|
 
 |
| CASE IN POINT - CLINICS IN PEDIATRIC CARDIOLOGY |
|
| Year : 2017 | Volume
: 3
| Issue : 4 | Page : 221-223 |
|
Stenting of complex coarctation of aorta with aberrant right subclavian artery
Anil K Singhi1, Sunip Banerjee2
1 Department of Pediatric Cardiology, Medica Superspecialty Hospital, Mukundapur, Kolkata, West Bengal, India
2 Department of Cardiology, Medica Superspecialty Hospital, Mukundapur, Kolkata, West Bengal, India
| Date of Web Publication | 7-Jul-2017 |
Correspondence Address:
Anil K Singhi
Department of Pediatric Cardiology, Medica Superspecialty Hospital, Mukundapur, Kolkata, West Bengal
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/astrocyte.astrocyte_18_17
Coarctation of aorta with aberrant right subclavian artery distal to coarctation is very rare. Detailed evaluation and planning is required for management of such case. Coarctation of the thoracic aorta (CoA) occurs at an incidence of approximately 1:1000 in the general population, ranging from 0.08 to 0.14% in large surveys. Anomalous origin of right subclavian artery (RSCA) is reported in up to 1% cases of CoA.Presence of aberrant RSCA makes treatment of such anomaly challenging. We report here successful placement of bare metal stent in a young lady with severe CoA and aberrant RSCA distal to CoA. Transcatheter stenting of CoA with aberrant RSCA is safe and effective procedure with good outcome. A detailed preprocedure evaluation and planning is the key to success. Keywords: Aberrant right subclavian artery, complex coarctation, stenting
How to cite this article:
Singhi AK, Banerjee S. Stenting of complex coarctation of aorta with aberrant right subclavian artery. Astrocyte 2017;3:221-3 |
| Introduction | |  |
Coarctation of the thoracic aorta (CoA) occurs at an incidence of approximately 1:1000 in the general population, ranging from 0.08 to 0.14% in large surveys.[1] Anomalous origin of right subclavian artery (RSCA) is reported in up to 1% cases of CoA.[2] Presence of aberrant RSCA makes treatment of such anomaly challenging. We report here successful placement of bare metal stent in a young lady with severe CoA and aberrant RSCA distal to CoA.
| Case Report | | |
A 25-year-old lady school teacher by profession had history of effort intolerance (functional class II). Her heart disease was suspected during routine evaluation on finding feeble right hand pulse. During clinical evaluation, her right arm systolic blood pressure (BP) was 80 mmHg, left arm BP was 130/80 mmHg, and lower limb systolic BP was 80 mmHg. Right arm and lower limb pulses were not well felt. Cardiac evaluation revealed normal heart sound and a short systolic murmur at the back. The chest X-ray showed evidence of rib notching [Figure 1]. Electrocardiogram (ECG) had sinus rhythm with left ventricular dominance. Echocardiogram showed severe CoA. The peak CoA gradient was 57 with diastolic tailing. The aortic valve was tri-leaflet without any evidence of aortic stenosis or regurgitation. There was concentric left ventricular hypertrophy and good ventricular systolic function. Computed tomographic (CT) aortogram was done to delineate the coarctation and aortic branch anatomy. It showed severe CoA [Figure 2]a and [Figure 2]b; CoA region was 17 mm distal to left subclavian artery (LSCA) and was measuring 3 mm. The RSCA was aberrant and originating just distal to the CoA. The aortic isthmus measured 18 mm. The plan of transcatheter stenting of the coarctation was discussed with the family. In view of severely narrowed CoA region which required more than three times dilatation of the narrow region, a covered stent could have been an ideal choice to avoid risk of dissection. We planned to use a bare metal stent with covered stent standby to preserve RSCA flow. The cardiothoracic surgical teams were kept in the loop. Right femoral artery and left radial artery access obtained. The basal ascending aortic pressure was 138/93/108 (mean) mm Hg and descending aorta pressure was 106/84/90 (mean) mm Hg. The RSCA angiogram showed it is originating below the level of coarctation [Figure 3], Video 1.
The coarctation was difficult to cross from below. It was crossed from above through the left radial artery access with the help of Terumo wire. The wire was snared from below to pass a pigtail catheter across the coarctation from below. The basal angiogram showed severe CoA and aberrant RSCA [Figure 4]a, Video 2.
A 12-F long Mullin sheath (Medtronic, Minnesota, USA) was taken across the CoA region over a Amplatzer super-stiff wire (St Jude Medical, Minnesota, USA) parked in the left ventricle. A 45 mm bare CP stent (NuMED Inc., NY, USA) mounted over a Z med 16–60 mm balloon (NuMED Inc., NY, USA) was taken and deployed slowly across the CoA under right ventricular pacing @200/min. After initial deployment the stent appeared unstable, hence it was postdilated with 18–60 mm Z med balloon. After the postdilatation with 18 mm balloon, the stent attained stable position without any residual narrowing or gradient [Figure 4]b, Video 3.
Selective cannulation of the RSCA for angiogram was avoided in a postcoarctation stenting scenario. Though the RSCA was not visualized in the final aortic angiogram, such as the basal aortic angiogram, the pulse was well appreciated. She was discharged on Aspirin 150 mg daily in view of aberrant RSCA in the stented region and metoprolol. On 9-month follow-up, she was doing well and had good effort tolerance. Echocardiogram at 9-month follow-up showed very stable stent position with good flow across. | Figure 1: Chest X-ray in posteroanterior view showing the rib notching (arrow).
Click here to view |
 | Figure 2: (a) Computed tomographic aortogram showing coarctation (bold arrow) in anteroposterior view; (b) Volume rendered three-dimensional CT aortogram showing coarctation from behind with origin of the aberrant right subclavian (arrow).
Click here to view |
 | Figure 3: Selective angiogram showing aberrant RSCA originating distal to the coarctation [Video 1].
Click here to view |
 | Figure 4: (a) Aortic angiogram in anteroposterior view showing severe coarctation of aorta (arrow) [Video 2]; (b) Poststenting aortic angiogram in anteroposterior view showing good stent position with well open coarctation segment (arrow) [Video 3].
Click here to view |
| Discussion | | |
Approximately, 1% of patients with postductal thoracic aortic coarctation have an associated anomalous RSCA.[3],[4] The triad of this condition are: (a) hypotension of the right upper and lower extremities, (b) hypertension of the left upper extremity, with (c) absence of a murmur over the right scapula.[5] The origin of aberrant subclavian is usually distal but can be proximal to CoA also.[2] Presence of anomalous RSCA affects the decision-making in intervention as well as in surgery. In the preintervention era, the surgical intervention of such anomaly was being affected by the type of arterial collateral circulation around the coarctation and involved different surgical techniques to deal with the vascular anomalies.[3] Preservation of the blood supply to the right arm is good if it can be achieved in cases of coarctation with aberrant RSCA. There are case reports where aneurysms close to LSCA or aberrant RSCA are effectively treated by endoluminal repair without the need for revascularization procedures with transient and mild ischemic symptoms.[6] Significantly narrowed coarctation region when the dilatation requirement is more than three times the narrowest diameter is best done with a covered stent in grownup patients. The vascular supply to the right arm in such case in presence of aberrant RSCA will be compromised, but possibly can be managed with minimal ischemic symptoms. We tried to preserve the right arm blood supply by adopting a strategy of stenting with bare metal stent and keeping covered stent as standby in case of emergency need. The plan was discussed with the patient and family members, which helped to successfully accomplish the intervention with good outcome in short term.
| Conclusion | | |
Transcatheter stenting of CoA with aberrant RSCA is safe and effective procedure with good outcome. A detailed preprocedure evaluation and planning is the key to success.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | DeBakey MF, Noon GP. Thoracic aorta and great vessels. In: Effler DB, editor. Blades' Surgical Diseases of the Chest. 4th ed. St. Louis: Mosby; 1978. p. 601-48.  |
| 2. | Abbott ME. Coarctation of the aorta of the adult type II: A statistical study and historical retrospect of 200 recorded cases with autopsy, of stenosis or obliteration of descending arch in subjects above the age of two years. Am Heart J 1928;3:381. Available from: http://www.sciencedirect.com/science/article/pii/S0002870328902811. [Last accessed on 2017 Mar 01]. |
| 3. | Reid DA, Foster ED, Stubberfield J, Alley RD. Anomalous right subclavian artery arising proximal to a postductal thoracic aortic coarctation. Ann Thorac Surg 1981;32:85-7. |
| 4. | Engelman RM, Madayag M, Spencer FC. Aneurysm of aberrant right subclavian artery. NY State J Med 1973;73:290-2. |
| 5. | Slager RF, Klassen KP. Anomalous right subclavian artery arising distal to a coarctation of the aorta. Ann Surg 1958;147:93-7. |
| 6. | Waltham M, Agrawal V, Bowman L, Hughes C, White GH. Right arm ischemia following intentional stent-graft coverage of an anomalous right subclavian artery. J Endovasc Ther 2005;12:110-4. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
|
Search Pubmed for