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Abstract A 13-year-old girl, who had undergone interrupted aortic arch repair with an 8-mm graft as a neonate and Fontan completion in childhood, developed ventricular fibrillation due to long-QT syndrome. Cardioverter defibrillator implantation was planned. Preoperative catheterization showed a 45-mmHg aortic pressure gradient and ventricular end-diastolic pressure of 11 mmHg. This indicated that recurrent coarctation had adversely affected ventricular function. After consideration of the patient’s age, symptoms and anatomical/surgical complexities, axillo-iliac bypass with cardioverter defibrillator implantation was performed. Postoperative ventricular end-diastolic pressure was 6 mmHg. Axillo-iliac bypass is a surgical option for coarctation that can reduce cardiac afterload. Axillo-iliac bypass, Coarctation, Afterload INTRODUCTION Recurrent coarctation of the aorta (re-CoA) after interrupted aortic arch (IAA) repair is a serious complication in single-ventricle patients . The optimal timing and method of intervention remain controversial, especially for young and asymptomatic patients. We describe a case of asymptomatic re-CoA treated by axillo-iliac bypass with implantation of an implantable cardioverter defibrillator (ICD). CASE REPORT A 13-year-old girl with a single right ventricle, right aortic arch, IAA (Type B) and left atrial isomerism had undergone IAA repair by interposition grafting (8-mm graft) via right thoracotomy as a neonate. In childhood, she underwent a bidirectional Glenn and Damus–Kaye–Stansel procedure followed by Fontan completion via median sternotomy. At 13 years, she developed ventricular fibrillation (VF) due to long-QT syndrome and was referred to our hospital for ICD implantation. Echocardiography showed a 5.6-m/s peak velocity across the interposed graft. Catheterization showed a 45-mmHg aortic pressure gradient and ventricular end-diastolic pressure of 11 mmHg. We inferred that elevated cardiac afterload due to re-CoA had adversely affected single ventricular function and pathogenesis of VF. Therefore, simultaneous intervention for re-CoA with ICD implantation was planned. Computed tomography showed a small-calibre graft in the right aortic arch, a right subclavian artery originating from the distal site of the re-CoA, Damus–Kaye–Stansel anastomosis in the aortic root and a Fontan conduit passing the left side of the dextrocardia (Fig. 1). Despite these anatomical complexities and a history of multiple surgeries, the patient had no symptoms (e.g. hypertension) related to CoA. After thoroughly discussing all surgical options with her family, we chose axillo-iliac bypass, which was not considered ideal but safer than anatomical graft replacement or apico-aortic/aorto-aortic bypass. Under general anaesthesia, epicardial pacing and defibrillator leads were implanted through redo sternotomy, tunnelled to the right subclavian area and connected to the generator. The left axillary artery and external iliac artery were exposed via the subclavian approach and retroperitoneal approach, respectively, and an 8-mm, ringed, expanded polytetrafluoroethylene graft was passed through the subcutaneous tunnel. The graft was anastomosed to each artery with an adequate lengthwise margin to allow further somatic growth. Computed tomography and echocardiography showed a patent graft and a decreased peak velocity (2.3 m/s) across the re-CoA site, respectively (Fig. 2). The ventricular end-diastolic pressure decreased to 6 mmHg. The patient developed no episodes of VF or CoA-related symptoms for 18 months postoperatively. Figure 1: View largeDownload slide Computed tomography showing (A) a small-calibre graft (asterisk), right subclavian artery (white arrow) and Damus–Kaye–Stansel anastomosis (black arrow) and (B) Fontan conduit (white arrow). Des: descending aorta; Eso: oesophagus. Figure 1: View largeDownload slide Computed tomography showing (A) a small-calibre graft (asterisk), right subclavian artery (white arrow) and Damus–Kaye–Stansel anastomosis (black arrow) and (B) Fontan conduit (white arrow). Des: descending aorta; Eso: oesophagus. Figure 2: View largeDownload slide (A) Echocardiography showing a decreased peak velocity across the interposed graft from 5.6 to 2.3 m/s and (B) computed tomography showing a patent axillo-iliac bypass graft. Figure 2: View largeDownload slide (A) Echocardiography showing a decreased peak velocity across the interposed graft from 5.6 to 2.3 m/s and (B) computed tomography showing a patent axillo-iliac bypass graft. DISCUSSION Re-CoA is a serious complication after IAA repair that increases cardiac afterload, which adversely affects long-term prognosis, especially in single-ventricle patients . Surgical options for re-CoA include anatomical graft replacement and apico-aortic/aorto-aortic bypass, which pose major risks , especially in patients with a history of multiple cardiac/aortic operations and anatomical complexities. Therefore, in this case, we selected axillo-iliac bypass because the patient had no CoA-related symptoms, and ICD implantation was the primary indication for surgery. Only a few cases of axillo-iliac/axillofemoral bypass for CoA, leading to improvement of refractory heart failure, have been reported [3, 4]. In our case, axillo-iliac bypass decreased the aortic pressure gradient and ventricular end-diastolic pressure, demonstrating the effectiveness of reducing cardiac afterload. This can potentially prevent or delay further invasive procedures. We chose the iliac artery instead of the femoral artery as the distal anastomosis site mainly because it provides better long-term patency with a shorter bypass and avoids kinking due to hip joint motion. A shorter bypass should be less affected by somatic growth, which potentially poses a risk of anastomotic dehiscence. We considered that our 13-year-old patient, whose growth in height was decreasing, was mature enough to undergo axillo-iliac bypass. However, this strategy should be avoided before the start of growth period in paediatric patients, and careful follow-up is needed. CONCLUSION In conclusion, we report a case of VF and asymptomatic re-CoA in a patient with a single ventricle. She was treated with ICD implantation and axillo-iliac bypass. Axillo-iliac bypass is a less invasive option to reduce cardiac afterload for re-CoA. Conflict of interest: none declared. REFERENCES 1 Cardis BM , Fyfe DA , Mahle WT. Elastic properties of the reconstructed aorta in hypoplastic left heart syndrome . Ann Thorac Surg 2006 ; 81 : 988 – 91 . Google Scholar CrossRef Search ADS PubMed 2 Ugur M , Alp I , Arslan G , Temizkan V , Ucak A , Yilmaz AT. Four different strategies for repair of aortic coarctation accompanied by cardiac lesions . Interact CardioVasc Thorac Surg 2013 ; 17 : 467 – 71 . Google Scholar CrossRef Search ADS PubMed 3 Nakao K , Shibata J , Kikutake S , Nagata T , Uraguchi K , Hidaka Y et al. A case of atypical aortic coarctation with refractory congestive heart failure-treatment with axillo-iliac artery bypass . Clin Cardiol 1998 ; 21 : 523 – 4 . Google Scholar CrossRef Search ADS PubMed 4 Ishizuka M , Yamada S , Maemura S , Yamamot K , Takizawa M , Uozumi H et al. Axillofemoral bypass markedly improved acute decompensated heart failure and kidney injury in a patient with severely calcified stenosis of thoracoabdominal aorta (Atypical aortic coarctation) . Int Heart J 2017 ; 58 : 820 – 3 . Google Scholar CrossRef Search ADS PubMed © The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. This article is published and distributed under the terms of the Oxford University Press, Standard Journals Publication Model (https://academic.oup.com/journals/pages/about_us/legal/notices)
Interactive CardioVascular and Thoracic Surgery – Oxford University Press
Published: Apr 6, 2018
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