© 2006, the AuthorsCongenit Heart Dis. 2006;1:46–49 Journal compilation © 2006, Blackwell Publishing, Inc.

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Blackwell Publishing IncMalden, USACHDCongenital Heart Disease1747-079X© 2006, Copyright the Authors; Journal compilation © 2006, Blackwell Publishing, Inc.? 200611-24649Case ReportADO Closure of Thoracic AVMEvans et al.

Novel Use of the Amplatzer™ Duct Occluder for Percutaneous Closure of a Large Subclavian Artery to Innominate Vein Fistula in a Neonate

Kelly Evans, MD,*† Thomas K. Jones, MD,*† and Troy A. Johnston, MD*†

DOI: 10.1111/j.1747-0803.2006.00008.x

A B S T R A C T

We report the use of the Amplatzer™ Duct Occluder for transcatheter closure of a rare subclavian artery toinnominate vein fistula in a neonate.

Key Words. Congestive Heart Failure; Amplatzer Duct Occluder; Arteriovenous Fistula

*Division of Pediatric Cardiology, Department of Pediatrics, University of Washington School of Medicine, Seattle,WA, USA; †Children’s Hospital and Regional Medical Center, Seattle, WA, USA

Introduction

ongenital arteriovenous fistulae are a rarecause of congestive heart failure in neonates

and infants. The 2 most common types of systemicarteriovenous fistula presenting in the neonatalperiod are intracranial and hepatic fistulae.1Intrathoracic arteriovenous communications out-side the pulmonary capillary bed are especiallyrare. The few cases reported have historically beenclosed by a surgical approach.2,3 We report a caseof a 3-day-old infant with congestive heart failurewho was found to have a subclavian artery toinnominate vein fistula. This vascular malforma-tion was successfully occluded by percutaneoustechnique using an Amplatzer™ Duct Occluder.

Case Report

The patient was a 40-week gestation term newbornfemale infant weighing 4.0 kg. The pregnancy wasreported to be normal with a spontaneous vaginaldelivery. A murmur was noted on the initial exam-ination birth and then again 1 day later. Tachypneaand difficulty feeding developed and the infant wasreferred for cardiology evaluation.

On physical examination, a III/VI continuousmurmur was present loudest at the right upper

Csternal border. Bounding peripheral pulses and ahyperdynamic precordium were also noted.

The chest radiograph demonstrated marked car-diomegaly. The echocardiogram revealed drainageof a large vascular structure into the innominatevein with a dilated superior vena cava, ascendingaorta, and left subclavian artery. Additionally,there was right atrial and ventricular enlargementwith normal systolic function, a moderate patentductus arteriosus, mild Ebstein’s malformation ofthe tricuspid valve, and a moderate atrial septaldefect.

Cardiac catheterization was performed on thethird day of life. The right ventricular systolicpressure was elevated at 50 mm Hg comparedwith a left ventricular systolic pressure of 60 mmHg. The right ventricular end-diastolic pressurewas 10 mm Hg. The Qp:Qs ratio was 2:1 with aprominent step up in saturation noted in theinnominate vein. Angiography demonstrated alarge, isolated fistula arising from the left subcla-vian artery and entering the distal innominatevein. The fistula was a tubular structure measuring8.7 mm in length and 4.1 mm in diameter. Therewas no discrete narrowing present (Figure 1).

Consultation with the cardiothoracic surgeonswas sought and the decision was made to proceedwith transcatheter occlusion. A 4-French BHW

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Congenit Heart Dis. 2006;1:46–49

Glide™ Catheter (Terumo Medical Corp, Somer-set, NJ, USA) was used to cross the fistula in aprograde fashion from the innominate vein. A0.035″ standard J-tipped guidewire was advancedinto the descending aorta and the catheterremoved. Over this guidewire a 6-FrenchAmplatzer™ Delivery Sheath (AGA MedicalCorporation, Golden Valley, Minn, USA) wasadvanced beyond the fistula, the dilator andguidewire was withdrawn and the sheath carefullyflushed with normal saline solution. A 6/4 mmAmplatzer™ Duct Occluder was delivered to thesite of the fistula and deployed within the tubularportion of the fistula. An angiogram performed inthe innominate vein (Figure 2) prior to devicerelease demonstrated appropriate placement ofthe device within the fistula. No complicationsoccurred during the catheterization. The infant’sheart failure quickly resolved and she was dis-charged home 2 days after the procedure.

At a follow-up evaluation 5 weeks after deviceplacement, the infant was clinically asymptomatic.Physical examination was only notable for dimin-ished lower extremity pulses. On echocardio-graphic examination the device appeared to be instable position and there was no residual flowthrough the fistula. Coarctation of the aorta waspresent, in the typical position, remote from the

device. Cardiac catheterization revealed a discretestenosis in the distal aortic arch (Figure 3).Angiography revealed a patent left subclavianartery. A 20 mm Hg systolic gradient was notedacross the coarctation site. Balloon angioplasty ofthe coarctation narrowing was successful with anincrease in the diameter of the coarctation lumenfrom 2.5 to 4.0 mm and a residual systolic gradientof only 4 mm Hg.

At follow-up evaluation 4 months later, theinfant was growing and developing normally withno recurrence of the arteriovenous fistula and clin-ical evidence of a well-tolerated mild residualcoarctation of the aorta.

Discussion

Intrathoracic ateriovenous fistulae outside the pul-monary vascular bed are very rare, yet they shouldalways be included in the differential of congestiveheart failure presenting in the neonatal period.2–5

This case illustrates the association with otherforms of congenital heart disease. At initial pre-sentation, the presence of the patent ductus arte-riosus and unusual flow pattern associated with thefistula confounded the ability to recognize thecoarctation. The coarctation was readily apparenton follow-up.

Figure 1. Angiogram performed in a LAO Caudal projec-tion demonstrates a fistula (arrow) between the subclavianartery and the innominate vein. The innominate vein (arrow-heads) is dilated. Balloon occlusion of the arterial end ofthe fistula was utilized to provide better visualization of thefistula.

Figure 2. Angiogram performed during deployment of a 6/4 Amplatzer™ Duct Occluder demonstrates the device priorto release. The retention disk (arrow) is positioned at thearterial end of the fistula. The device protrudes into thelarge innominate vein.

48 Evans et al.

Congenit Heart Dis. 2006;1:46–49

To our knowledge, this is the first reported caseof successful closure of a subclavian artery toinnominate vein malformation by nonsurgicalapproach in a newborn infant. Only 2 prior casessimilar to this one have been described in the lit-erature. Sapire et al. described a neonate with aleft subclavian artery to innominate vein fistulaewho presented in congestive heart failure.2 A sub-clavian artery to subclavian vein fistula also pre-senting in a neonate in heart failure was describedby Dogan et al.3 In both of these cases, the fistulawas closed by surgical ligation.

Nonsurgical closure of arteriovenous fistulaefirst utilized coils and umbrella devices. Morerecently the Amplatzer™ devices have beenused.6–8 The Amplatzer™ Duct Occluder seems tobe particularly suited for occlusion of large, high-flow fistulae. It is available in multiple sizes, repo-sitionable, detachable, and placed using relativelysmall sheaths. The ability to control the deploy-ment and assess position prior to release is animportant advantage. In conditions such as thiscase, where unusual anatomy is encountered andthe catheter course is not straightforward, thesecharactersistics may be more beneficial. Addition-ally, the Amplatzer™ Duct Occluder includesocclusive thrombogenic material that reducesresidual leaks and hemolysis.

Utilization of alternative closure devices wasconsidered. It was felt that coil occlusion would

have required multiple coils and had a higher riskof incomplete closure and embolization. TheAmplatzer™ Vascular Plug was considered; how-ever, the lack of occlusive material within the plugappears to make it less suitable for high-velocityshunts.9

Conclusion

This case illustrates a novel approach to a rare, butimportant, cause of congestive heart failure in theneonate. Nonsurgical closure is less invasive andprovides the benefits of a shorter hospital stay,rapid recovery, and no thoracic scar. There arerisks associated with device placement in the car-diac catheterization lab, including embolization ofthe device, radiation exposure, potential thom-boembolic events, and residual leaks. Thoughtfulconsideration of the potential risks, benefits, andalternatives is important when utilizing novelapproaches to rare conditions.

Corresponding Author: Troy A. Johnston, MD,Children’s Hospital and Regional Medical Center—Cardiology, Heart Center 4800 Sand Point Way, NESeattle, WA 98105, USA. Tel.: (+1) 206-987-2266;Fax: (+1) 206-987-3839; E-mail: troy.johnston@seattlechildrens.org

Accepted in final form: December 26, 2005.

References

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2 Sapire DW, Lobe TE, Swischuk LA, et al. Subcla-vian-artery-to-innominate-vein fistula presentingwith congestive heart failure in a newborn infant.Pediatr Cardiol. 1983;4:155–157.

3 Dogan R, Yulmaz M, Ozkutlu S, et al. Congenitalsubclavian artery to subclavian vein fistula presentingwith congestive heart failure in an infant. Pediatr Car-diol. 2000;21:269–270.

4 Gamba PG, Longo M, Zanon GF, et al. Arterio-venous fistulae between descending aorta and hemi-azygous vein. Eur J Pediatr Surg. 1991;1:49–50.

5 Baspinar O, Kervancioglu R, Kilinc M, et al. Con-genital systemic arteriovenous fistula between thedistal thoracic aorta and hemiazygous vein in a child.Eur J Pediatr. 2005;164:458–460.

6 Godart F, Houmany M, Francart C. Congenital aor-tocaval fistula responsible for congestive heart failure.Closure with the Amplatzer Duct Occluder. CardiolYoung. 2004;14:676–677.

Figure 3. Angiogram performed at follow-up catheteriza-tion demonstrates the presence of a coarctation of the aorta(arrow). The device (arrowhead) is distant from the coarc-tation. Flow into the left subclavian artery is present. Theascending aorta and proximal subclavian artery are dilated.

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7 Al-Ata J, Amin M, Galal MO, et al. Transcatheterocclusion of a large coronary artery to right superiorvena cava fistula using the Amplatzer duct occluderdevice. Pediatr Cardiol. 2004;25:70–72.

8 Saliba Z, Bonnet D, Hausse A, et al. Transcatheterocclusion of a large aortoazygous fistula using the

Amplatzer device. J Interventional Cardiol. 2002;15:205–207.

9 Cheatham JP. Not so fast with that novel use: doesAVP = PDA. Catheter Cardiovasc Interv. 2005;65:581–583.