challenges. This report details the case of a 1-year-old female patient with multiple congenital anomalies, including a large PDA, multiple small muscular ventricular septal defects (VSDs), interrupted IVC, growth retardation, chronic diarrhea, cranio-caudal dysgenesis, sacral agenesis, and skeletal dysplasia. The patient weighed 5 kg at the time of presentation and had significant growth and developmental delays.

Patient presentation

Our patient is a a 1-year-old female, presented with severe growth retardation, weighing only 5 kg. She had a complex medical history, including chronic diarrhea, cranio-caudal dysgenesis, sacral agenesis, and skeletal dysplasia (Figure 1). The skeletal dysplasia contributed to difficulties with motor development and caused significant challenges during percutaneous procedures, particularly regarding femoral venous access. Echocardiography revealed a large PDA and multiple small muscular VSDs, with an associated interrupted IVC and azygos continuation of venous return. Given the complexity of her anatomy and her underlying health conditions, a non-surgical approach to PDA closure was preferred.

Figure 1.

Displastic lower extremities of the patient, illustrating significant skeletal dysplasia.

Method

Initially, access was obtained via the left femoral vein. However, due to the interrupted IVC, the catheter could not be advanced through the usual venous route to the right atrium (Figure 2a). Venous ultrasonography and angiography confirmed that the venous return was rerouted through the azygos vein into the superior vena cava (SVC). As a result, we opted to continue the catheterization through the azygos vein (Figure 2b). The catheter was successfully advanced through the azygos vein, into the SVC, and subsequently into the right atrium, right ventricle, pulmonary artery, and across the PDA into the descending aorta (Figure 2c).

Figure 2a.

Venogram showing the interrupted IVC with venous return rerouted through the azygos vein.

Figure 2b.

Catheter advanced through the azygos vein into the superior vena cava (SVC), continuing to the right atrium.

Figure 2c.

Catheter successfully crossing the patent ductus arteriosus (PDA) from the pulmonary artery to the descending aorta.

While a retrograde approach via the femoral artery is commonly used in the literature for PDA closure, it was not preferred in this case due to the patient’s small size and associated skeletal dysplasia, which posed technical challenges for arterial access and catheter manipulation. The antegrade approach via the azygos vein was chosen to minimize procedural risks and ensure device placement.

Given the presence of multiple congenital anomalies and the significant left-to-right shunting caused by the wide PDA, a catheter-based intervention was planned. Due to the echocardiographic assessment of Qp/Qs > 1.5 and the hemodynamically insignificant nature of the muscular VSDs, a detailed hemodynamic study was deemed unnecessary during catheter angiography. The procedure focused on addressing the hemodynamic burden caused by the PDA.

Once the catheter was in place, a guidewire was introduced, and the delivery system for a duct occluder device was deployed. Due to the patient’s small size and anatomical complexity, careful attention was paid to avoid complications such as device embolization or hemodynamic instability. After successfully advancing the catheter through the azygos system, a second catheter was introduced through the aorta to facilitate the precise placement of the 6x4 mm Amplatzer Ductal Occluder II (Figure 2d). A post-deployment angiogram confirmed the device was securely positioned, with no residual shunting across the PDA.

Figure 2d.

Post-deployment angiogram confirming the secure positioning of the 6x4 mm Amplatzer Ductal Occluder II with no residual shunt across the PDA.

Results

The procedure was completed successfully, and the patient’s hemodynamic status remained stable throughout. Post-deployment echocardiography and angiography showed complete closure of the PDA, with no evidence of residual flow. The patient tolerated the procedure well, and there were no immediate complications, such as device embolization, vascular injury, or arrhythmias. Her post-operative recovery was uneventful, and follow-up imaging confirmed the correct placement and stability of the occluder device. Given the complexity of her skeletal and vascular anatomy, the successful use of the azygos venous route was crucial in overcoming the challenges posed by her interrupted IVC.

Discussion

The closure of PDA in patients with interrupted IVC and other congenital anomalies, such as skeletal dysplasia, presents significant technical challenges. In this patient, traditional percutaneous femoral venous access was initiated through the left femoral vein, but the interrupted IVC prevented further advancement of the catheter through the usual pathway. Interrupted IVC occurs in approximately 0.1% of the population, often in association with left isomerism or complex congenital heart conditions. Reference Koc and Oguzkurt3 In our patient, venous return through the azygos vein allowed us to successfully continue the procedure via the azygos venous route.

In the literature, a total of 10 cases and a case series involving four patients with interrupted IVC who underwent transcatheter PDA closure have been reported. Reference Al-Hamash4–Reference Koh14 Of these, five cases were performed using a retrograde approach through the femoral artery, Reference Çelebi6,Reference Sivakumar and Francis11,Reference Rajan13,Reference Koh14 while the remaining five were done using an antegrade approach through the internal jugular vein. Reference Akhtar, Samad and Atiq5,Reference Subramanian8–Reference Jawahirani10,Reference Patel12 Due to the technical difficulties associated with catheter manipulation and the advancement of the ductal occluder device in patients with interrupted IVC, only four cases have been successfully completed using the femoral vein for antegrade closure. Reference Al-Hamash4,Reference Tefera and Bermudez-Cañete7,Reference Rajan13 One of these cases resulted in device embolization into the right pulmonary artery Reference Rajan13

In our case, the challenges of skeletal dysplasia further complicated femoral access. The abnormal development of the pelvic bones and femoral structures made catheter manipulation challenging, increasing the risk of vascular injury or procedural failure. By carefully advancing the catheter through the azygos vein into the superior vena cava (SVC), we were able to bypass the interrupted IVC and achieve successful device placement. The azygos venous route has proven to be an effective alternative when traditional venous access routes are obstructed or unavailable, as demonstrated by this case.

Although complications such as device embolization and hemodynamic instability are risks in these procedures, advancements in device technology have minimized these complications. In particular, the use of flexible, modern occlusion devices facilitated the safe deployment of the device in our patient without causing any hemodynamic disturbances. Given that one of the previously reported cases of antegrade femoral venous closure resulted in device embolization, our success using the azygos venous route underscores the importance of individualized procedural strategies based on each patient’s unique anatomy.

In conclusion, the management of PDA in patients with interrupted IVC, skeletal dysplasia, and other congenital anomalies requires a highly individualized approach. The challenges posed by abnormal vascular and skeletal anatomy can be successfully overcome with alternative venous access routes, such as the azygos system, and the use of modern closure devices. This case highlights the importance of adapting procedural techniques to the specific needs of each patient and demonstrates the efficacy of the azygos route in overcoming femoral venous access difficulties.

Acknowledgements

None

Financial support

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

Competing interests

None

References

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Figure 0

Figure 1. Displastic lower extremities of the patient, illustrating significant skeletal dysplasia.

Figure 1

Figure 2a. Venogram showing the interrupted IVC with venous return rerouted through the azygos vein.

Figure 2

Figure 2b. Catheter advanced through the azygos vein into the superior vena cava (SVC), continuing to the right atrium.

Figure 3

Figure 2c. Catheter successfully crossing the patent ductus arteriosus (PDA) from the pulmonary artery to the descending aorta.

Figure 4

Figure 2d. Post-deployment angiogram confirming the secure positioning of the 6x4 mm Amplatzer Ductal Occluder II with no residual shunt across the PDA.