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Year : 2015  |  Volume : 1  |  Issue : 3  |  Page : 270-272

Ortner's (cardiovocal) syndrome: A rare presenting manifestation of critical mitral stenosis

1 Department of Medicine, ESI Medical College, Joka, Kolkata, West Bengal, India
2 Department of Medicine, Medical College, Joka, Kolkata, West Bengal, India

Date of Web Publication23-Feb-2016

Correspondence Address:
Krishnarpan Chatterjee
B-7/11, Diamond Park, Diamond Harbour Road, Joka, Kolkata - 700 104, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2395-5414.177265

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We present a case of left recurrent laryngeal palsy in a 50-year-old lady who was the sole manifestation of critical mitral stenosis. In the absence of a giant left atrium, pulmonary artery hypertension with entrapment of recurrent laryngeal nerve (RLN) in the aortic window may have contributed to compression of left RLN. Mitral stenosis was relieved with balloon mitral valvotomy which lead to significant improvement in her hoarseness.

Keywords: Cardiovocal syndrome, critical mitral stenosis, Ortner′s syndrome

How to cite this article:
Chatterjee K, Sen C. Ortner's (cardiovocal) syndrome: A rare presenting manifestation of critical mitral stenosis. J Pract Cardiovasc Sci 2015;1:270-2

How to cite this URL:
Chatterjee K, Sen C. Ortner's (cardiovocal) syndrome: A rare presenting manifestation of critical mitral stenosis. J Pract Cardiovasc Sci [serial online] 2015 [cited 2023 Jun 4];1:270-2. Available from: https://www.j-pcs.org/text.asp?2015/1/3/270/177265

  Introduction Top

Ortner's (cardiovocal) syndrome is a clinical condition with left recurrent laryngeal nerve (RLN) palsy and hoarseness secondary to cardiovascular disease. In this case report, a 50-year-old lady presented with an isolated complaint of hoarseness of voice with no history of cardiac illness or clinical findings pointing to a valvular disease. After investigations, she was diagnosed to have critical mitral stenosis with pulmonary artery hypertension. Mitral stenosis has often been found as a cause for cardiovocal syndrome. Ortner's syndrome as the presenting manifestation of critical mitral stenosis in an endemic region such as India is a rare occurrence.

  Case Report Top

A 50-year-old postmenopausal woman presented to us with insidious, progressive hoarseness of voice for the past 2 years. She had no history of dysphagia or odynophagia. There was no history of cough, expectoration, anorexia, or weight loss. She denied any history of breathlessness, palpitation, chest pain, syncope, or swelling of the legs. There was no past history of episodic fever with arthritis. She was a nonsmoker and had no history of diabetes, hypertension, or heart disease.

On examination, her pulse rate was 82 bpm and regular. Blood pressure was 110/70 mm of Hg in the upper limb. There was no pallor, icterus, cyanosis, clubbing, lymphadenopathy, or edema. Cardiovascular examination revealed a parasternal heave and a loud S 1 . There were no audible murmurs. The rest of the systemic examination was normal.

Direct laryngoscopy revealed a fixed, immobile left vocal cord. The electrocardiogram showed atrial fibrillation with left atrial enlargement. Chest X-ray showed cardiomegaly with prominence of upper lobe pulmonary veins [Figure 1]. Echocardiography revealed a stenotic mitral valve with an area of 1.0 cm 2 ; left atrial diameter was 4.47 cm [Figure 2] and [Figure 3]. Mean mitral valve pressure gradient was 25 mm of Hg with moderate pulmonary artery hypertension and tricuspid regurgitation. There was no mitral regurgitation. Echo score was 9. Left ventricular function was normal. Aortic root calcification was present without involvement of the aortic valve leaflets. A diagnosis of Ortner's syndrome due to critical mitral stenosis was made.
Figure 1: Chest X-ray showing cardiomegaly and antler sign.

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Figure 2: Mitral valve Doppler gradient and area by pressure half time.

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Figure 3: Left atrium enlargement by M-mode.

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She was worked up and then underwent balloon mitral valvotomy successfully. She was started on oral anticoagulation therapy and advised rheumatic fever prophylaxis at discharge. After 6 months follow-up, her hoarseness had improved significantly.

  Discussion Top

Ortner was the first to describe left RLN palsy, leading to hoarseness in three cases with mitral stenosis. [1] Later, it was also described with various other cardiovascular diseases like congenital heart disease, aortic aneurysm, valvular heart disease, and secondary pulmonary hypertension. [2] Ortner ascribed the RLN palsy to compression of the left RLN by a dilated left atrium (LA). Later studies showed that compression of the left RLN was more likely between the aorta and pulmonary artery near the ligamentum arteriosum. [3],[4]

RLN is a branch of the vagus nerve [Figure 4]. There are two RLNs, and they emerge from the vagus nerve at the level of the arch of aorta, and then travel up the side of the trachea to the larynx. The left nerve loops under the aortic arch, and the right nerve loops under the right subclavian artery. The nerves follow a "recurrent" course, moving in the opposite direction to the nerve they branch from, and this is why they are called the RLNs.
Figure 4: Recurrent laryngeal nerve.

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RLN palsy causes the ipsilateral vocal cord to be paralyzed in the paramedian position. This leads to hoarseness, difficulty in coughing, and breathlessness during speech. Risk of aspiration is also increased. The full extent of symptoms depends on how much the normal vocal cord is able to compensate for the paralyzed vocal cord.

In mitral stenosis, the incidence of cardiovocal syndrome was reported to range between 0.6% and 5% in 1972. [5] Cardiovocal syndrome secondary to mitral stenosis is rare nowadays due to early diagnosis and prompt management. Reported cases have documented giant (LA diameter up to 13 cm) and pulmonary artery hypertension. [6],[7]

Our case is unique as Ortner's syndrome was the presenting syndrome of critical mitral stenosis. Second, in our patient, LA was moderately enlarged (diameter 4.47 cm). In this scenario, compression of the left RLN in the aortic window might have contributed to palsy.

Treating the underlying cause remains the primary management of Ortner's syndrome. If there is no improvement, vocal cord medialization surgery can be done.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Ortner N. Recurrent laryngeal nerve paralysis due to mitral valve stenosis. Wien Klin Wochenschr 1897;10:753-5.  Back to cited text no. 1
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. 2
Fetterolf G, Norris G. The anatomical explanation of paralysis of left recurrent laryngeal nerve found in certain case of mitral stenosis. Am J Med Sci 1911;141:625-38.  Back to cited text no. 3
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. 4
Solanki SV, Yajnik VH. Ortner′s syndrome. Indian Heart J 1972;24:43-6.  Back to cited text no. 5
Gulel O, Koprulu D, Kucuksu Z, Yazici M, Cengel S. Images in cardiovascular medicine. Cardiovocal syndrome associated with huge left atrium. Circulation 2007;115:e318-9.  Back to cited text no. 6
Plastiras SC, Pamboucas C, Zafiriou T, Lazaris N, Toumanidis S. Ortner′s syndrome: A multifactorial cardiovocal syndrome. Clin Cardiol 2010;33:E99-100.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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