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  Table of Contents 
Year : 2017  |  Volume : 18  |  Issue : 2  |  Page : 82-85

Airway and esophageal compression from double aortic arch in a case of pentalogy of Fallot: Anesthetic management

Department of Cardiac Anesthesia, All Institute of Medical Sciences, New Delhi, India

Date of Submission16-Sep-2017
Date of Acceptance26-Sep-2017
Date of Web Publication12-Dec-2017

Correspondence Address:
Prof. Sambhunath Das
Department of Cardiac Anesthesia, 7th Floor, Cardio Thoracic Sciences Centre, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/TheIAForum.TheIAForum_20_17

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Double aortic arch (DAA) is a rare disease requiring high index of clinical suspicion for diagnosis. If undiagnosed, it can pose a serious challenge during anesthetic induction because of dynamic nature of tracheal compression. When DAA is associated with other congenital heart diseases, anesthetic management becomes even more challenging. We report the perioperative anesthetic management of a very rare case of DAA associated with pentalogy of Fallot.

Keywords: Airway compression, double aortic arch, esophageal compression, pentalogy of Fallot, transesophageal echocardiography

How to cite this article:
Das S, Aggarwal S. Airway and esophageal compression from double aortic arch in a case of pentalogy of Fallot: Anesthetic management. Indian Anaesth Forum 2017;18:82-5

How to cite this URL:
Das S, Aggarwal S. Airway and esophageal compression from double aortic arch in a case of pentalogy of Fallot: Anesthetic management. Indian Anaesth Forum [serial online] 2017 [cited 2023 Jan 31];18:82-5. Available from: http://www.theiaforum.org/text.asp?2017/18/2/82/220553

  Introduction Top

Double aortic arch (DAA) is a rare congenital aortic arch (AA) anomaly having two separate right and left aortic arches.[1] Surgical intervention usually required when symptoms appear such as respiratory distress and dysphagia. The association with other congenital cardiac disease masks the diagnosis and adds more risk during management.[2] The combination of atrial septal defect (ASD), ventricular septal defect (VSD), right ventricular hypertrophy, right ventricular outflow tract obstruction (RVOTO), and overriding of the aorta is called Pentalogy of Fallot (POF). We discuss an extremely rare case of DAA associated with POF.

  Case Report Top

A 1-year-old boy, 5-kg weight, presented to our hospital with a history of recurrent pneumonia and bluish discoloration of nail beds. He also had difficulty in swallowing associated with suck rest suck cycle and hypercyanotic spell. Transthoracic echocardiography (TTE) diagnosed him as a case of POF but with spatial disorientation. There was single subaortic VSD with ostium secundum ASD with RVOTO and pulmonary valvular stenosis. The PA branches were bilaterally confluent. Computed tomography (CT) angiography [Figure 1] was performed for identification of aortopulmonary collaterals. DAA was detected in the CT with narrowing of the distal trachea. The left arch of DAA was compressing the left main stem bronchus. The thoracic aorta was right sided. The patient was planned for DAA repair with intracardiac repair (ICR) for POF.
Figure 1: Angiography of double aortic arch

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The patient was advised for fasting and premedication as per the institutional protocol. Inside the operating room, electrocardiogram, pulse oximetry in both upper limbs, and noninvasive blood pressure monitoring were started; and inhalational induction using incremental concentrations of sevoflurane was initiated. Intravenous line was established with spontaneous respiration. After confirming successful bag and mask ventilation, fentanyl 2 mcg/kg, and rocuronium 1 mg/kg were administered. The patient was intubated successfully with 4-mm uncuffed endotracheal tube, and bilateral equal air entry was confirmed. Arterial pressure and central venous catheters were secured in femoral vessels. Neonatal transesophageal echocardiography (TEE) probe was inserted in addition to temperature probe and urinary catheter, and near-infrared spectroscopy (NIRS) sensor was applied. Intraoperative TEE confirmed the presence of POF and detected the presence of DAA [Figure 2]a and [Figure 2]b.
Figure 2: (a) Two-dimensional transesophageal echocardiographic modified upper esophageal ascending aortic color compare view showing two aortic arches (double aortic arch, R-right aortic arch, and L-left aortic arch). (b) Two-dimensional transesophageal echocardiographic midesophageal four chamber color compare view showing atrial septal defect and ventricular septal defect (indicated by white arrows)

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After midline sternotomy, left AA [Figure 3] was clamped to test the blood flow in the aorta from femoral artery pressure, NIRS, [Table 1] and oxygen saturation in bilateral upper limbs. There was no vascular compromise with the test clamp. Left-sided AA was divided; the stumps were sutured and closed. ICR was also planned on cardiopulmonary bypass (CPB) in the same sitting. Septal defects were closed using Dacron patch along with resection of the right ventricular infundibular tract. A tiny interatrial septal opening was left open which was supposed to act as patent foramen ovale and was helping in decompressing the increased right-sided chamber pressures. The child was weaned off CPB uneventfully.
Figure 3: Intraoperative image showing looping of two arches of ascending aorta

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Table 1: The near-infrared spectroscopy value during dissection of left aortic arch

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Postoperatively, TEE findings showed successful repair of DAA, no residual VSD, and mean gradient across RVOT of 8 mmHg. Heparin was reversed with protamine. Hemodynamics was maintained with fluid, blood, blood components, sodium nitroprusside, dobutamine, and dopamine. Intraoperative course was uneventful, and the patient shifted to the intensive care unit. He was extubated next day, and later, discharged without any residual esophageal or airway compression.

  Discussion Top

DAA is an abnormality of AA as a consequence of the failure of regression and persistence of right and left fourth aortic arches during fetal life.[1] It may form an incomplete or complete vascular ring.[1] It could be right or left dominant or balanced.[1] The incidence of DAA is rare among congenital heart disease.[2] It can have concomitant TOF, coarctation of aorta, VSD, and patent ductus arteriosus.[3],[4],[5] The approximate incidence of POF combined with DAA is 1 in 24 × 106 per live birth.[6] The presentations depend on the degree of compression of esophagus and trachea.[5] Our patient presented with recurrent pneumonia, respiratory distress, dysphagia, and cyanosis. All the findings might be attributed to TOF. Although the patient had dysphagia, DAA was never suspected in diagnosis.

TTE may not be reliable for diagnosing DAA as happened in our case.[7] The definitive diagnosis is made either by angiography or CT.[5],[8] We were fortunate that CT angiography was advised to find out aortopulmonary collaterals; otherwise, the diagnosis might have been missed.

The treatment is surgical ligation and division of minor segment. The surgeon should ensure that division should be carried out without compromising blood flow to the descending thoracic aorta or the carotid arteries. This is usually done by ipsilateral thoracotomy in fourth intercostal space. However, if intracardiac defects are associated, then median sternotomy is preferred, similar to our case.

The anesthetic concerns include securing a patent airway.[9] Blood loss from injury to major vessels during dissection and multiple anastomosis is a possibility. The extensive combined surgery requires adequate pain control using intravenous or neuroaxial opioids. Our patient had no major respiratory compression, so the induction was smooth without any difficulty. We used sevoflurane for induction with the maintenance of spontaneous respiration keeping in view the dynamic nature of airway obstruction.

Intraoperative TEE could diagnose DAA by careful scanning of the upper esophageal views as in our case. Choosing a smaller size TEE probe is better option to negotiate through the compressed esophagus due to DAA. TEE helped to diagnose the interatrial septal aneurysm, confirm the findings of POF, monitoring of cardiac functions, de-airing of intracardiac air, and postoperative assessment of the surgical corrections.

Major complications include persistence of respiratory symptoms as result of tracheomalacia, injury to the diaphragm, recurrent laryngeal nerve, thoracic duct, and phrenic nerve. Fortunately, no such complications occurred in our patient postoperatively.

Since PA branches were confluent, the two major surgeries in one sitting were possible; it reduced the hospital stay and financial burden. Otherwise, a staged repair is planned after a palliative shunt.[4] TEE monitoring and neurologic monitoring such as NIRS are helpful to guide, detect, and manage the cardiac abnormality and neurological adverse events during manipulation, clamping, and dissection of DAA.

  Conclusion Top

Association of DAA and POF is a very rare presentation. If not diagnosed early, it can be fatal due to missed diagnosis with overlapping symptoms. Therefore, it requires a high clinical suspicion for early recognition. Management of airway and esophageal compression from DAA is essential. Neurological and cardiovascular monitoring and appropriate intervention are helpful for the successful outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


The authors would like to thank Dr P. Rajashekhar (Associate Professor, CTVS, AIIMS, New Delhi) for describing the intraoperative surgical findings.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Elumalai G, Senguttuvan E. Double aortic arch embryological basis and surgical implications. Elixir Int J 2016;100:43414-9.  Back to cited text no. 1
Kumar S, Aggarwal S, Satyarthi S, Satsangi D. Tetralogy of Fallot with double aortic arch: A rare association. Indian J Thorac Cardiovasc Surg 2015;31:28-30.  Back to cited text no. 2
Harper AR, Dai M, Prabhatha RM. Tetralogy of fallot with double aortic arch. Cardiol Young 2011;21:695-6.  Back to cited text no. 3
Singh Y. Double aortic arch with tetralogy of Fallot: A rare association. Asian Cardiovasc Thorac Ann 2009;17:433-4.  Back to cited text no. 4
Alsenaidi K, Gurofsky R, Karamlou T, Williams WG, McCrindle BW. Management and outcomes of double aortic arch in 81 patients. Pediatrics 2006;118:e1336-41.  Back to cited text no. 5
Tiraboschi R, Manasse E, Parenzan L. Tetralogy of Fallot associated with a double aortic arch: Palliation with a prosthetic tube graft. Tex Heart Inst J 1988;15:131-3.  Back to cited text no. 6
Li ZQ, Liu AJ, Li G, Liu Y, Zhu YB, Liu YL, et al. Two cases of a rare association: Double aortic arch with tetralogy of Fallot. J Cardiothorac Surg 2013;8:39.  Back to cited text no. 7
Backer CL, Mavroudis C, Rigsby CK, Holinger LD. Trends in vascular ring surgery. J Thorac Cardiovasc Surg 2005;129:1339-47.  Back to cited text no. 8
Andndropoulos DB, Stayer SA, Russell IA, Mossad EB. Anaesthesia for Congenital Heart Diease. 2nd ed. Wiley-Blackwell; 2011. p. 480-2.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


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