Case Report
Right‑sided aortic arch with Kommerell’s aneurysm Sanjay Orathi Patangi, Rajendra Kumar Singh, Henning Pauli Department of Cardiothoracic Anaesthesia and Intensive Care, Freeman Hospital, Newcastle Upon Tyne, UK
ABSTRACT
Received: 27‑01‑14 Accepted: 28‑08‑14
We present a case report of a 55‑year‑old lady who presented with progressive dysphagia and was diagnosed with a Kommerell’s aneurysm and a right‑sided aortic arch. This case report outlines our management strategy and the challenges encountered during the perioperative period in a patient with this rare anomaly. Key words: Anesthetic management; Kommerell’s aneurysm
INTRODUCTION A right‑sided aortic arch is a rare defect first described by Fioratti and Aglietti.[1] This condition is asymptomatic unless aneurismal disease develops. This usually occurs at the origin of the left subclavian artery and is known as a Kommerell’s aneurysm.[2] Surgical correction of this anomaly is challenging. We present a case report which describes the perioperative management of this condition. CASE REPORT
Access this article online
Website: www.annals.in PMID: *** DOI: 10.4103/0971-9784.142075 Quick Response Code:
A 55‑year‑old woman presented with progressive dysphagia and upper gastrointestinal endoscopic finding of severe extrinsic compression of the upper esophagus. Chest X‑ray showed a right‑sided aortic arch, tracheal deviation, scoliosis, and mediastinal widening [Figure 1]. A computerized tomography (CT) scan and cardiac magnetic resonance imaging confirmed the presence of a right‑sided aortic arch and aberrant left subclavian artery originating from the Kommerell’s aneurysm [Figures 2 and 3]. Angiography revealed normal coronaries and pulmonary function testing showed an obstructive defect. She was hypertensive and had recovered from an episode of viral myocarditis 8 months ago. Echocardiography demonstrated moderate bi‑ventricular function. Her preoperative medications
included an angiotensin‑converting enzyme inhibitor and a β blocker. Blood investigations were within normal limits. After a routine premedication, a peripheral venous and right radial arterial cannulae were inserted under local anesthetic. General anesthesia for thoracotomy was instituted. Lung isolation was achieved using a bronchial blocker and confirmed with fiberoptic bronchoscopy. Further monitoring included a central venous catheter in the left internal jugular vein, cerebral oximetry, nasopharyngeal temperature probe and a urinary catheter. The left radial artery and right femoral artery were cannulated to display both proximal and distal pressures in case thoracic aortic clamping became necessary. A nasogastric (NG) tube was inserted. The patient was positioned in the right lateral position for surgery. A lower body forced air heating blanket was used to maintain body temperature. Left thoracotomy was performed, and the abnormal anatomy identified. Care was taken to identify the phrenic and recurrent laryngeal nerves. The left subclavian artery was located and separated from the Kommerell’s aneurysm. The original surgical plan of re‑implanting the left subclavian artery onto the left common carotid artery was abandoned after vascular surgical consultation because of a very short left
Address for correspondence: Dr. Sanjay Orathi Patangi, Department of Cardiothoracic Anaesthesia and Intensive Care, Freeman Hospital, Newcastle Upon Tyne, NE7 7DN, UK. E‑mail: [email protected]
Annals of Cardiac Anaesthesia Vol. 17:4 Sep-Dec-2014
311
Patangi, et al.: Right-sided aortic arch with Kommerell’s aneurysm
Figure 1: Chest radiographs posteroanterior and lateral view. Right sided aortic arch, tracheal deviation, scoliosis and mediastinal widening
the SBP on the right arm and femoral artery were 110 mmHg with a MAP of 70 mmHg. The capillary refill time was normal on the left arm, and it was warm and well perfused with no rise in systemic lactate levels. The Kommerell’s aneurysm was dissected from the aortic arch and esophagus, clamped and ligated. To facilitate this SBP was reduced to 60 mmHg using an infusion of sodium nitroprusside. Concurrently the SBP on the left arm was 40 mmHg with a MAP of 26 mmHg. A paravertebral catheter was inserted for postoperative pain relief. After hemostasis, the bronchial blocker was withdrawn, left lung re‑inflated, and the thoracotomy closed. The patient was transferred to the Intensive Care Unit (ICU) on sedation and was extubated uneventfully within 2 h. There was no rise in lactate levels, and the left arm continued to be well perfused. The patient had an uneventful stay on the ICU and was discharged to the ward the following day. She did not complain of any deficits in the left upper limb during her hospital stay. DISCUSSION
Figure 2: Computed tomography thorax with contrast. (A) Trachea, (B) compressed esophagus, (C) origin of left subclavian artery, (D) Kommerrells aneurysm, (E) distal aortic arch
Figure 3: Computed tomography angiogram (volume rendered image). (A) Ascending aorta, (B) Kommerrells aneurysm, (C) left carotid artery, (D) aberrant left subclavian artery, (E) right subclavian artery, (F) right carotid artery, (G) right sided descending aorta
common carotid artery. The invasive pressures in the left arm showed a systolic blood pressure (SBP) of 70 mmHg with a mean arterial pressure (MAP) of 50 mmHg with evidence of ejection on the trace indicating collateral blood supply. Concurrently 312
A right‑sided aortic arch is a rare congenital anomaly present in 0.05-0.1% of the radiology series[3] and 0.04-0.1% of autopsy series.[2,4] About 50% of right‑sided arches are associated with an aberrant left subclavian artery.[2,4] Several classifications of this anomaly have been described [5,6] after Kommerell first reported it in 1936.[7] The etiology of right arch anomalies is unknown; however, a deletion in chromosome 22q11 is known to be associated with 24% incidence of isolated anomalies of the aortic arch laterality of branching. [8] A right‑sided aortic arch may be asymptomatic, but symptoms develop in adulthood due to early atherosclerotic changes of anomalous vessels/aneurismal dilatation causing compression of adjoining structures manifesting as dysphagia, dyspnea, wheezing, cough, choking spells, recurrent pneumonia, obstructive emphysema or chest pain.[2] The elective operative mortality for this defect ranges widely between 8.3% and 16.6%.[2,9] Various surgical approaches have been advocated to handle this anomaly, including right/left thoracotomy, bilateral thoracotomy, median sternotomy, partial/no cardio‑pulmonary bypass and deep hypothermic circulatory arrest[2] but since the numbers involved are few the best approach cannot be stated. A right thoracotomy provides good exposure to the ascending, transverse arch and descending thoracic aorta in such cases.[2] The choice of approach depends on the individual anatomy and size of the anomaly. Our approach was via a left thoracotomy with a backup plan Annals of Cardiac Anaesthesia Vol. 17:4 Sep-Dec-2014
Patangi, et al.: Right-sided aortic arch with Kommerell’s aneurysm
of going on to left heart bypass if needed. There have been reports, which state that when the reconstruction of the aberrant left subclavian artery is difficult closing its orifice is an acceptable option since it would result in decreased blood loss and shorter surgical times with a caveat that if arm ischemia occurs, vascular reconstruction would be required.[10] Other authors[2] have recommended reconstruction of the left subclavian artery in all patients to prevent arm claudication, and subclavian steal syndrome. In our case we closely monitored the arm for color changes, capillary refill time and lactate levels on the arterial blood gases. Dysphagia has been equivocally stated to be a contraindication for transesophageal echocardiography (TEE).[11] Although the benefits of TEE are well‑known we decided against its use because of severe dysphagia and the endoscopic findings. A NG tube was inserted to help the surgeon to identify the esophagus. Lung isolation for this procedure using double lumen tubes have been debated in literature,[12,13] but in view of the degree of tracheal deviation we opted to use a single lumen tube with a bronchial blocker.
2.
3.
4.
5. 6.
7.
8.
9. 10.
11.
CONCLUSION The presence of a right‑sided aortic arch with Kommerell’s aneurysm is a rare entity. Careful radiologic assessment of the patient’s anatomy and planning of operative strategy with the surgical team is crucial for a successful outcome. REFERENCES 1. Fioratti F, Aglietti F. A case of human right aorta. Anat Rec 1763;45:365.
Annals of Cardiac Anaesthesia Vol. 17:4 Sep-Dec-2014
12.
13.
Cinà CS, Althani H, Pasenau J, Abouzahr L. Kommerell’s diverticulum and right‑sided aortic arch: A cohort study and review of the literature. J Vasc Surg 2004;39:131‑9. Shuford WH, Sybers RG, Gordon IJ, Baron MG, Carson GC. Circumflex retroesophageal right aortic arch simulating mediastinal tumor or dissecting aneurysm. AJR Am J Roentgenol 1986;146:491‑6. Hastreiter AR, D’Cruz IA, Cantez T, Namin EP, Licata R. Right‑sided aorta. I. Occurrence of right aortic arch in various types of congenital heart disease. II. Right aortic arch, right descending aorta, and associated anomalies. Br Heart J 1966;28:722‑39. Felson B, Palayew MJ. The two types of right aortic arch. Radiology 1963;81:745‑59. Stewart JR, Kincaid OW, Edwards JE. An Atlas of Vascular Rings and Related Malformations of the Aortic Arch System. Springfield, Ill: Charles C Thomas; 1964. p. 3‑129. Kommerell B. Verlagerung des oesophagus durcheine abnorm verlaufende arteria subclavia dextra (arteria lusoria). Fortschr Geb Roentgenstr 1936;54:590‑5. McElhinney DB, Clark BJ 3 rd , Weinberg PM, Kenton ML, McDonald‑McGinn D, Driscoll DA, et al. Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching. J Am Coll Cardiol 2001;37:2114‑9. Austin EH, Wolfe WG. Aneurysm of aberrant subclavian artery with a review of the literature. J Vasc Surg 1985;2:571‑7. Osawa H, Shinohara D, Orii K, Hosaka S, Fukuda S, Akashi O, et al. Right aortic arch and Kommerell’s diverticulum repaired without reconstruction of aberrant left subclavian artery. Case Rep Vasc Med 2013;2013:840804. Thys D, Abel M, Brooker R, Cahalan M, Connis R, Nikinovich D, et al. Practical guidelines for peri‑operative transesophageal echocardiography. An updated report by the American Society of Anaesthesiologists and the Society of Cardiovascular Anaesthesiologists Task Force on Transesophageal Echocardiography. Anaesthesiology 2010;112:1‑13. Ishikawa N, Oi M, Maruta K, Iizuka H, Kawaura H. Surgical treatment for right aortic arch with Kommerell’s diverticulum. Asian Cardiovasc Thorac Ann 2013;21:724‑6. Mossad E, Farid I, Youssef G, Ando M. Diverticulum of Kommerell: A review of a series and a report of a case with tracheal deviation compromising single lung ventilation. Anesth Analg 2002;94:1462‑4.
Cite this article as: Patangi SO, Singh RK, Pauli H. Right-sided aortic arch with Kommerell's aneurysm. Ann Card Anaesth 2014;17:311-3. Source of Support: Nil, Conflict of Interest: None declared.
313
Copyright of Annals of Cardiac Anaesthesia is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Right‑sided aortic arch with Kommerell’s aneurysm Sanjay Orathi Patangi, Rajendra Kumar Singh, Henning Pauli Department of Cardiothoracic Anaesthesia and Intensive Care, Freeman Hospital, Newcastle Upon Tyne, UK
ABSTRACT
Received: 27‑01‑14 Accepted: 28‑08‑14
We present a case report of a 55‑year‑old lady who presented with progressive dysphagia and was diagnosed with a Kommerell’s aneurysm and a right‑sided aortic arch. This case report outlines our management strategy and the challenges encountered during the perioperative period in a patient with this rare anomaly. Key words: Anesthetic management; Kommerell’s aneurysm
INTRODUCTION A right‑sided aortic arch is a rare defect first described by Fioratti and Aglietti.[1] This condition is asymptomatic unless aneurismal disease develops. This usually occurs at the origin of the left subclavian artery and is known as a Kommerell’s aneurysm.[2] Surgical correction of this anomaly is challenging. We present a case report which describes the perioperative management of this condition. CASE REPORT
Access this article online
Website: www.annals.in PMID: *** DOI: 10.4103/0971-9784.142075 Quick Response Code:
A 55‑year‑old woman presented with progressive dysphagia and upper gastrointestinal endoscopic finding of severe extrinsic compression of the upper esophagus. Chest X‑ray showed a right‑sided aortic arch, tracheal deviation, scoliosis, and mediastinal widening [Figure 1]. A computerized tomography (CT) scan and cardiac magnetic resonance imaging confirmed the presence of a right‑sided aortic arch and aberrant left subclavian artery originating from the Kommerell’s aneurysm [Figures 2 and 3]. Angiography revealed normal coronaries and pulmonary function testing showed an obstructive defect. She was hypertensive and had recovered from an episode of viral myocarditis 8 months ago. Echocardiography demonstrated moderate bi‑ventricular function. Her preoperative medications
included an angiotensin‑converting enzyme inhibitor and a β blocker. Blood investigations were within normal limits. After a routine premedication, a peripheral venous and right radial arterial cannulae were inserted under local anesthetic. General anesthesia for thoracotomy was instituted. Lung isolation was achieved using a bronchial blocker and confirmed with fiberoptic bronchoscopy. Further monitoring included a central venous catheter in the left internal jugular vein, cerebral oximetry, nasopharyngeal temperature probe and a urinary catheter. The left radial artery and right femoral artery were cannulated to display both proximal and distal pressures in case thoracic aortic clamping became necessary. A nasogastric (NG) tube was inserted. The patient was positioned in the right lateral position for surgery. A lower body forced air heating blanket was used to maintain body temperature. Left thoracotomy was performed, and the abnormal anatomy identified. Care was taken to identify the phrenic and recurrent laryngeal nerves. The left subclavian artery was located and separated from the Kommerell’s aneurysm. The original surgical plan of re‑implanting the left subclavian artery onto the left common carotid artery was abandoned after vascular surgical consultation because of a very short left
Address for correspondence: Dr. Sanjay Orathi Patangi, Department of Cardiothoracic Anaesthesia and Intensive Care, Freeman Hospital, Newcastle Upon Tyne, NE7 7DN, UK. E‑mail: [email protected]
Annals of Cardiac Anaesthesia Vol. 17:4 Sep-Dec-2014
311
Patangi, et al.: Right-sided aortic arch with Kommerell’s aneurysm
Figure 1: Chest radiographs posteroanterior and lateral view. Right sided aortic arch, tracheal deviation, scoliosis and mediastinal widening
the SBP on the right arm and femoral artery were 110 mmHg with a MAP of 70 mmHg. The capillary refill time was normal on the left arm, and it was warm and well perfused with no rise in systemic lactate levels. The Kommerell’s aneurysm was dissected from the aortic arch and esophagus, clamped and ligated. To facilitate this SBP was reduced to 60 mmHg using an infusion of sodium nitroprusside. Concurrently the SBP on the left arm was 40 mmHg with a MAP of 26 mmHg. A paravertebral catheter was inserted for postoperative pain relief. After hemostasis, the bronchial blocker was withdrawn, left lung re‑inflated, and the thoracotomy closed. The patient was transferred to the Intensive Care Unit (ICU) on sedation and was extubated uneventfully within 2 h. There was no rise in lactate levels, and the left arm continued to be well perfused. The patient had an uneventful stay on the ICU and was discharged to the ward the following day. She did not complain of any deficits in the left upper limb during her hospital stay. DISCUSSION
Figure 2: Computed tomography thorax with contrast. (A) Trachea, (B) compressed esophagus, (C) origin of left subclavian artery, (D) Kommerrells aneurysm, (E) distal aortic arch
Figure 3: Computed tomography angiogram (volume rendered image). (A) Ascending aorta, (B) Kommerrells aneurysm, (C) left carotid artery, (D) aberrant left subclavian artery, (E) right subclavian artery, (F) right carotid artery, (G) right sided descending aorta
common carotid artery. The invasive pressures in the left arm showed a systolic blood pressure (SBP) of 70 mmHg with a mean arterial pressure (MAP) of 50 mmHg with evidence of ejection on the trace indicating collateral blood supply. Concurrently 312
A right‑sided aortic arch is a rare congenital anomaly present in 0.05-0.1% of the radiology series[3] and 0.04-0.1% of autopsy series.[2,4] About 50% of right‑sided arches are associated with an aberrant left subclavian artery.[2,4] Several classifications of this anomaly have been described [5,6] after Kommerell first reported it in 1936.[7] The etiology of right arch anomalies is unknown; however, a deletion in chromosome 22q11 is known to be associated with 24% incidence of isolated anomalies of the aortic arch laterality of branching. [8] A right‑sided aortic arch may be asymptomatic, but symptoms develop in adulthood due to early atherosclerotic changes of anomalous vessels/aneurismal dilatation causing compression of adjoining structures manifesting as dysphagia, dyspnea, wheezing, cough, choking spells, recurrent pneumonia, obstructive emphysema or chest pain.[2] The elective operative mortality for this defect ranges widely between 8.3% and 16.6%.[2,9] Various surgical approaches have been advocated to handle this anomaly, including right/left thoracotomy, bilateral thoracotomy, median sternotomy, partial/no cardio‑pulmonary bypass and deep hypothermic circulatory arrest[2] but since the numbers involved are few the best approach cannot be stated. A right thoracotomy provides good exposure to the ascending, transverse arch and descending thoracic aorta in such cases.[2] The choice of approach depends on the individual anatomy and size of the anomaly. Our approach was via a left thoracotomy with a backup plan Annals of Cardiac Anaesthesia Vol. 17:4 Sep-Dec-2014
Patangi, et al.: Right-sided aortic arch with Kommerell’s aneurysm
of going on to left heart bypass if needed. There have been reports, which state that when the reconstruction of the aberrant left subclavian artery is difficult closing its orifice is an acceptable option since it would result in decreased blood loss and shorter surgical times with a caveat that if arm ischemia occurs, vascular reconstruction would be required.[10] Other authors[2] have recommended reconstruction of the left subclavian artery in all patients to prevent arm claudication, and subclavian steal syndrome. In our case we closely monitored the arm for color changes, capillary refill time and lactate levels on the arterial blood gases. Dysphagia has been equivocally stated to be a contraindication for transesophageal echocardiography (TEE).[11] Although the benefits of TEE are well‑known we decided against its use because of severe dysphagia and the endoscopic findings. A NG tube was inserted to help the surgeon to identify the esophagus. Lung isolation for this procedure using double lumen tubes have been debated in literature,[12,13] but in view of the degree of tracheal deviation we opted to use a single lumen tube with a bronchial blocker.
2.
3.
4.
5. 6.
7.
8.
9. 10.
11.
CONCLUSION The presence of a right‑sided aortic arch with Kommerell’s aneurysm is a rare entity. Careful radiologic assessment of the patient’s anatomy and planning of operative strategy with the surgical team is crucial for a successful outcome. REFERENCES 1. Fioratti F, Aglietti F. A case of human right aorta. Anat Rec 1763;45:365.
Annals of Cardiac Anaesthesia Vol. 17:4 Sep-Dec-2014
12.
13.
Cinà CS, Althani H, Pasenau J, Abouzahr L. Kommerell’s diverticulum and right‑sided aortic arch: A cohort study and review of the literature. J Vasc Surg 2004;39:131‑9. Shuford WH, Sybers RG, Gordon IJ, Baron MG, Carson GC. Circumflex retroesophageal right aortic arch simulating mediastinal tumor or dissecting aneurysm. AJR Am J Roentgenol 1986;146:491‑6. Hastreiter AR, D’Cruz IA, Cantez T, Namin EP, Licata R. Right‑sided aorta. I. Occurrence of right aortic arch in various types of congenital heart disease. II. Right aortic arch, right descending aorta, and associated anomalies. Br Heart J 1966;28:722‑39. Felson B, Palayew MJ. The two types of right aortic arch. Radiology 1963;81:745‑59. Stewart JR, Kincaid OW, Edwards JE. An Atlas of Vascular Rings and Related Malformations of the Aortic Arch System. Springfield, Ill: Charles C Thomas; 1964. p. 3‑129. Kommerell B. Verlagerung des oesophagus durcheine abnorm verlaufende arteria subclavia dextra (arteria lusoria). Fortschr Geb Roentgenstr 1936;54:590‑5. McElhinney DB, Clark BJ 3 rd , Weinberg PM, Kenton ML, McDonald‑McGinn D, Driscoll DA, et al. Association of chromosome 22q11 deletion with isolated anomalies of aortic arch laterality and branching. J Am Coll Cardiol 2001;37:2114‑9. Austin EH, Wolfe WG. Aneurysm of aberrant subclavian artery with a review of the literature. J Vasc Surg 1985;2:571‑7. Osawa H, Shinohara D, Orii K, Hosaka S, Fukuda S, Akashi O, et al. Right aortic arch and Kommerell’s diverticulum repaired without reconstruction of aberrant left subclavian artery. Case Rep Vasc Med 2013;2013:840804. Thys D, Abel M, Brooker R, Cahalan M, Connis R, Nikinovich D, et al. Practical guidelines for peri‑operative transesophageal echocardiography. An updated report by the American Society of Anaesthesiologists and the Society of Cardiovascular Anaesthesiologists Task Force on Transesophageal Echocardiography. Anaesthesiology 2010;112:1‑13. Ishikawa N, Oi M, Maruta K, Iizuka H, Kawaura H. Surgical treatment for right aortic arch with Kommerell’s diverticulum. Asian Cardiovasc Thorac Ann 2013;21:724‑6. Mossad E, Farid I, Youssef G, Ando M. Diverticulum of Kommerell: A review of a series and a report of a case with tracheal deviation compromising single lung ventilation. Anesth Analg 2002;94:1462‑4.
Cite this article as: Patangi SO, Singh RK, Pauli H. Right-sided aortic arch with Kommerell's aneurysm. Ann Card Anaesth 2014;17:311-3. Source of Support: Nil, Conflict of Interest: None declared.
313
Copyright of Annals of Cardiac Anaesthesia is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
