Patent Ductus Arteriosus Device Closure in Interrupted Inferior Vena Cava: Challenges Overcome and Lessons Learnt: A Case Series.

Interrupted inferior vena cava (IVC) with azygous continuation provides technical challenge in many percutaneous cardiac interventions. They are performed via the femoral venous access route. We describe four such cases in whom, patent ductus arteriosus (PDA) device closure was done in interrupted IVC. All the four cases were done using the femoral route. Two cases were done via retrograde approach through femoral artery access, in which one was closed with a muscular VSD device and the other with a Gianturco coil. The introduction of newer closure devices helps in the retrograde approach. Two cases were done via anterograde approach through the femoral venous route, with a loop through the azygous vein. One child had transient bradycardia due to cardiac stretch which normalized after the device deployment and introducer system removal. Thus, we describe different femoral approaches in interrupted IVC patients for PDA closure, with the difficulties faced and the complications managed. Copyright:

INTRODUCTION

The percutaneous closure of Patent Ductus Arteriosus (PDA) has become the treatment of choice in all cases wherever it is feasible. It is a fairly simple procedure in patients with normal venous anatomy. However, PDA device closure is challenging in case of interrupted Inferior Vena Cava (IVC) and azygous continuation of IVC where multiple technical challenges can be encountered and need experienced operator for successful completion of the procedure.

CASE PRESENTATIONS

The authors describe challenges encountered during PDA device closure in four cases [Table 1] of azygous continuation of IVC, two of which were closed via azygous route, and the rest were done via arterial route.

Table 1

Details of the patients along with the procedure

Number	Age (years)	Sex	Weight (kg)	PDA size (mm)	Route	Approach	Device
Case 1	3	Male	14	4	Femoral vein	Anterograde	8 mm × 6 mm lifetech PDA device
Case 2	2	Female	9	4	Femoral artery	Retrograde	6 mm cocoon muscular VSD device
Case 3	3	Male	11	3	Femoral vein	Anterograde	8 mm × 6 mm ADO I
Case 4	6	Female	20	2	Femoral artery	Retrograde	5 mm × 4 mm Gianturco coil

ADO: Amplatzer duct occlude, PDA: Patent ductus arteriosus, VSD: Ventricular septal defect

Case 1

A 3-year-old male child weighing 14 kg with 4 mm PDA [Figure 1a] was taken up for device closure. 5Fr Femoral Arterial and Venous Access were taken. Venous angiogram via the femoral route showed the azygous continuation of IVC [Figure 1b]. The femoral sheath was exchanged with FlexorR Balkin guiding sheath (Cook Medical, USA) which was parked in the mid-right atrium. A 5Fr Multipurpose catheter (Cook Medical, USA) with 0.035” straight tip Terumo GlidewireR (Terumo, Japan) was used to cross PDA. The wire was exchanged with AmplatzerR extra-stiff 0.035” wire (AGA Medical Corporation, USA) which was parked in descending aorta [Figure 1c]. This caused transient bradycardia and hypotension due to the stretch, which resolved on withdrawal of Amplatzer wire. An 8 mm × 6 mm Cera™ duct occluder device (Lifetech Scientific Inc., China) was deployed successfully [Figure 1d] over a 6-Fr SteerEase™ sheath (Lifetech Scientific Inc., China). The post deployment angiogram showed no residual flow across the device.

Figure 1

Case 1: (a) Aortogram showing 4 mm patent ductus arteriosus; (b) venogram showing interrupted inferior vena cava; (c) patent ductus arteriosus crossed via azygous route from the femoral venous access; (d) lifetech 8 mm × 6 mm patent ductus arteriosus device deployed

Case 2

A 2-year-old female child weighing 11 kg with 4 mm PDA [Figure 2a] was taken up for device closure. The child was found to have the azygous continuation of IVC during the procedure. PDA was crossed via the femoral venous route and attempt was made with 8 mm × 6 mm Amplatzer Duct Occluder (ADO) I (AGA Medical Corporation, USA) device [Figure 2b]. As soon as the cable was released, the device embolised into the right pulmonary artery (PA) probably because of the rebound force of the extra loop-tension of the cable device [Figure 2c]. The device was snared and retrieved from the right PA [Figure 2d]. It could be pulled into the superior vena cava (SVC) but could not be retrieved into the azygous vein [Figure 2e]. Hence a second snare was introduced from the internal jugular vein (IJV) access and the device was fed to the second snare from the first snare in the SVC [Figure 2e and f]. The device was pulled back into IJV [Figure 2g] and retrieved by surgical cut down on the table. In the same sitting, a 6 mm Cocoon muscular VSD device (Vascular Innovations, Thailand) was used successfully to close from the aortic end via the femoral route [Figure 2h].

Figure 2

Case 2: (a) Aortogram showing 4 mm patent ductus arteriosus; (b) Amplatzer Duct Occluder I device 8 mm × 6 mm via femoral venous route; (c) device (White arrow) embolized into right pulmonary artery; (d) embolized device snared; (e) Snared device stuck at superior vena cava-azygous junction; (f) device retrieved via 2nd snare via internal jugular vein access; (g) device in internal jugular vein; (h) cocoon 6 mm muscular device closure of patent ductus arteriosus via femoral arterial route

Case 3

A 2-year-old girl child weighing 11 kg with 3 mm PDA with the azygous continuation of IVC was successfully closed with ADO I device from the femoral venous route without much difficulty [Figure 3a].

Figure 3

(a) Case 3: 3 mm patent ductus arteriosus closed with 8 mm × 6 mm Amplatzer Duct Occluder I device via femoral venous route; (b) Case 4: 2 mm patent ductus arteriosus closed with 5 mm × 4 mm Gianturco coil via femoral arterial route

Case 4

A 6-year-old girl child with tiny PDA and azygous continuation of IVC was successfully closed with 5 mm × 4 mm Gianturco coil (Cook Medical, USA) from the arterial side [Figure 3b].

DISCUSSION

Patent Ductus Arteriosus (PDA) is one of the common congenital heart diseases accounting for 5%–10% of congenital heart defects.[12] Porstmann[3] was the first to close PDA by the percutaneous route in 1966. PDA devices have undergone a sea of change since then, with the basic technique being the same. Interruption of IVC is rare with an incidence of 0.1%.[4] It is more commonly associated with left isomerism and complex congenital heart defects while relatively rare with other conditions. However, if it is associated, percutaneous access to the cardiac chambers for device closure via the femoral venous route is difficult.

The options for percutaneous closure of PDA in interrupted cases are

Difficulty in tracking the femoral venous sheath, kinking of catheters and delivery sheath, hemodynamic instability due to stretch caused by the system in the heart are the problems faced while using this access. Snaring the wire from the arterial end and forming an arterio-venous loop might be necessary when difficulty is encountered in advancing the system.

With the newer devices like ADO II and muscular VSD device, the closure has been made possible from the arterial end. Alternatively, coils can be deployed in case of small PDA. The need for technical expertise and the availability of devices at the procedure time are essential. Larger bore access at the arterial end in a younger child can be a deterrent in this approach.

IJV access is used for the closure. Smaller children might need intubation and mechanical ventilation for undisturbed access. The technical difficulty is facing 2 U-turns in the route: First at the SVC-Right Atrium-Right ventricle-PA track and second at PA-PDA-aorta track.

Various case reports [Table 2] have been made in this particular aspect with different technical challenges faced. In the first case, difficulty in tracking was overcome by using a long FlexorR Balkin sheath. Transient hemodynamic instability was faced due to extra-stiff wire in both case 1 and case 2 (during the initial attempt via femoral venous route). Caution is advised due to the rebound torque of the delivery system while unscrewing the device, due to which we faced a device embolization. We did not face the necessity to form an arterio-venous loop in any of our cases for tracking. In selective cases, device or coil deployment through arterial route can be undertaken (case 2 and case 4). The newer devices like ADO II are particularly useful in this regard.

Table 2

Cases compilation of patent ductus arteriosus device closure with interrupted inferior vena cava from literature

Author	Year	Age (years)	Sex	Weight (kg)	PDA size (mm)	Route	Approach	Device
Al-Hamash[5]	2006	9	F	24	4	Femoral vein	Anterograde	ADO I, 8 mm × 6 mm
		3	M	15	4	Femoral Vein	Anterograde	ADO I, 8 mm × 6 mm
Sivakumar and Francis[6]	2007	N/A	N/A	N/A	8	Femoral Artery	Retrograde	ADO I, 12 mm × 10 mm (reversed)
Koh et al.[7]	2009	37	F	N/A	3.5	Femoral Artery	Retrograde	ADO II, 6 mm × 6 mm
Patel et al.[8]	2009	9	F	21	5.1	IJV	Anterograde	ADO I, 12 mm × 10 mm
Akhtar et al.[9]	2010	14	F	N/A	5	IJV	Anterograde	ADO I, 12 mm × 10mm
Celebi et al.[10]	2013	¼	F	5.4	4.3	Femoral Artery	Retrograde	Lifetech, 6 mm × 8mm
Tefera and Bermudez-Cañete[11]	2014	12	F	39	5	Femoral Vein	Anterograde	ADO, 8 mm × 6 mm
Subramanian et al.[12]	2014	1 1/2	N/A	N/A	4.7	IJV	Anterograde	Lifetech PDA Occluder, 10 mm × 12 mm
Aggarwal et al.[13]	2015	3	F	8	3	IJV	Anterograde	6 mm × 4 mm device
Jawahirani et al.[14]	2018	5	F	8	6	IJV	Anterograde	Cocoon duct occluder 10 mm × 8 mm

ADO: Amplatzer duct occluder, IJV: Internal jugular vein, N/A: Not available, PDA: Patent ductus arteriosus

CONCLUSION

PDA device closure in interrupted IVC via the femoral route is challenging as extra loop and tension of wire can cause transient hypotension or rebound device embolization during release. One can use coils, muscular devices, or newer devices like ADO II from the aortic end in appropriate cases or use an IJV route.

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.14

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.