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 Table of Contents  
Year : 2019  |  Volume : 4  |  Issue : 2  |  Page : 49-52

Aortic arch aneurysm presenting as ortner's syndrome

1 Department of Interventional Cardiology, Indraprastha Apollo Hospital, New Delhi, India; Department of Internal Medicine, Kaduna State University, Kaduna, Nigeria
2 Department of Internal Medicine, Kaduna State University, Kaduna, Nigeria
3 Department of Interventional Cardiology, Indraprastha Apollo Hospital, New Delhi, India

Date of Submission11-May-2018
Date of Decision27-Jan-2019
Date of Acceptance08-Apr-2019
Date of Web Publication20-Jan-2020

Correspondence Address:
Peter Dodo Yakubu
Consultant Cardiologist/Lecturer Barau Dikko Teaching Hospital/Kaduna State University and Clinical Associate (Fellow- Interventional Cardiology and Vascular Interventions) Indraprastha Apollo Hospital, New Delhi

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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/archms.archms_17_18

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Ortner's syndrome is defined as left recurrent laryngeal nerve palsy resulting from cardiovascular disease. Though it was first reported in patients with dilated left atrium in mitral stenosis, many cases have been associated with other cardiovascular diseases such as thoracic aortic aneurysm, enlarge pulmonary artery, abberent subclavian artery etc. We report a case of 45 year old presented with hoarseness and dyspnea who was confirmed to have aortic arch aneurysm. His symptoms resolved completely after transthoracic endovascular aortic repair (TEVAR)

Keywords: Aortic arch, aneurysm presenting, Ortner's syndrome

How to cite this article:
Yakubu PD, Khanna NN, Edafe EA, Rao S, Abubakar AB. Aortic arch aneurysm presenting as ortner's syndrome. Arch Med Surg 2019;4:49-52

How to cite this URL:
Yakubu PD, Khanna NN, Edafe EA, Rao S, Abubakar AB. Aortic arch aneurysm presenting as ortner's syndrome. Arch Med Surg [serial online] 2019 [cited 2024 Feb 29];4:49-52. Available from: https://www.archms.org/text.asp?2019/4/2/49/276181

  Introduction Top

Ortner's syndrome, also referred to as cardiovocal syndrome, is a clinical condition associated with left recurrent laryngeal nerve palsy resulting from cardiovascular disease. This syndrome can result from enlargement or aneurysm of the surrounding structures. It was first described in 1897 by Norbert Ortner (Austrian physician) who ascribed hoarseness of voice with left recurrent laryngeal nerve palsy secondary to a dilated left atrium in three patients with mitral stenosis. We report a case of Ortner's syndrome complicating thoracic aortic aneurysm successfully managed by transthoracic endovascular aortic repair (TEVAR).

  Case Report Top

A 45-year-old male presented with a 2-month history of hoarseness of voice and dyspnea. There were no cough, no anterior neck swelling, no hematemesis, and no dysphagia. He had no history of fever and no weight loss. He is a known hypertensive and diabetic diagnosed during the course of this illness in a referring hospital and has been on treatment. Laryngoscopic examination showed features of left vocal cord paralysis, and no mass was detected in the upper respiratory tract. His blood pressure was 120/70 mmHg, and other examination findings were not remarkable. Fasting blood glucose was 125 mg/dL.

Computerized tomographic angiography (CTA) revealed aneurysm of the arch of the aorta [Figure 1]. A diagnosis of Ortner's syndrome complicating aortic arch aneurysm was made.
Figure 1: Aortic aneurysm

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The patient was planned for combined carotid-carotid bypass and hybrid aortic stent-graft technique. Carotid-carotid bypass created by a vascular surgeon was noted to have block. This necessitated the change of technique to aortic hybrid graft extending from the region just below the left subclavian artery and deployment of subclavian chimney graft to preserve the vessel due to inadequate landing zone. Immediate postprocedure check revealed adequate flow with no endoleaks. His symptoms improved remarkably immediate postprocedure, and he was discharged 2 days later on antiplatelets, antibiotics, oral antidiabetics, and antihypertensives. He was seen 2 weeks later with complete resolution of the hoarseness and dyspnea.

He, however, presented 2 months later with fever, pus discharge, and pains in the neck (surgical site). He then, who had wound dressings, treated with antibiotics, and bypass graft was removed. He responded to treatment and was discharged after 2 weeks of admission. The patient has remained asymptomatic since then and has been on follow-up.

Computerized tomographic angiography (CTA) done at 2½ years of follow-up showed endograft in place with no expansion of the aneurysm and no endoleaks [Figure 2].
Figure 2: After percutaneous endovascular aortic repair

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  Discussion Top

Ortner's syndrome also known as “cardiovocal syndrome” is a rare syndrome of left recurrent laryngeal nerve palsy resulting from cardiovascular disease.[1] Although it usually presents with hoarseness of voice, dysphagia and dyspnea on speech may also occur.[1]

The syndrome was first described by the Australian physician Norbert Ortner in 1897 when he reported hoarseness due to left recurrent laryngeal nerve palsy in three patients with enlarged left atrium in mitral stenosis.[2]

Although Ortner's syndrome was first reported in cases of mitral stenosis with left atrial enlargement, the syndrome has also been observed in patients with thoracic aortic aneurysm, severe pulmonary hypertension, enlarged pulmonary artery, aberrant subclavian artery, left atrial myxoma, and complication of cardiothoracic surgeries.[3],[4]

Along its course, the left recurrent laryngeal nerve loops around the arch of the aorta and then passes between the aortic arch and the pulmonary artery before entering the tracheoesophageal fascia inferior to the level of the second thoracic vertebra.[5] This makes it susceptible to compression in cases of aortic arch aneurysms/dissection or pulmonary artery enlargement leading to its paralysis resulting in cardiovocal syndrome.

Ortner's syndrome is considered an uncommon cause of vocal cord paralysis.[6] It is only considered after excluding the more common etiologies such as laryngitis, tracheitis, and laryngeal tumors.[6],[7] However, the practice is likely to change as there are an increasing number of cases of aortic aneurysm in recent years.[8] With the increasing number of patients having thoracic aortic aneurysm, more cases of cardiovocal syndrome are to be expected. Aortic aneurysm is a cause of Ortner's syndrome only in 5% of cases.[9] The hoarseness may be of sudden onset, subtle, intermittent, or progressive in nature and its occurrence connotes imminent disease.[4],[9] Most asymptomatic aortic aneurysms are discovered incidentally, and symptomatic patients usually present with complications.[9],[10] Hoarseness may be the only symptom of aortic dissection.[11]

In a review of 500 patients with thoracic aneurysm who had open surgical repair, de Bakey et al. reported hoarseness due to recurrent laryngeal nerve palsy in 43 (8.6%).[12] Teixido and Leonetti observed vocal cord palsy presenting with hoarseness in 8 (4.8%) of 168 cases of thoracic aortic aneurysms.[13] Only one regained vocal cord function after surgical repair of the aneurysm.[13]

The management of thoracic aortic aneurysm depends on the aneurysm size, presence or absence of complications, affectation of aortic arch vessels, presence or absence of penetrating ulcers or dissections, and the rate of aneurysm expansion.[14] The most important aspect of the primary treatment is adequate blood pressure control. Beta-blockers are the first-line agents. If the maximum tolerated dose is reached and blood pressure control is not achieved, other antihypertensives are added to achieve target blood pressure value of 105–120 mmHg.[14] This may aid to reduce the rate of expansion of the aneurysm, thereby preventing the occurrence of acute aortic syndromes.[14] Patients are to be monitored on follow-up bases with regular imaging surveillance to monitor the expansion of the aneurysm for early intervention.[14]

Aortic repair is indicated according to the current guidelines in asymptomatic patients when the maximum diameter of the aneurysm is >5.5 cm or if the rate of expansion is rapid (>5 mm in 6 months).[15] The guidelines also recommended aortic repair when aneurysm reaches 5 cm in individuals with increased risk of aortic dissections such as Marfan syndrome, bicuspid aortic valves, or saccular aneurysm.[15] Aortic repair is also recommended for aneurysm <5 cm for patients with extremely high risk of dissection.[15] For patients with increased operative risk such as elderly, morbidly obese, presence of severe renal disease, and severe pulmonary disease, target diameter of 6 cm is recommended.[14],[15]

Open surgery was the only treatment modality for many years. It was, however, associated with high mortality and morbidity, especially in patients with severe aneurysm symptoms, increasing age, cardiogenic shock, preoperative angina, severe renal disease, diabetics, postoperative tracheostomy, stroke, postoperative heart dysfunction, etc.[16]

In 1994, Dake et al. first reported the use of endovascular stent graft constructed by them to repair descending thoracic aneurysm in 44 high-risk patients for open surgical repair.[17] Only one patient required conversion to open heart surgery. Three early deaths and two late deaths were reported.[17] This paves the way for the emergence of thoracic endovascular aortic repair (TEVAR) which has been found to have low morbidity and mortality but requires lifelong follow-up.[18]

Gore TAG trial was the randomized trial for thoracic endograft in the United States. The results showed that TAG device was superior to open heart surgery in terms of efficacy and safety. It has lower mortality of 11.7% while open heart surgery had mortality 21%.[18] It also had markedly lower rates of paraparesis/paraplegia, respiratory difficulties, and renal dysfunction when compared to open surgery procedure. This result led to the United States Food and Drug Administration approval of Gore TAG endograft system in 2005 for endovascular repair of descending thoracic aneurysm.[18]

International controlled clinical trial of TEVAR with the Zenith TX2 endovascular graft showed that the TX2 endograft is a safer and effective alternative to open surgical repair for the treatment of anatomically suitable descending thoracic aneurysms and ulcers at 1 year of follow-up.[19] Result of a prospective multicenter trial of CTAG thoracic endograft designed to evaluate the safety and effectiveness of the device in the repair of descending aneurysm showed low device events, with no graft compression, no fractures, no conversions, and aneurysm ruptures of the treated segment through years.[20]

Endovascular aortic repair has gained wide acceptance as the preferred treatment option over open surgery for treating thoracic aortic aneurysm over the past decade.[21] For the purpose of treatment, the thoracic aorta has been divided into five zones (Z0–Z4). Z0 extends from the aortic root to the end of the innominate artery, Z1 from the end of the innominate artery to the end of the left common carotid, Z2 from the end of the left common carotid to the end of the left subclavian artery, Z3 from the end of the left subclavian artery to the end of the aortic arch, and Z4 is the descending thoracic aorta. TEVAR has been the treatment option for zone 3 and 4 aneurysms.[14],[21]

TEVAR has also been used for the treatment of zone 2 and 1 diseases with the advent of chimney grafts, fenestrated grafts, and hybrid techniques. Multilayered flow modulator is used for aneurysms involving zone 0–2 and complex aortic aneurysms.[14],[15],[21] TEVAR has been used as a treatment modality with favorable outcome for complicated Type B dissections such as patients with persistent or recurrent pains, uncontrolled hypertension despite maximum drug therapy, early aortic expansion, inadequate perfusion, and clinical features of aortic rupture.[14],[21]

Thoracic endovascular aortic repair is contraindicated in patients with connective tissue disease like Marfan syndrome except as a salvage procedure in emergency cases or bridge to open surgical repair. Sometimes, it can also be performed in connective tissue disease as a treatment option following previous open surgical aortic repair if the landing zones are within the position of the previous prosthetic grafts.[14],[15],[21] Conventionally, adequate fixation zones of at least 2 cm is necessary for proper deployment, and long-lasting fixation of endograft on either side of the aortic disease is very vital in patients being prepared for TEVAR. Hybrid technique combined open and endovascular techniques. It involves debranching the arch to create the adequate proximal landing zone followed by stenting over the aortic arch. Chimney graft is used to preserve the aortic arch branches in patients with aortic disease. Chimney technique involves the deployment of stents into the aortic arch branches with the proximal part positioned parallel to the thoracic endograft.[22] It is placed slightly above it to provide adequate blood flow.[22] Access vessel of at least 8 mm in diameter is required for a standard 24 French delivery device.[15],[21],[22] If femoral access vessel size is not adequate, a conduit of 10 mm can be used to attach the iliac artery through the retroperitoneal incision.[21],[22] Spinal drainage catheter is recommended for patients with previous abdominal aortic aneurysm repair or individuals requiring extensive stent graft coverage.[15],[21],[22] This serves as a preventive measure for paraplegia/paraparesis from spinal injury.[15],[21],[22]

After completion of TEVAR, close monitoring of peripheral pulses, hemodynamic function, and neurological examination are necessary. Furthermore, lifelong follow-up and serial imaging to assess for symptoms recurrence, endoleaks, and aneurysm expansion/shrinkage are required.[14],[15],[21],[22]

  Conclusion Top

Thoracic aortic aneurysm is an important cause of Ortner's syndrome, and a high index of suspicion is required for the diagnosis. Transthoracic endovascular aortic repair (TEVAR) has emerged as the preferred treatment modality for aortic aneurysm with favorable 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.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

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Ortner N. Recurrent laryngeal nerve palsy in patients with mitral stenosis. Wien Klin Wochenschr (German) 1897;10:753-5.  Back to cited text no. 2
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Escribano JF, Carnés J, Crespo MA, Antón RF. Ortner's syndrome and endoluminal treatment of a thoracic aortic aneurysm: A case report. Vasc Endovascular Surg 2006;40:75-8.  Back to cited text no. 4
Haller JM, Iwanik M, Shen FH. Clinically relevant anatomy of recurrent laryngeal nerve. Spine (Phila Pa 1976) 2012;37:97-100.  Back to cited text no. 5
Rizvi MM, Singh RB, Jain A, Sarkar A. Asymptomatic aortic aneurysm causing right vocal cord palsy and hoarseness: A rare presentation. Anesth Essays Res 2014;8:397-400.  Back to cited text no. 6
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Grabenwoger M, Alfonso F, Bachet J, Bonser R, Czerny M, Eggerbrecht H, et al. Thoracic endovascular repair for the treatment of aortic diseases: A position from the European Association for Cardio – Thoracic Surgeons (EACTS) and European Society of Cardiology (ESC), in collaboration with the European Association of Percutaneous Cardiovascular Interventions (EAPCI). Eur Heart J 2012;33:1558-63. [doi: 10.1093].  Back to cited text no. 15
Crawford ES, Svensson LG, Coselli JS, Safi HJ, Hess KR. Surgical treatment of aneurysm and/or dissection of the ascending aorta, transverse aortic arch, and ascending aorta and transverse aortic arch. Factors influencing survival in 717 patients. J Thorac Cardiovasc Surg 1989;98:659-73.  Back to cited text no. 16
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  [Figure 1], [Figure 2]


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