Percutaneous Device Closure in A Toddler with PDA and Interrupted IVC

Mani Ram Krishna¹, Suraj Narasimhan², Karuppiah³

¹Paediatric Cardiologist, Kauvery Hospital, Trichy-Heartcity, India

²Cardiologist, Kauvery Hospital, Trichy-Heartcity, India

³Cardiac Anesthetist, Kauvery Hospital, Trichy-Heartcity, India

Abstract

Per-cutaneous occlusion of the patent ductus arteriosus is achieved by deploying an occluder from the femoral vein. Abnormalities of the systemic venous pathway such as interruption of the inferior vena cava necessitate modification of the technique of per-cutaneous occlusion. We report a 1-year-old child with interruption of IVC and PDA in whom we closed the PDA utilizing a novel maneuver.

Keywords:Ductal Occluder, Interruption of IVC, Internal Jugular Venous Access

Background

Patent Ductus Arteriosus (PDA) occlusion is conventionally done using an occluder deployed from a femoral venous access. The catheter courses through the Inferior Vena Cava (IVC) to the right heart and pulmonary artery and from there to the ductus arteriosus. In rare instances, when the IVC is interrupted, additional techniques are employed to occlude the PDA. These include Hepatic Venous Access, Internal Jugular Venous (IJV) access [1], arterial access with a specialized device [2,3] and femoral venous access with multiple loops in the heart [4,5].
We report a 1-year-old child with interrupted IVC in whom the PDA was occluded using an unusual technique.

Case Presentation

A 19-month-old girl was referred for evaluation of poor weight gain, recurrent respiratory infections, and a murmur. On evaluation, she weighed 7.5 kg. There was minimal tachypnea, but her chest was clear on auscultation. Her femoral pulses were easily palpable. Her first heart sound was normal, and second heart sound was normally split. A continuous murmur was heard in the left infra-clavicular area and a mid-diastolic murmur was heard at the apex.
A trans-thoracic echocardiogram was obtained. The liver was visualized in the right side of the abdomen and the heart was in the left hemi-thorax (levocardia). The atrial appendages could not be adequately visualized. There were bilateral Superior Vena Cava (SVC) with the left SVC draining into a roofed Coronary Sinus (CS). The supra-renal portion of the IVC was interrupted with a left sided ascending (hemi-azygos) vein continuing to drain into the left SVC. The atrio-ventricular (AV) and ventriculo-arterial (VA) connections were concordant. There was left heart enlargement and a 2.8 mm PDA was seen shunting left to right. There was no evidence of pulmonary arterial hypertension.

After discussion with the family, per-cutaneous occlusion of the PDA was planned. We evaluated the various approaches available for device closure. A femoral venous access has been used in children with interrupted IVC and an azygos drainage to the right SVC with a loop in the SVC and an additional loop in the pulmonary artery. In our case, as the hemi-azyogos drained to the left SVC, an additional loop at the CS precluded using the femoral venous access. We were not familiar with hepatic venous access in small children and a double disk device such as Amplatzer Ductal Occluder II or Multi Flow Occluder was more expensive. Hence, we decided to adopt an IJV access for device closure.

The procedure was performed under conscious sedation. Right Femoral Vein (RFV), Right Femoral Artery (RFA) and right IJV access were obtained. An angiogram was obtained to confirm interruption of IVC (Fig. 1). The baseline hemodynamics were obtained, and pulmonary arterial pressure was found to be normal. The PDA was crossed from the IJV access and an Amplatzer extra-stiff wire was placed in the descending aorta. A 7 Fr Mullins sheath was advanced into the descending aorta and a ductal angiogram was obtained. A larger sheath was intentionally used so that the wire could be retained while advancing the device. This would prevent kinking of the non-braided sheath while the device is advanced. A 6/4 Lifetech ductal occluder was advanced along the sheath. There was difficulty in advancing the device across the curve from the right ventricle to the pulmonary artery (Fig. 2). As the tip of the sheath was already in the aorta, we decided to advance a 10 mm snare from the arterial access. The tip of the sheath was then snared (Fig. 3) and pulled down the descending aorta till the device reached the aorta (Fig. 4). The sheath was then retracted, and the device deployed across the duct. The stability of the device was checked by echocardiography and angiography and the device released (Fig. 5). The child was discharged the next day and at review one month later, there were no residual shunts across the duct.

Fig. 1. A still image from the inferior vena cava angiogram showing a left sided hemi-azygos vein draining into the left sided SVC which in turn drains into the coronary sinus. CS: Coronary Sinus, LSVC: Left Sided Superior Vena Cava.

Fig. 2. A fluoroscopic image showing the device (green arrow) stuck in the curve from the right ventricle to the pulmonary artery.

Fig. 3. A fluoroscopic image showing the tip of the sheath being snared (green arrow) from the arterial end using a 10 mm snare.

Fig. 4. A fluoroscopic image demonstrating the device having advanced into the descending aorta (green arrow) after the sheath was snared into the abdominal aorta.

Fig. 5. A fluoroscopic image showing the final device position after release.

Discussion

Per-cutaneous closure of the PDA is a safe and effective procedure which is considered the standard of care for a child or adult with a PDA worldwide. The standard procedure involves deployment of a ductal occluder from the femoral venous approach. When the IVC is interrupted, alternate approaches are used. We considered all possible approaches and decided that the jugular venous approach was the most appropriate for our child.

A major concern in the jugular venous approach is the sharp curve in the right ventricle which could result in hemodynamic compromise. We hypothesized that the use of a less stiff non-braided sheath will overcome this problem. We also utilized a larger sheath to allow us to retain the wire and prevent kinking of the sheath.

The snaring of the sheath from the aortic end to enable advancement of the device had not been described to the best of our knowledge. On a thorough literature review, this maneuver was described in one case of PDA with IVC interruption where deployment was considered from the femoral venous approach [5]. However, this had resulted in hemodynamic instability and the authors had abandoned the approach in favor of an IJV access.

Conclusion

Our case highlights the important of a thorough pre-procedure non-invasive imaging, the importance of pre-procedure consideration of all possible approaches and the importance of improvising on the table when facing challenging situations while performing pediatric cardiac interventions.

Acknowledgement

The cath lab staff and technologists without whose skill and dedication the procedure would not have been possible. Mrs. Pitchaimmal for assisting the family during the hospitalization.

References

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Percutaneous Device Closure in A Toddler with PDA and Interrupted IVC

Percutaneous Device Closure in A Toddler with PDA and Interrupted IVC

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