Transcatheter therapeutic intervention in adult coarctation of the aorta Shaun Smithson, Kongkiat Chaikriangkrai, C. Huie Lin PII: DOI: Reference:

S0167-5273(14)01014-6 doi: 10.1016/j.ijcard.2014.05.031 IJCA 18203

To appear in:

International Journal of Cardiology

Received date: Accepted date:

25 April 2014 12 May 2014

Please cite this article as: Smithson Shaun, Chaikriangkrai Kongkiat, Lin C. Huie, Transcatheter therapeutic intervention in adult coarctation of the aorta, International Journal of Cardiology (2014), doi: 10.1016/j.ijcard.2014.05.031

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Transcatheter therapeutic intervention in adult coarctation of the aorta

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Running title: Transcatheter Management of Coarctation of the Aorta

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DeBakey Heart & Vascular Center, Houston, Texas

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Shaun Smithson MD1, Chaikriangkrai, Kongkiat MD1, C. Huie Lin MD PhD1

Word Count: 908 words

Correspondence should be addressed to: Shaun D.V. Smithson, MD 6650 Fannin St. Smith Tower, Suite 1901a Houston, Texas 77030 Office: (713) 441-2690 Email: [email protected]

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A 44-year-old man presented with one-year history of headache that was progressively worsening for the past three months. He denied family history of congenital heart disease; however, his mother had resistant hypertension. Physical examination showed elevated blood pressure with a systolic murmur at left upper sternal region as well as diminished femoral pulses in both lower extremities. He was started on antihypertensives by his primary care doctor for essential hypertension, but intermittently continued to have headaches. Four extremity blood pressures demonstrated a 60mm systolic gradient from upper to lower extremities. Echocardiography showed preserved biventricular function. Aortic valve was bicuspid without stenosis or regurgitation. He was subsequently evaluated by cardiac MRI which demonstrated 3039% stenosis in the isthmus of the aorta with peak velocity of 3.25 m/sec and the ascending aorta was aneurysmal at 4.1 cm. Given his symptomatic presentation, he elected transcatheter intervention.

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The patient was taken to the catheterization laboratory where access was obtained in the right femoral artery and vein and the right radial artery. Right and left heart catheterization were performed demonstrating a 40mm gradient across the coarct, followed by descending aortography via a 4-French pigtail catheter advanced from the right radial artery. Rotation angiography was performed (Figure 1) and 3D reconstruction (Figure 2) demonstrated that the coarctation appeared to be approximately 5 mm in diameter with the reference vessel range between 18 to 20 mm in diameter at the aortic isthmus at approximately 20 to 22 mm in diameter distal to the coarctation and numerous collaterals. Baseline hemodynamic s revealed a 40mmHg peak-to-peak gradient across the coarctation. Coarctation stenting equipment included a 14-French 75-cm Mullins sheath, a 0.035/260 cm Lunderquist wire (Cook Medical, Bloomington IN), a CP8Z45 Covered Cheatham-platinum stent, CCPS, (Figure 3) mounted on a 18 x 5 cm Balloon-in-Balloon (BIB) catheter (NuMed Cornwall, ON). The stent was deployed during which rapid ventricular pacing was performed. Post-stent angioplasty was performed using a 20 x 2 Vida balloon (Bard Peripheral Vascular, Murray Hill NJ). Following successful intervention, there was no evidence of residual gradient across the coarctation. Post-stent angiography demonstrated that the stent was well opposed and there appeared to be no evidence of extravasation of contrast or dissection (Figure 4).

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Following intervention, the patient was extubated in the cath lab and observed overnight on telemetry. The next day, he complained of some mild pleuritic pain radiating to the back (likely related to stretch of the aorta), however follow-up echocardiography demonstrated no pericardial effusion, stable aorta, and no further gradient across the coarctation. He was discharged the day after intervention with plan for follow-up cross-sectional imaging at one month. At home, his need for antihypertensives improved significantly.

Aortic coarctation is a relatively common congenital heart disease with a reported incidence of 4 in 10,000 live births or 5-8% of all congenital heart defects [1] and is frequently associated with bicuspid aortic valve stenosis. Additional associated lesions include cerebral “berry” aneurysm and aortopathy (risk of cystic medial necrosis, aneurysm and dissection). Most patients are diagnosed during childhood; however a minority are not diagnosed until adulthood. ACC/AHA Guidelines have recommend (Class I) intervention for aortic coarctation when a peak-to-peak coarctation gradient is greater than 20 mmHg and/or there is radiological evidence of significant coarctation and collateral flow [2]. Most commonly performed procedures are stent implantation, balloon angioplasty or surgical repair. Common major complications for all modalities include recurrent coarctation and aortic aneurysm which can evolve into aortic dissection and rupture. In adults, both open surgical repair and endovascular therapy are associated with comparable mortality rate. Most studies reported 0% mortality with a few narrating 1-3% deaths [3]. Risk of re-coarctation has been shown to be higher in stent implantation (5-25%) than balloon angioplasty (8-22%) and open surgical repair (2-9%) which has led to higher re-intervention in endovascular therapy groups[3], however, this may be confounded by the fact that many interventionalists in the US would consider a staged approach if the coarcted segment is less than 20% the native segment to minimize aortic injury given the absence of FDA-approved balloon expandable covered stents. Data regarding aneurysmal dilatation at the

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repair site is very limited as follow up imaging studies are not routinely performed. Open surgical repair was reported to have aortic aneurysm rate ranged from 1.8 to 6% [4, 5], 3-11% after stent implantation [5-8] and 6-8% after balloon angioplasty [9-11]. In a contemporary prospective study, stent patients had fewer acute complications than surgical and balloon angioplasty patients (2.3%, 8.1%, and 9.8%, P < 0.001) with an aortic wall injury rate of 12.6% in surgical, 7.1% in stent, and 43.6% in balloon angioplasty patients [12]. As a result of concern for aortic injury during catheter balloon angioplasty and bare metal stent implantation, most centers outside the US have moved to the routine use of covered balloon expandable stents such as the Covered Cheatham Platinum Stent (used in this case via Compassionate Use Exemption) or the V12 (Atrium, Hudson, NH). The ongoing US COAST II trial seeks to demonstrate the safety and efficacy of the CCPS in coarctation intervention.

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44 year old male with severe coarctation of the aorta with 40mmHg gradient at baseline underwent successful transcatheter intervention with implantation of a Covered Cheatham-platinum stent. Primary stent-based treatment of coarctation of the aorta is safe and effective, however, these patients continue to require lifelong follow-up at an adult congenital heart center for surveillance of long-term complications such as aortic valve dysfunction, aortic aneurysm or dissection, and recurrent coarctation.

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References 1. Rao, P.S., Coarctation of the aorta. Curr Cardiol Rep, 2005. 7(6): p. 425-34. 2. Warnes, C.A., et al., ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol, 2008. 52(23): p. e143263. 3. Carr, J.A., The results of catheter-based therapy compared with surgical repair of adult aortic coarctation. J Am Coll Cardiol, 2006. 47(6): p. 1101-7. 4. Fletcher, S.E., J.P. Cheatham, and S. Froeming, Aortic aneurysm following primary balloon angioplasty and secondary endovascular stent placement in the treatment of native coarctation of the aorta. Cathet Cardiovasc Diagn, 1998. 44(1): p. 40-4. 5. Johnston, T.A., R.G. Grifka, and T.K. Jones, Endovascular stents for treatment of coarctation of the aorta: acute results and follow-up experience. Catheter Cardiovasc Interv, 2004. 62(4): p. 499-505. 6. Harrison, D.A., et al., Endovascular stents in the management of coarctation of the aorta in the adolescent and adult: one year follow up. Heart, 2001. 85(5): p. 561-6. 7. Ledesma, M., et al., Results of stenting for aortic coarctation. Am J Cardiol, 2001. 88(4): p. 460-2. 8. Cheatham, J.P., Stenting of coarctation of the aorta. Catheter Cardiovasc Interv, 2001. 54(1): p. 112-25. 9. Fawzy, M.E., et al., Long-term outcome (up to 15 years) of balloon angioplasty of discrete native coarctation of the aorta in adolescents and adults. J Am Coll Cardiol, 2004. 43(6): p. 1062-7. 10. Saba, S.E., et al., Balloon coarctation angioplasty: follow-up of 103 patients. J Invasive Cardiol, 2000. 12(8): p. 402-6. 11. Paddon, A.J., et al., Long-term follow-Up of percutaneous balloon angioplasty in adult aortic coarctation. Cardiovasc Intervent Radiol, 2000. 23(5): p. 364-7. 12. Forbes TJ, Kim DW, Du W, Turner DR, Holzer R, Amin Z, et al. Comparison of surgical, stent, and balloon angioplasty treatment of native coarctation of the aorta: an observational study by the CCISC (Congenital Cardiovascular Interventional Study Consortium). J Am Coll Cardiol. 2011 Dec 13;58(25):266474.

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Figure 1

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Figure 2

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Figure 4

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Variable 10/9 (8) mmHg 30/4 mmHg 27/10 (6) mmHg (10) mmHg 91/11 mmHg 106/77 mmHg 66/55 mmHg 4.4 L/min 4.4 L/min 1

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Baseline Right Atrium Right Ventricle Left Pulmonary Artery Left Capillary Wedge Left Ventricle Ascending Aorta Descending Aorta Qs Qp Qp/Qs

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Table 1: Baseline Hemodynamics

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Transcatheter therapeutic intervention in adult coarctation of the aorta.

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