A novel procedure for reconstructing an extensive hypoplastic aortic arch in older children

CASE REPORT – CONGENITAL Interactive CardioVascular and Thoracic Surgery 24 (2017) 132–134 doi:10.1093/icvts/ivw320 Advance Access publication 22 September 2016 Cite this article as: Wu S, Yang Y, Hu S, Zhao T. A novel procedure for reconstructing an extensive hypoplastic aortic arch in older children. Interact CardioVasc Thorac Surg 2017;24:132–4. A novel procedure for reconstructing an extensive hypoplastic aortic arch in older children Sijie Wu, Yifeng Yang, Shijun Hu and Tianli Zhao* Department of Cardiovascular Surgery, The Second Xiangya Hospital, Central South University, Changsha, China * Corresponding author. Department of Cardiovascular Surgery, The Second Xiangya Hospital, Central South University, Changsha 410011, China. Tel: +86-731-85296106; fax: +86-731-85296606; e-mail: (T. Zhao). Received 19 April 2016; received in revised form 14 August 2016; accepted 25 August 2016 Abstract Aortic arch reconstruction is the key to successfully repairing an interrupted aortic arch (IAA) with tubular hypoplasia of the aortic arch (THAA), especially in older children. We report a novel reconstruction technique involving aortapulmonary fusion that was used to treat THAA in a 9 year-old patient with IAA. In this procedure, the underside of the aortic arch and the upside of the main pulmonary artery were fused to reconstruct the aortic arch. The short-term outcome of the procedure has been promising. This procedure may represent an alternative for repairing extensive THAA in older children. Keywords: Congenital heart disease • Interrupted aortic arch • Hypoplastic arch • Older children INTRODUCTION Tubular hypoplasia of the aortic arch (THAA) often occurs in patients with interrupted aortic arch (IAA) and severe coarctation of the aorta. Many aortic arch reconstruction techniques have been used in neonates and infants with this malformation [1, 2]. However, surgeons face more challenges regarding the management of extensive THAA in older children. We developed a novel procedure to successfully repair THAA in older children with IAA. CASE REPORT A 9 year-old girl presenting with shortness of breath on exertion was admitted for further evaluation and treatment. Preoperative transthoracic echocardiography (TTE) and multidetector CT (MDCT) showed an IAA and THAA, a large but short patent ductus arteriosus (PDA), an isolated apical muscular ventricular septal defect (VSD), a secundum atrial septal defect and pulmonary hypertension [mean pulmonary artery pressure (mPAP) 60 mmHg] (Fig. 1A). Catheterization revealed that her Qp/Qs ratio was 5.3 and that her pulmonary vascular resistance was 8.5 Wood Units·m2. After median sternotomy, the muscular VSD was occluded with an appropriate device under transoesophageal echocardiographic guidance. For aortic arch reconstruction, an arc incision was made along the anterior aspect of the main pulmonary artery (MPA) and extended to the undersurface of the aortic arch and the ascending aorta. Ductal tissue was not identified during the procedure, so the PDA was left intact. A bovine pericardial patch was sutured to the MPA and the reconstructed aortic arch to separate the two vessels. The edges of the incision along the anterior aspect of the MPA were anastomosed to those on the undersurface of the aortic arch and ascending aorta using a running suture (Fig. 2). The atrial septal defect was closed with a pericardial patch. The patient had an uneventful postoperative course and has received oral Bosentan since discharge. Postoperative MDCT demonstrated a well-reconstructed aortic arch and descending aorta without any obstructions (Fig. 1B). TTE showed that her mPAP decreased (mPAP 28 mmHg) and that the pressure gradient across the isthmus was 24 mmHg. The pressure gradient was slightly increased (48 mmHg, 3 m/s), and a 3 mm residual muscular VSD shunt (V = 2 m/s) was present at 3 months after surgery; however, at 2 years after surgery, TTE showed that the PG had stabilized at
36 mmHg (2.5 m/s), and no residual shunt was observed. Neither cardiac valve dysfunction nor pulmonary stenosis was observed during follow-up. COMMENTS Aortic arch reconstruction is the key to successfully repairing an IAA with THAA. The extended end-to-end anastomosis and aortic arch advancement are preferred in neonates and young infants [3, 4]. An artificial blood vessel may be used only in adult patients. Pulmonary homograft material may also be used, but it is not available in China. Thus, we developed the aorta–pulmonary fusion procedure, in which the growth potential of the reconstructed arch is preserved due to the use of autologous vessel tissues. Meanwhile, extensive dissection can be avoided, reducing both operation time and the risk of nerve injury and bleeding. © The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. S. Wu et al. / Interactive CardioVascular and Thoracic Surgery 133 CASE REPORT Figure 1: Preoperative (A) and postoperative (B) multidetector computed tomography examination results. MPA: main pulmonary artery. Figure 2: Schematic illustration of the aortic arch reconstruction. (A) An incision was made in the main pulmonary artery (MPA) parallel to the inferior margin of the aortic arch. (B) The orifices of the descending aorta and bilateral pulmonary arteries were visible after incision. A large but short patent ductus arteriosus (PDA) connected directly to the descending aorta orifice (DAO). (C) A bovine pericardial patch was placed to act as the dome of the pulmonary artery and the base of the reconstructed aortic arch. (D) The aortic arch was reconstructed via the creation of an anastomosis joining the anterior aspect of the MPA and the inferior surface of the aortic arch. LPA: left pulmonary artery; RPA: right pulmonary artery. 134 S. Wu et al. / Interactive CardioVascular and Thoracic Surgery The limitations of this technique should be discussed. It should be used in patients with a sufficiently large MPA. In this case, the PDA was almost the same size as the descending aorta and ductal tissue was not identified during the procedure. To reduce the risks of injury and bleeding, we elected to leave the PDA intact. Pulmonary valve reflux is a possible complication of the procedure, but no pulmonary valve abnormalities were observed in this patient at 2 years of follow-up, most likely because the reconstruction site was sufficiently distal to the pulmonary valves. Conflict of interest: none declared. REFERENCES [1] Hart JC, Waldhausen JA. Reversed subclavian flap angioplasty for arch coarctation of the aorta. Ann Thorac Surg 1983;36:715–7. [2] Backer CL, Paape K, Zales VR, Weigel TJ, Mavroudis C. Coarctation of the aorta: repair with polytetrafluoroethylene patch aortoplasty. Circulation 1995;92:II-132–6. [3] Kotani Y, Anggriawan S, Chetan D, Zhao L, Liyanage N, Saedi A et al. Fate of the hypoplastic proximal aortic arch in infants unde (...truncated)