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Current Problems in Diagnostic Radiology 000 (2020) 1 6

Current Problems in Diagnostic Radiology

journal homepage: www.cpdrjournal.com

Cardiovascular Hoarseness (Ortner’s Syndrome): A Pictorial Review

Si Wei Kheok, FRCRa,d,*, Parag Ratnakar Salkade, FRCRa,d,*, Ajay Bangaragiri, FRCRb,
Natalie Si Ya Koh, MRCPc, Robert Chun Chen, MDa,d
a
Department of Diagnostic Radiology, Singapore General Hospital, Singapore
b
North Cumbria University Hospital, Carlisle, United Kingdom
c
Department of Cardiology, National Heart Centre, Singapore
d
Singhealth Duke NUS Academic Medical Centre, Singapore

Ortner's syndrome, also known as cardiovocal syndrome, encompasses any cardiac or vascular process that affects the recurrent laryngeal nerve(s), subse-
quently leading to vocal cord palsy. Various causes of Ortner’s syndrome have been described in the literature, which include but are not limited to aortic aneur-
ysms, pulmonary hypertension, left atrial enlargement, and congenital cardiac disorders. We hereby present a pictorial review of the more commonly reported
entities, which have been implicated in this syndrome. Ultimately, understanding the pathophysiology underlying Ortner’s syndrome will help in diagnosis and
institution of potentially life-saving treatment.
© 2020 Elsevier Inc. All rights reserved.

Introduction
When vocal cord (VC) palsy is attributable to a cardiovascular pro-
cess, it is termed Ortner’s syndrome or cardiovocal syndrome. This
was initially described by Norbert Ortner in 1897 for left VC palsy
caused by mitral stenosis. Since his initial description, Ortner’s syn-
drome has been expanded to include other cardiovascular condi-
tions,1 such as left atrial enlargement due to various cardiac or
valvular abnormalities, pulmonary hypertension, aortic disorders,
right subclavian artery aneurysm, and ductus arteriosus aneurysm.1-5
This condition accounts for approximately 1.5% to 6.3% of all the etiol-
ogies for unilateral VC palsy.3 To diagnose this condition, it is important
to scrutinize the entire course of the recurrent laryngeal nerves, includ-
ing its course through the aortopulmonary window. Scanning should
extend inferiorly to include the pulmonary bifurcation.
In some of the cardiovascular pathologies that cause VC palsy,
timely intervention may reverse hoarseness of voice.6,7 More impor-
tantly, VC palsy may serve as a red herring, masking a potentially life-
threatening cardiovascular process requiring immediate treatment.
Anatomy of the Recurrent Laryngeal Nerves
Both recurrent laryngeal nerves (RLN) arise from their respective
vagus nerves. The right vagus nerve gives off the right RLN at the level
of the subclavian artery. The right RLN loops from anterior to poste-
rior around this artery before ascending into the neck within the tra-
cheoesophageal groove. The left vagus nerve gives off the left RLN at
the level of the transverse aortic arch. It loops from anterior to poste-
rior around the vessel and ascends along the posterolateral tracheal
margin. As the left RLN traverses underneath the aortic arch, it passes
*Reprint requests: Si Wei Kheok FRCR, Department of Diagnostic Radiology, Singa-
pore General Hospital, Outram Road, Singapore 169608.
E-mail address: kheok.si.wei@singhealth.com.sg (S.W. Kheok).
through the narrow aortopulmonary window and lies directly poste-
rior to the ligamentum arteriosum (Fig 1).8
Eventually, the RLNs enter the larynx under the cricopharyngeal
muscle and innervate the intrinsic laryngeal muscles responsible for
phonation; dysfunction of the nerves may therefore cause hoarseness.8
Imaging Findings of Vocal Cord Palsy
The most sensitive imaging features that suggest presence of VC
palsy are: (1) abnormal dilatation of the ipsilateral pyriform sinus
(Fig 2a), medial deviation of the aryepiglottic fold (Fig 2b), and ipsilat-
eral laryngeal ventricle dilatation, commonly referred to as the “sail
sign” (Fig 2c and 3).2,8
Etiopathogenesis
Ortner believed that an enlarged left atrium directly compressed
the left RLN nerve. However, anatomical studies by Fetterolf and
Noris9 suggested that the dilated atrium indirectly pushes the left
pulmonary artery cranially toward the aorta, and the left RLN is sub-
sequently compressed between the left pulmonary artery and aortic
arch or ligamentum arteriosum.
Essentially any underlying cardiovascular abnormality that com-
presses the RLN within the aortopulmonary window, which can be as
narrow as 4 mm on cadaveric studies,9 may cause Ortner’s syndrome
(Fig 4).
Aortic Disorders
A meta-analysis of 256 patients with Ortner’s syndrome showed
that aortic pathology accounted for over half of such cases. Aortic
aneurysms from various etiologies account for the bulk of pathology
causing Ortner’s syndrome; large vessel vasculitis and aortic

https://doi.org/10.1067/j.cpradiol.2020.09.015
0363-0188/© 2020 Elsevier Inc. All rights reserved.
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2 S.W. Kheok et al. / Current Problems in Diagnostic Radiology 00 (2020) 1 6

FIG. 1. Diagrammatic representation of the left recurrent laryngeal nerve (purple), which arises from the left vagus nerve (yellow). The left recurrent laryngeal nerve hooks under
the aortic arch, just posterior to the ligamentum arteriosum. It rises in the trachea-esophageal groove and enters the larynx. (Color version of figure is available online.)

fistulization to adjacent organs are less common causes.1 With imme-
diate treatment, the prognosis may be favorable with resolution of
the hoarseness of voice and associated dysphagia. Symptoms may
improve after a few weeks to months.6
Thoracic Aortic Dissecting Aneurysm
Aortic dissection, which accounts for approximately 6.6% of pri-
mary disorders resulting in Ortner’s syndrome,1 can be a life-threat-
ening condition. Although uncommon, it can present insidiously as a
painless process10 or with hoarseness of voice.1
Sudden weakening of the thoracic aorta due to dissection at the
tunica media causes an increase aortic diameter; when it affects the
distal aortic arch,10 it can potentially compress the left RLN at the aor-
topulmonary window.
Diagnosis on chest radiography is difficult, where it presents with
abnormal mediastinal widening (Fig 5a) and displacement of the aor-
tic calcification.11
On computed tomography, a dissection flap(s) separating the true
lumen from the false lumen is usually visualized (Fig 5b). The true
lumen is continuous with the undissected aorta (Fig 5c), while the
false lumen tends to have a wider area compared to the true lumen
(Fig 5d), and may contain a “cobweb” sign that refers to wispy strands
of tunica media due to incomplete shearing in the false lumen.11
Infective Aortic Aneurysm
Infective aortic aneurysm is a rare cause of VC palsy5 that is pre-
sumably due to compression on the RLN.1 There is lack of robust
information whether surrounding inflammation contributes to VC
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S.W. Kheok et al. / Current Problems in Diagnostic Radiology 00 (2020) 1 6
FIG. 2. CT neck of a person with left vocal cord palsy proven on laryngoscopy, shows imaging features compatible with left vocal cord palsy. (A) Axial contrast enhanced CT neck
shows dilatation of the left pyriform sinus (star), (B) medialisation of the left aryepiglottic fold (white arrow) (C) and dilatation of the left laryngeal ventricle (white arrow) at the
level of the true vocal cords.
palsy. The condition is associated with high morbidity and mortality if
untreated.12
Risk factors include damage to the endothelium by atherosclerosis,
iatrogenic arterial injury, and precedent infection.13 It may develop
from hematogenous spread, contiguous spread from adjacent infection
or direct infectious inoculation.12 The most common micro-organisms
cultured are Staphylococcus spp. and Salmonella spp.12,13
Multidetector CT angiography is currently the imaging modality of
choice for diagnosis. Imaging features on any cross sectional imaging
are similar and include a usually saccular aneurysmal outpouching,
(Fig 6a), irregular configuration of the aneurysm wall, disrupted aortic
wall calcification (Fig 6a), surrounding soft tissue fat stranding
FIG. 3. A gentleman with Ortner’s syndrome due to an aortic arch dissecting aneurysm
(black star). Coronal CT of the neck shows dilatation of the left laryngeal ventricle
(white arrow) and atrophic left true vocal cord (black arrow) suggestive of long stand-
ing palsy.
3
(Fig 6b), and periaortic fluid and gas.12,13 A few isolated cases demon-
strated FDG uptake on FDG PET.12,14
Radiological findings overlap in infective and noninfective inflam-
matory aortic aneurysm, such as Ig-G4 disease and large vessel vascu-
litis. These conditions can be distinguished by clinical course and
acuity of symptoms, fever, positive blood cultures, serum IgG4 levels,
and interval change on repeat imaging.13
Pulmonary Hypertension (PH)
The 2013 NICE classification for pulmonary hypertension has
divided pulmonary hypertension into 5 groups (1) pulmonary arterial
hypertension, which includes idiopathic, hereditary, toxin-induced,
connective tissue disease related conditions, (2) PH due to left heart
disease, (3) PH due to lung diseases and/or hypoxia, (4) chronic
thromboembolic pulmonary hypertension, and (5) multifactorial
mechanisms.15,16
The final common pathway for the multitude of entities causing
PH is an enlarged pulmonary artery, which compresses the RLN
upward against the aorta (Fig 7a).4 Hence, if a CT neck is performed to
identify the cause of VC palsy, it is essential that it covers the bifurca-
tion of the main pulmonary artery on the axial plane. On imaging,
pulmonary hypertension (PH) can be suggested when an enlarged
main pulmonary artery diameter measures 29 mm at its bifurcation
FIG. 4. CT thorax of a normal individual (coronal image at the level of the left atrium)
with a normal size heart shows preserved fat in the aortopulmonary window (star).
The left main pulmonary artery (white dotted arrow) is not in contact with the aortic
arch (white solid arrow).
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FIG. 5. Ortner’s syndrome secondary to aortic dissection. A gentleman with voice hoarseness denies having chest pain or trauma. (A) Chest radiograph shows widening of the medi-
astinum and a large bulge of the aortic arch (black arrow). The trachea is displaced to the right (white arrow) (B) Coronal CT aortogram shows thoracoabdominal aortic dissection
and thoracic aneurysm. The aortic dissection is identified by the dissection flap (black arrows) and the mural calcification is displaced toward the lumen (curved white arrows). The
thoracic dissecting aortic aneurysm likely stretched and compressed the left recurrent laryngeal nerve, which travels beneath it. No other abnormality was detected along the left
vagus and recurrent laryngeal nerves that would account for left vocal cord palsy. (C) CT aortogram in sagittal view shows that the true lumen of the aortic aneurysm, containing
denser contrast (black star), is in continuity with the undissected aorta. The dissecting flap (black arrow) separates the true lumen from the false lumen, which has less dense con-
trast (white star). The aortic aneurysm with dissection compresses on the left main pulmonary artery (white triangle), and the fat plane of the underlying aortopulmonary window
is obliterated (purple line). (D) Axial CT thorax shows the thoracic aortic arch aneurysm with hypodense mural thrombus (curved arrow). The aortic dissection extends into the
descending thoracic aorta. In the descending thoracic aorta, the false lumen (white arrow) is larger than the true lumen (black arrow).

(Fig 7b)15 or when its diameter is greater than that of the adjacent
normal aorta.16
Although less sensitive, chest radiograph shows central enlarge-
ment of the pulmonary arteries with rapid pruning of their peripheral
branches (Fig 7c).15,16
When treatment is considered, a cardiac catheterization for the
definitive diagnosis of pulmonary hypertension is required. Pulmo-
nary hypertension is defined as mPAP of 25 mm Hg at rest.17
Left Atrial Enlargement
Left atrial enlargement accounts for approximately 8% of the
reported cases of Ortner’s syndrome.1 It can indirectly compress on
the RLN by pushing the left pulmonary artery cranially toward the
aorta,10 effacing the aortopulmonary window (Fig 8a and b).
Furthermore, left sided cardiac diseases can cause pulmonary hyper-
tension and dilatation, compounding the mass effect on the nerve.
Left atrial enlargement may occur secondary to underlying valvu-
lar disease and cardiomegaly from high output failure.1 The underly-
ing cardiac pathology can be easily assessed with echocardiography.
Chest radiograph shows abnormal widening of the carina (Fig 8c),17
“double density sign’” due to superimposed outline of the left atrium
over the right heart border, and a convex left atrial appendage.
2D echocardiography has also been used to diagnose left atrial
enlargement causing Ortner’s syndrome1. Based on chamber quantifi-
cation guidelines,18 left atrial values indexed to body surface area
should be used. On 2D echocardiography, the upper normal left atrial
volume is 34 mL/m2,18 which is usually less than that measured on
cardiac MRI, where the upper limit of normal for indexed LA volume
is 53 mL/m2 for women and 52 mL/m2 for men.19

FIG. 6. Ortner’s syndrome secondary to infective aortic aneurysm. This patient has pneumonia and bacteremia. He subsequently developed hoarseness of voice. CT neck (not
shown) showed features of vocal cord palsy and no abnormality within the neck to account for the findings. (A) His CT thorax coronal view shows an inferior saccular aneurysm of
the distal aortic arch (white arrow) with disrupted calcifications and irregular wall. (B) Axial CT thorax demonstrates soft tissue thickening and stranding (star) predominantly sur-
rounding the saccular aortic aneurysm (black arrow). (C) Axial CT thorax (lung window) shows other features of thoracic infection, such as a cavitating nodule in the left lung (black
arrow).
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FIG. 7. Ortner’s syndrome secondary to pulmonary hypertension. This patient with has left vocal cord palsy without an apparent cause in the neck. CT thorax was performed and
subsequent 2D echo showed raised mean arterial pressure of 39 mm Hg, in keeping with pulmonary hypertension. (A) Coronal CT pulmonary angiography shows a dilated main pul-
monary trunk (black star) compressing on the aortic arch (white star). The left recurrent laryngeal nerve is likely compressed between pulmonary trunk/left main branch and the
aorta. (B) Axial CT pulmonary angiography demonstrates an enlarged main pulmonary trunk of 5.9 cm (line with arrowed ends). No thromboembolism or lung parenchymal abnor-
mality was noted. (C) Chest radiograph shows enlargement of central pulmonary arteries (solid arrow) with rapid pruning of the peripheral pulmonary arteries. The main pulmo-
nary trunk bulge is prominent (dotted arrow).

FIG. 8. Ortner’s syndrome secondary to left atrial enlargement. This patient has left vocal cord palsy diagnosed on laryngoscopy. (A) Coronal CT neck showed a left atrium (white
star) displacing the pulmonary trunk and left pulmonary artery (black star) upward against the aorta (white arrow), compressing on the expected path of the left recurrent laryngeal
nerve (black arrow). (B) Axial CT neck extended below the aortopulmonary window revealed cardiomegaly with left atrial enlargement (white star) and pericardial effusion (white
arrow) in the same patient. No feature of pulmonary hypertension was detected in the CT or 2D echocardiography (not shown). (C) CXR shows cardiomegaly with cardiothoracic
ratio (a/b) > 0.5 and widened carina (angle) due to left atrial enlargement.

Congenital Causes abnormalities and potentially life-threatening diseases. In the evalua-

tion of vocal cord palsy, it is important to image the entire course of
Congenital causes for Ortner’s syndrome described in the literature the recurrent laryngeal nerves to identify these rare but important
include but are not limited to atrial septal defect, ventricular septal etiologies.
defect, double outlet right ventricle, Eisenmenger’s complex, Ebstein’s
anomaly, patent ductus arteriosus with or without associated patent
Declarations of Interest
ductus arteriosus aneurysm, and aortopulmonary window.1,5
Zaki et al highlighted the importance of left RLN compression by a
None.
dilated pulmonary artery against the aorta in many of the congenital
This research did not receive any specific grant from funding
causes. They presented 3 infant cases of complex congenital heart
agencies in the public, commercial, or not-for-profit sectors.
conditions consisting of several cardiovascular abnormalities, all
complicated by severe pulmonary hypertension with severe pulmo-
nary dilatation. They concluded that in a child with congenital heart Acknowledgment
disease and hoarseness of voice, it is imperative to evaluate for a
dilated pulmonary artery as the causative factor.20 We thank Mr Evan Lim for his help with the medical illustration.

Conclusion
References
Vocal cord palsy can be caused by cardiovascular pathology. The
left recurrent laryngeal nerve is susceptible to compression at the
aortopulmonary window by displacement or enlargement of the
heart and adjacent vessels. Being cognizant of Ortner’s syndrome
allows for early treatment with the possibility of reversing vocal cord
palsy. It enables early diagnosis of underlying cardiovascular
1. Ortner YS. (Cardio-vocal) syndrome: A collective review. Kuwait Med J 2014;46:3–
13.
2. Paquette CM, Manos DC, Psooy BJ. Unilateral vocal cord paralysis: A review of CT
findings, mediastinal causes, and the course of the recurrent laryngeal nerves.
Radiographics 2012;32:721–40.
3. Benninger MS, Gillen JB, Altman JS. Changing etiology of vocal fold immobility.
Laryngoscope 1998;108:1346–50.
 ARTICLE IN PRESS
6 S.W. Kheok et al. / Current Problems in Diagnostic Radiology 00 (2020) 1 6
4. Song SW, Jun BC, Cho KJ, et al. CT evaluation of vocal cord paralysis due to thoracic
diseases: A 10-year retrospective study. Yonsei Med J 2011;52:831–7. Available
from http://dx.doi.org/10.3349/ymj.2011.52.5.831.
5. Mulpuru SK, Vasavada BC, Punukollu GK, et al. Cardiovocal syndrome: A system-
atic review. Heart Lung Circ 2008;17:1–4.
6. Ismazizi Z, Zainal AA. Thoracic aortic aneurysm as a cause of Ortner’s syndrome - A
case series. Med J Malaysia 2016;71:139–41. Available from http://www.embase.
com/search/results?subaction=viewrecord&from=export&id=L611597197.
7. Tammiraju I, Radha Krishna T, Vittal Prasad P, et al. Ortner’s syndrome (cardio
vocal hoarsness) - A rare entitiy in modern era. A case report. IHJ Cardiovasc Case
Rep 2018;2:82–4.
8. Vachha B, Cunnane M, Mallur P, et al. Losing your voice: Etiologies and imaging
features of vocal fold paralysis. J Clin Imaging Sci 2013;3:15. Available from http://
www.clinicalimagingscience.org/text.asp?2013/3/1/15/109751.
9. Fetterolf GNG. The anatomical explanation of paralysis of left recurrent laryngeal
nerve found in certain cases of mitral stenosis. Am J Med Sci 1911;141:625–38.
10. Rylski B, Blanke P, Beyersdorf F, et al. How does descending aorta geometry change
when it dissects? J Am Coll Cardiol 2014;63:1311–9.
11. McMohan MA, Squirrell CA. Multidetector CT of aortic dissection: A pictorial
review. Radiographics 2010;30:445–60.
12. Lee W-K, Mossop PJ, Little AF, et al. Infected (mycotic) aneurysms: Spectrum of
imaging appearances and management. RadioGraphics 2008;28:1853–68. Avail-
able from http://pubs.rsna.org/doi/10.1148/rg.287085054.
13. Kato T, Inoko M. Imaging features and diagnosis of infected aortic aneurysms and
inflammatory diseases involving the aorta. Angiology 2013;1:2–5.
14. Sailer AM, Bakers FC, Daemen JW, et al. 18F-FDG PET / MRI in the diagnosis of an
infected aortic aneurysm. Cardiovasc Diagn Ther 2018;8(Suppl 1):208–11.
 N, Humbert M, Vachiery J-L, et al. 2015 ESC/ERS Guidelines for the diagnosis and
15. Galie
treatment of pulmonary hypertension. Eur Heart J 2016;37:67–119. Available from
https://academic.oup.com/eurheartj/article-lookup/doi/10.1093/eurheartj/ehv317.
16. Aluja Jaramillo F, Gutierrez FR, Díaz Telli FG, et al. Approach to pulmonary hyper-
tension: From CT to clinical diagnosis. RadioGraphics 2018;38:357–73. Available
from http://pubs.rsna.org/doi/10.1148/rg.2018170046.
17. Taskin V, Bates MC, Chillag SA. Tracheal carinal angle and left atrial size. Arch
Intern Med 1991;151:307–8.
18. Lang RM, Badano LP, Mor-Avi V, et al. Recommendations for cardiac chamber
quantification by echocardiography in adults: An update from the American soci-
ety of echocardiography and the European association of cardiovascular imaging.
Eur Heart J Cardiovasc Imaging Elsevier Inc 2015;16:233–71. Available from
http://dx.doi.org/10.1016/j.echo.2014.10.003.
19. Maceira AM, Cosín-Sales J, Roughton M, et al. Reference left atrial dimensions and
volume estimation by steady state free precession cardiovascular magnetic reso-
nance. J Cardiovasc Magn Reson 2010;12:65. Available from http://www.jcmr-
online.com/content/12/1/65.
20. Zaki SA, Asif S, Shanbag P. Ortner syndrome in infants. Indian Pediatr
2010;47:351–3.