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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 7  |  Issue : 4  |  Page : 168-170

Ortner's syndrome: Looking back at this rarity


Department of ENT and Head and Neck Surgery, Mahatma Gandhi Medical College and Research Institute, Sri Balaji Vidyapeeth, Puducherry, India

Date of Web Publication15-Oct-2018

Correspondence Address:
Dr. Satvinder Singh Bakshi
House Number B2, Shree Pushpa Complex, 15th Bharathi Street, Ananda Nagar, Puducherry - 605 009
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JCPC.JCPC_24_18

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  Abstract 

Ortner's syndrome or the cardiovocal syndrome is characterized by hoarseness of voice which is caused by the paralysis of the left recurrent laryngeal nerve as a result of cardiovascular causes. We report two cases; the first one was a 40-year-old male, who presented with hoarseness of voice, dyspnea on exertion, and central chest pain of 6-month duration. Videolaryngoscopy revealed left vocal cord palsy and an echocardiograph showed severe calcific mitral stenosis. The patient underwent mitral valve (MV) replacement and recovered left vocal cord function within 3 months. The second patient was a 52-year-old female, who presented with dyspnea on exertion, intermittent palpitations, and hoarseness of voice. Videolaryngoscopy revealed left vocal cord palsy. Echocardiography revealed features of rheumatic heart disease with severe mitral stenosis, moderate mitral regurgitation, severe tricuspid regurgitation, dilated left atrium, right atrium and right ventricle, and a left atrial appendage thrombus. The patient was subsequently taken up for MV replacement and left atrial thrombectomy, but the left vocal cord palsy persisted after surgery. Cardiovocal syndrome is rare in the general population but associated with causes such as aneurysms and MV stenosis. A high index of suspicion is needed to make an early diagnosis which can lead to surgical correction of the underlying cardiovascular disease, and the pals may resolve spontaneously following treatment.

Keywords: Cardiovocal syndrome, left atrium, mitral stenosis, Ortner's syndrome, vocal palsy


How to cite this article:
Kaipuzha RR, Pulimoottil DT, Bakshi SS, Gopalakrishnan S. Ortner's syndrome: Looking back at this rarity. J Clin Prev Cardiol 2018;7:168-70

How to cite this URL:
Kaipuzha RR, Pulimoottil DT, Bakshi SS, Gopalakrishnan S. Ortner's syndrome: Looking back at this rarity. J Clin Prev Cardiol [serial online] 2018 [cited 2018 Dec 16];7:168-70. Available from: http://www.jcpconline.org/text.asp?2018/7/4/168/243255


  Introduction Top


The causes for vocal cord paralysis are numerous, and the left vocal cord is most at risk, primarily due to the longer course of the left recurrent laryngeal nerve as compared to the right.[1] Of these causes, a quite distinctive cause was first described in 1897 by Norbert Ortner, a Viennese physician who described two patients with mitral stenosis and left recurrent laryngeal nerve paralysis.[2] Ortner's syndrome or the Cardiovocal syndrome is characterized by hoarseness of voice which is caused by the paralysis of the left recurrent laryngeal nerve as a result of cardiovascular causes.[3] This condition was previously quite common due to the high prevalence of untreated rheumatic valvular heart disease, but it is rare these days and hence poses a diagnostic and therapeutic challenge to the modern physician. Here, we report two cases of Ortner's syndrome due to its rarity in our settings.


  Case Reports Top


Case I

A 40-year-old male presented to the Outpatient Department of Otorhinolaryngology of a tertiary care center with complaints of hoarseness of voice, dyspnea on exertion (NYHA Class III), and central chest pain of 6-month duration. The progressive hoarseness of voice had increased in severity over the preceding 3 months. There were no symptoms of cough of swallowing liquids, regurgitation, or dysphagia. General examination showed a normal pulse and blood pressure. Videolaryngoscopy revealed a left vocal cord palsy with the cord in cadaveric position, falling forward of the left aryepiglottic fold, the presence of a phonatory gap [Figure 1]a, and the right vocal cord was seen to be compensating. On auscultation of the chest, the first heart sound and the pulmonary component of the second heart sound were loud, with a mid-diastolic murmur over the cardiac apex (Grade III/VI). Chest radiograph (posteroanterior view) showed cardiomegaly. An electrocardiogram showed a normal sinus rhythm and signs suggestive of left atrial enlargement. A two-dimensional and M-mode echocardiograph was done which showed severe calcific mitral stenosis (mitral valve [MV] orifice Area = 0.9 cm2) and pulmonary artery pressure was 30 mmHg [Figure 1]d. Based on these findings, a diagnosis of Ortner's syndrome secondary to severe calcific mitral stenosis was made, and the patient subsequently underwent MV replacement. The postoperative echocardiogram showed normal prosthetic valve movements and function. A postoperative videolaryngoscopy at 3 months revealed recovery of left vocal cord function.
Figure 1: (a and b) Left vocal cord palsy in patient I and II, respectively; (c) the chest radiograph (PA view) of patient II with features of enlarged LA (black arrow) and enlarged pulmonary artery (white arrow); (d) the echocardiograph of patient I with features of left atrial enlargement (RV = Right ventricle, LV = Left ventricle, RA = Right atrium, LA = Left atrium, MV = Mitral valve, PA = Posteroanterior)

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Case II

A 52-year-old female patient presented to the outpatient department with symptoms of dyspnea on exertion (NYHA Grade III) since 6 years, intermittent palpitations since 1 year, and hoarseness of voice since 6 months. The patient had a history of rheumatic fever in her childhood. General examination revealed a heart rate of 145/min and a pulse rate of 138/min with atrial fibrillation. Cardiovascular examination revealed a loud first heart sound and the pulmonary component of the second heart sound was also loud with a mid-diastolic murmur over the cardiac apex. The electrocardiogram showed a heart rate of 152/min and features of atrial fibrillation. Echocardiography revealed features of rheumatic heart disease with severe mitral stenosis, moderate mitral regurgitation, severe tricuspid regurgitation, pulmonary artery pressure of 112 mmHg, dilated left atrium (LA), right atrium and right ventricle, and a left atrial appendage thrombus. On admission, the chest radiograph showed features of an enlarged LA [Figure 1]c and pulmonary edema. The patient was subsequently taken up for MV replacement and left atrial thrombectomy. The postoperative period was uneventful except for persistent hoarseness of voice. A videolaryngoscopy at 3 months postoperatively showed left vocal cord palsy [Figure 1]b probably due to left recurrent laryngeal nerve compression by a grossly dilated LA and pulmonary artery.


  Discussion Top


The cardiovocal syndrome is characterized by the presence of hoarseness of voice secondary to left recurrent laryngeal nerve palsy due to cardiac or great vessel disease. Although the incidence of the syndrome is infrequent, patients with mitral stenosis are at an increased risk of developing this disease, having an incidence of 0.6%–5%.[4] The left recurrent laryngeal nerve leaves the left vagus nerve at the anterolateral border of the aortic arch in a space between the main pulmonary artery and the aortic arch (aortopulmonary window), just behind the ligamentum arteriosum. Any structure that compresses the nerve within the aortopulmonary window can lead to paralysis of the left vocal cord.[5] Ortner postulated that the enlarged LA which was caused due to mitral stenosis, pushed the left recurrent laryngeal nerve upward, compressing it against the aortic arch, leading to ischemic injury and degeneration of the nerve fibers.[2] Subsequent investigators, however, have contradicted this theory and have concluded that the primary mechanism is the compression of the left recurrent laryngeal nerve between the hypertensive enlarged left pulmonary artery and the aorta near the ligamentum arteriosum.[6] A consensus seems to be lacking on the true pathophysiology, and the two patients in our series seem to inhabit either of these two hypotheses. The ancillary factors that can contribute to the primary mechanism of the injury include mediastinitis, pericarditis, lymphadenopathy, and scarring within the aortic window and anatomic variations of the left bronchus and the ligamentum atreriosum.[7] Other cardiopulmonary disorders which have been associated with Ortner's syndrome include left atrial myxoma, MV prolapsed, aortic aneurysm, patent ductus arteriosus (PDA), primary pulmonary hypertension, atrial and ventricular septal defects, Eisenmenger's syndrome, and recurrent pulmonary embolism.[7] It has been hypothesized by some that patients with arteriosclerotic heart diseases suddenly suffered left recurrent laryngeal nerve paralysis because rapid onset of left ventricular failure produced sudden pulmonary hypertension with acute dilatation of the pulmonary vessels. This phenomenon has been termed as dynamic dilatation. Hoarseness associated with acute cardiac exacerbation has been reported in patients with long-standing PDA, thus corroborating the above proposition.[8]

Since slow or partial injury to the nerve may not always result in hoarseness, routine examination of the vocal fold in all cases of heart disease has been advocated. If palsy of the left vocal fold is visualized, then raised pulmonary artery pressure may be safely deduced. Owing to the rarity of vocal fold paralysis in heart disease, the importance of a constantly dilated pulmonary artery under tension has been stressed.[9]

The most common manifestation of Ortner syndrome is hoarseness of voice. Unilateral vocal cord paralysis increases the risk of aspiration which may be present in up to 40% of patients.[10] Most of the patients with mitral stenosis have gradual development of left atrial hypertension resulting in a large, compliant left atrium and only a passive rise in pulmonary-artery-pressure. As the disease progresses, their cardiac output may become fixed and eventually fall. The large, compliant LA and the low cardiac output may serve to prevent a sudden rise in the pulmonary venous pressure and protect them from paroxysmal dyspnea and pulmonary edema. Such patients may remain asymptomatic for many years and even after the appearance of symptoms, generally shortness of breath and fatigue, may lead comfortable lives. Their degree of disability may be slight. This could explain why patients with Ortner's syndrome do not develop symptoms of heart failure despite significant enlargement of the LA or pulmonary artery. The onset of atrial fibrillation which usually denotes underlying pulmonary hypertension often heralds a progressive decline in the patient's condition and is a poor prognostic sign.[10] Ortner syndrome is suspected from history and clinical examination; a chest X-ray or computed tomography scans of the chest, fibrotic laryngoscopy, and laryngeal electromyography help confirmation of the diagnosis.[10]

The prognosis of recurrent laryngeal nerve paralysis depends on the degree and duration of nerve compression. The treatment of this syndrome depends on the possibilities of managing the underlying cause. If detected early, the syndrome is usually reversible. Patients who can tolerate MV surgery have a good chance of recovering the voice as a result of the reduction in pulmonary artery pressure. Early surgical treatment for all symptomatic and asymptomatic aneurysms has been recommended to avoid complications such as thrombosis, rupture, dissection, or peripheral embolization.[9],[10] The treatment of unilateral vocal cord palsy consists of early rehabilitation, treatment of the primary etiology, and endoscopic insertion of a prosthesis or injection of fat or collagen. Definitive treatment should be considered if aspiration is severe or if no improvement is visible after alleviation of the cardiac problem, depending on the patient's need for better vocalization for occupational or social reasons. There are two absolute indications for surgery: aspiration pneumonia and the patient's desire to improve the voice-related quality of life. Correction of the underlying cause can lead to reversal of the hoarseness, although experience with this remains limited.


  Conclusion Top


Cardiovocal syndrome is rare in the general population but associated with causes such as aneurysms and MV stenosis. A high index of suspicion is needed to make an early diagnosis which can lead to surgical correction of the potentially life-threatening, underlying cardiovascular disease. Following such treatment, the cardiovocal syndrome usually resolves spontaneously as vocal cord function returns, often without a need for additional invasive treatment.

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

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Hamdan AL, Moukarbel RV, Farhat F, Obeid M. Vocal cord paralysis after open-heart surgery. Eur J Cardiothorac Surg 2002;21:671-4.  Back to cited text no. 1
    
2.
Ortner NI. Recurrenslähmung bei mitralstenose. Wien Klin Wochenschr 1897;10:753-5.  Back to cited text no. 2
    
3.
Das SK, Ghosh I, Das A, Dey SK. Mitral regurgitation: A rare cause of Ortner's syndrome. J Clin Diagn Res 2012;6:99-100.  Back to cited text no. 3
    
4.
Solanki SV, Yajnik VH. Ortner's syndrome. Indian Heart J 1972;24:43-6.  Back to cited text no. 4
    
5.
Ali FR, Hails AJ, Yung B. Left recurrent laryngeal nerve palsy secondary to an aortic aneurysm (Ortner's syndrome). Br J Cardiol 2004;11:69-70.  Back to cited text no. 5
    
6.
Ari R, Harvey WP, Hufnagel CA. Etiology of hoarseness associated with mitral stenosis: Improvement following mitral surgery. Am Heart J 1955;50:153-60.  Back to cited text no. 6
    
7.
Chan P, Lee CP, Ko JT, Hung JS. Cardiovocal (Ortner's) syndrome left recurrent laryngeal nerve palsy associated with cardiovascular disease. Eur J Med 1992;1:492-5.  Back to cited text no. 7
    
8.
Nakahira M, Nakatani H, Takeda T. Left vocal cord paralysis associated with long-standing patent ductus arteriosus. AJNR Am J Neuroradiol 2001;22:759-61.  Back to cited text no. 8
    
9.
Meyer E, Jonas NE, Zühlke LJ. Ortner syndrome. S Afr J Child Health 2008;2:170-1.  Back to cited text no. 9
    
10.
Mulpuru SK, Vasavada BC, Punukollu GK, Patel AG. Cardiovocal syndrome: A systematic review. Heart Lung Circ 2008;17:1-4.  Back to cited text no. 10
    


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