Percutaneous Stenting of Interrupted Aortic Arch toTreat Compressive Myelopathy

Nagaraja Moorthy,* MD, DM, Rajiv Ananthakrishna, MD, DM, andManjunath C. Nanjappa, MD, DM

Neurological complications of coarctation of aorta include spontaneous SAH, intracere-bral hemorrhage, and cerebral abscess. Interrupted aortic arch (IAA) present as compres-sive myelopathy is not known. We describe an adult male presenting to neurologydepartment with progressive paraparesis and was detected to have IAA with intraspinalcollaterals causing compressive myelopathy. He was successfully treated with percutane-ous stenting of IAA with dramatic improvement in paraparesis. VC 2014 Wiley Periodicals, Inc.

Key words: coarctation of aorta; interrupted aortic arch; paraparesis; stenting

INTRODUCTION

Interrupted aortic arch (IAA) is a congenital malfor-mation characterized by a complete separationbetween the ascending aorta and the descending aorta[1,2]. IAA present in adults is extremely rare [3].Neurological complications of coarctation of aorta(CoA) include spontaneous subarachnoid hemorrhage(SAH), intracerebral hemorrhage, and cerebral abscess.IAA present as compressive myelopathy is notdescribed. Even though optimal therapy of CoA is de-batable, with the introduction of endovascular stents,catheter-based intervention in the treatment of aorticcoarctation now represents a viable alternative to sur-gery [4]. However complete occlusion of the aorticarch makes surgical repair as a universally recom-mended therapy.

TEXT

A 42-year-old man was referred to neurology depart-ment with the history of progressive weakness of bothlower limbs of 4 years duration. The weakness wasgradual in onset and progression. He also gave historyof one episode of paraplegia 2 years back which recov-ered over 3 months duration. On examination, bilateralfemoral pulses were relatively weaker with radio-femoral delay. Blood pressure in upper limb was 140/90 mm Hg and in lower limbs was 130/88 mm Hg.Continuous murmur was heard over upper back. Cardi-ovascular system examination revealed short systolicmurmur at aortic area. Neurological examinationshowed symmetrical paraparesis with 3/5 muscle powerin lower limbs. There was no bowel and bladderinvolvement. Electrocardiography was unremarkable.Chest radiograph showed bilateral rib notching involv-

ing 3–6 ribs. Echocardiography showed mild concen-tric left ventricular hypertrophy (LVH) with normalleft ventricle (LV) function. Magnetic resonance aor-tography showed short segment total occlusion of de-scending thoracic aorta just distal to left subclavianartery origin (Fig. 1). There were extensive collateralsaround upper thoracic vertebrae. MRI of dorsal spinalcord showed intraspinal tortuous collaterals (Fig. 2A)with a small aneurysm compressing the spinal cord atD2 vertebra (Fig. 2B). Cardiac catheterization showedcomplete occlusion of descending throracic aorta (Fig.3A, Supporting Information Video 1) just distal to leftsubclavian artery origin with extensive collateralsaround cervico-dorsal spine (Fig. 3B, Supporting Infor-mation Video 2). In view of IAA with tortuous collat-erals resulting in compressive myelopathy,percutaneous aortoplasty with stenting was planned.Left radial and right femoral accesses were obtained. A6 Fr multipurpose guide catheter was engaged in the

Additional Supporting Information may be found in the online ver-

sion of this article.

Department of Cardiology, Sri Jayadeva Institute of Cardio-vascular Sciences and Research, Bangalore, India, 560069

Conflict of interest: Nothing to report.

*Correspondence to: Dr. Nagaraja Moorthy, MD, DM, Department

of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and

Research, Bangalore, India 560069. E-mail: drnagaraj_moorthy@

yahoo.com

Received 20 October 2013; Revision accepted 20 January 2014

DOI: 10.1002/ccd.25408

Published online 23 January 2014 in Wiley Online Library

(wileyonlinelibrary.com)

VC 2014 Wiley Periodicals, Inc.

Catheterization and Cardiovascular Interventions 84:815–819 (2014)

proximal cap of occlusion and using MiracleBros0.01400 percutaneous transluminal coronary angioplasty(PTCA) guidewire lesion was attempted to cross. ButMiracleBros wire was noted to be in the subintimalplane. The initial MiracleBros wire was left in placeand using Shinobi (Cordis, Argentina) 0.00700 PTCAguidewire the occluded segment was crossed using

“parallel wire technique” (Fig. 4, Supporting Informa-tion Video 3). The lesion was serially dilated with 2.5mm � 15 mm, 4 mm � 15 mm semi compliant bal-loon followed by Numed 16 mm � 40 mm percutane-ous transluminal angioplasty (PTA) balloon (NumedInc. Hopkinton, NY). Following balloon dilatationthere was recoil of the lesion with residual gradient of

Fig. 1. A: Magnetic resonance aortography showing type-A IAA (Arrow) just distal to originof left subclavian artery. B: Magnetic resonance aortography showing extensive collateralsbridging the interruption.

Fig. 2. A: Magnetic resonance imaging of cervico-dorsal spine showing dilated intra-spinalcollaterals (Arrow) compressing the spinal cord. B: Magnetic resonance imaging of cervico-dorsal spine showing a intraspinal aneurysm (Arrow) compressing the spinal cord.

816 Moorthy et al.

Catheterization and Cardiovascular Interventions DOI 10.1002/ccd.Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).

40 mm Hg across the lesion (Fig. 5A, Supporting In-formation Video 4). The residual lesion was stentedwith 16 mm � 40 mm uncovered balloon expandablePalmaz stent (Cordis, Miami, FL). Following stentingthere was no residual gradient and the collateralsaround the spinal column disappeared completely (Fig.5B, Supporting Information Video 5). Patient had grad-ual improvement in paraparesis and at 4 weeks follow-up he was able to walk without support.

DISCUSSION

IAA is an extremely rare congenital malformationthat accounts for 1% of all congenital heart disease [3].This anomaly is an extreme form of aortic coarctation,characterized by total luminal and anatomic interrup-tion between the ascending and descending thoracicaorta [5]. IAA present in adults is extremely rare [1,6].Survival into adulthood is dependent upon the develop-ment of substantial collateral circulation. These collat-eral vessels are subject to atrophy, atherosclerosis, andeven spontaneous rupture, resulting in secondary com-plications [7]. However collaterals resulting compres-sive myelopathy is not described in the literature.

Our patient primarily presented to neurology depart-ment with compressive myelopathy and clinical examina-tion revealed type-A IAA which was confirmed by MRaortogram. Our patient was normotensive and his lowerlimb blood pressure was nearly equal to upper limb pres-sure due to extensive collaterals bridging the interruptedsegment of aorta. The primary indication to interveneinterruption was to treat compressive myelopathy.

In our patient short segment of interruption, nipple-like projection in the proximal stump, and adequategap between the origin of left subclavian artery and in-terrupted segment posed favorable anatomy for percu-taneous intervention. After risks involved with surgicalcorrection percutaneous intervention of IAA wasplanned.

With the introduction of endovascular stents,catheter-based intervention in the treatment of aortic

Fig. 3. A: Aortic arch angiography showing type-A IAA. B: Aortic arch angiography showingextensive collaterals simulating “bag of worms appearance.”

Fig. 4. Cine imaging showing two PTCA wires across the in-terrupted segment, one in false lumen (Arrow) and the otherin true lumen.

Percutaneous Stenting of IAA 817

Catheterization and Cardiovascular Interventions DOI 10.1002/ccd.Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).

coarctation is considered as an effective alternative tosurgery. However percutaneous stenting of IAA istechnically challenging. We opted for antegradeapproach through left radial artery as there was nipple-like projection in the proximal stump and left subcla-vian artery was coaxial with the interrupted segment,which provides better catheter support during penetra-tion. However the risks and potential complicationsrelated to this procedure like false lumen creation, ves-sel wall injury, or disruption and acute vessel compro-mise were of major concern [7]. In our patient, initialPTCA wire entered the false lumen hence was left inposition and the interrupted segment was crossed withanother wire using “parallel wire technique” whichprevents repeated entry into false lumen and directs theentry of second wire.

In coarctoplasty with stenting is better when com-pared to balloon angioplasty due to minimal residualgradient, improved diameter, and sustained hemody-namic benefit. The use of stents have also achievedgood results and reduced the complications of angio-plasty [8]. The recoiling after initial balloon dilatationand residual gradient compelled stenting in our patient.After stenting there was complete collapse of the intra-and extraspinal collaterals. However complete clinicalrecovery took 3–4 weeks.

Percutaneous treatment is feasible even in patientswith interrupted aortic arches or extremely severeforms of CoA. However we strongly recommend suchprocedures should be attempted by experienced opera-

tors, in carefully selected cases of IAA with favorableanatomy and, all necessary equipments including cov-ered stents (sizes range between 12 mm and 24 mmrequiring 12–14 Fr introducer) must be readily avail-able in the cardiac catheterization laboratory. Coveredstents help in bail out procedure when complicationsrelated to coarctoplasty like dissection, aortic rupture,and aneurysm formation occur. In addition, immediateaccess to cardiac surgery is required in case of compli-cations.

CONCLUSION

Isolated IAA present in adults as compressive mye-lopathy is extremely rare. Percutaneous stenting ofIAA is feasible and effective alternative to surgery canpromptly relieve paraparesis.

REFERENCES

1. Kosucu P, Kosucu M, Dinc H, Korkmaz L. Interrupted aortic

arch in a adult: Diagnosis with MSCT. Int J Cardiovasc Imaging

2006;22:735–739.

2. Mishra PK. Management strategies for interrupted aortic arch

with associated anomalies. Eur J Cardiothorac Surg 2009;35:

569–576.

3. Messner G, Reul GJ, Flamm SD, Gregoric ID, Opfermann UT.

Interrupted aortic arch in an adult: Single-stage extra anatomic

repair. Tex Heart Inst J 2002;29:118–121.

4. Mullen MJ. Coarctation of the aorta in adults: Do we need sur-

geons? Heart 2003;89:3–5.

Fig. 5. A: Post balloon dilatation of interruption showing good recoiling with significantresidual stenosis. B: Post stenting of interrupted segment showing disappearance ofcollaterals.

818 Moorthy et al.

Catheterization and Cardiovascular Interventions DOI 10.1002/ccd.Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).

5. Ceviz N, Kantarci M, Okur A. Electrocardiographic gated multi-

slice computed tomography of Uhl’s anomaly. Heart 2004;90:

886.

6. Wong CK, Cheng CH, Lau CP, Leung WH, Chan FL. Inter-

rupted aortic arch in an asymptomatic adult. Chest 1989;96:

678–679.

7. Prasad SV, Gupta SK, Reddy KN, Murthy JS, Gupta SR,

Somnath HS. Isolated interrupted aortic arch in adult. Indian

Heart J 1988;40:108–112.

8. Hijazi ZM. Catheter intervention for adult aortic coarctation:

be very careful! Catheter Cardiovasc Interv 2003;59:536–

537.

Percutaneous Stenting of IAA 819

Catheterization and Cardiovascular Interventions DOI 10.1002/ccd.Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).