|Year : 2015 | Volume
| Issue : 2 | Page : 44-46
Student's corner: Cardiovocal hoarseness - Challenges in diagnosis and management
Paresh Naik, Jayita Poduval
Department of ENT, Pondicherry Institute of Medical Sciences, Puducherry, India
|Date of Web Publication||13-Jun-2016|
Dr. Jayita Poduval
5-B-7, JIPMER Quarters, Dhanvantari Nagar, Puducherry - 605 006
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Cough and hoarseness are common symptoms that may be encountered in general or specialist clinical practice. Hoarseness, especially when found in isolation, could have an origin other than in the airway. An important example of this is cardiovocal hoarseness. A literature review of cardiovocal hoarseness or Ortner's syndrome reveals many clinical presentations. Several case reports have been published, but none so far has put into context issues that could come in the way of diagnosis and management of such patients. Hoarseness as a symptom, when not explained by usual causes such as a preceding upper respiratory tract infection or allergy, must be expediently looked into by simple means such as an indirect laryngoscopy. If a vocal cord paralysis is evident, especially on the left side, the possibility of an underlying cardiac condition must be borne in mind. Surgical or nonsurgical intervention could resolve the problem except in cases where the patient is extremely moribund.
Keywords: Cardiovocal, hoarseness, Ortner′s syndrome
|How to cite this article:|
Naik P, Poduval J. Student's corner: Cardiovocal hoarseness - Challenges in diagnosis and management. J Laryngol Voice 2015;5:44-6
| Introduction|| |
Cough and hoarseness are common complaints in clinical practice. A cough is considered more sinister of the two, and as a matter of public education, a medical consultation is advised for any cough that persists for more than 3 weeks. In sharp contrast, however, hoarseness as a symptom is considered less urgent for evaluation. As a matter of fact, the American Academy of Otolaryngology - Head and Neck Surgery Foundation recommends evaluation of hoarseness by means of laryngoscopy only if the hoarseness has been there for 3 months or more, although some authors have strongly refuted this. 
| Discussion|| |
The recurrent laryngeal nerves arise from the vagus or the 10 th cranial nerve on either side, even though their origin might be at a slightly different level for the left and right side. The right recurrent laryngeal nerve curves at the level of the right subclavian artery and hooks around this artery to reach its final destination at the right cricothyroid joint to supply the right vocal cord. The left recurrent laryngeal nerve is longer as it descends into the chest before it branches from the vagus nerve at the level of the transverse aortic arch to reach the left cricothyroid joint to supply the left vocal cord. It hooks under the arch of the aorta and lies posterior to the ligamentum arteriosum before it makes its way toward the neck between the trachea and the esophagus. Because of the longer and more circuitous course of the left recurrent laryngeal nerve, vocal cord paralysis due to an extracervical cause is more common on the left side.
Cardiovocal hoarseness, also known as Ortner's syndrome, is a rare entity.  Conservative estimates place the incidence of this phenomenon between 0.6% and 5%. Cardiovocal hoarseness is so named after Norbert Ortner, who was the first to describe this in 1897. He found this condition in a series of cases of mitral valve stenosis leading to enlargement of the left atrium.  Subsequently, this terminology was also extended to other cardiac conditions.  The more common among these are aortic aneurysms  and pulmonary hypertension,  and even mitral valve prolapse  and mitral valve regurgitation.  Atrial myxoma,  aortic dissection  and pulmonary embolism  have also been implicated in certain cases. Procedures such as defibrillation,  transcatheter access for the ablation of atrial fibrillation,  and major cardiothoracic surgery  such as open heart surgery and heart-lung transplantation  could also be responsible. It was initially assumed that an enlarged left atrium was the main culprit, but it is now appreciated that it is the arterial pressure in the pulmonary artery  that is, responsible for causing the compression of the recurrent laryngeal nerve-either left or right in most of the cases. Congestive heart failure due to systemic hypertension  could also lead to the same elevated pulmonary artery pressure found in valvular heart disease, and make up a fair number of cases of the cardiovocal syndrome. Congenital heart disease such as Eisenmenger complex  (ventricular septal defect with the pulmonary vascular obstructive disease), atrial septal defect  and a patent ductus arteriosus  can, in the same manner, account for increase in arterial pressure in the pulmonary circulation, thereby leading to the cardiovocal hoarseness.
Cardiovocal hoarseness could exist along with cough in the same patient. It could also be accompanied by pain due to mucosal inflammation or muscular and chest wall strain caused by the cough. When cough is painless, however, it could be due to previous cardiac surgery that might have caused denervation of the sympathetic supply to the heart.  This is especially of concern in the case of an aortic dissection that might present with hoarseness instead of the acute chest pain that it is commonly associated with. Even bronchiectasis  has been reported with mitral stenosis.
The recurrent laryngeal palsy leading to vocal cord immobility was attributed to the compression of the recurrent laryngeal nerve between a dilated pulmonary artery and the corresponding segment of the aorta and/or aortic ligament, based on an assessment of the chest X-ray and also from postmortem studies in the affected patients. 
Nowadays, the term Ortner's syndrome is generally ascribed to any cardiac, intrathoracic or nonmalignant involvement of either recurrent laryngeal nerve.  Furthermore, in theory, it is possible for the right recurrent laryngeal nerve to be affected in cases of situs inversus though no such case has been reported so far.
Several factors could come in the way of both diagnosis and management of cardiovocal hoarseness. First, without a high index of suspicion, many would attribute the hoarseness to the patient's smoking habit or a recent URI, or even debility due to age. Thus, it has been suggested that an indirect laryngoscopy should, in fact, be included in the initial evaluation of a patient with hoarseness even when the patient is primarily under treatment by another specialist. 
Second, empirical treatment for infection, allergy or gastroesophageal reflux is often instituted and continued for indefinite periods of time.  Indirect laryngoscopy, if and when it really has been undertaken, is often vague and nonspecific and is quite often observer dependent, thus leading to errors in diagnosis. 
Third and a significant challenge to diagnosis is that complete evaluation of the cardiac condition is many a time difficult owing to medical co-morbidities. For example, a chest X-ray showing mediastinal widening could be suggestive of a cardiac condition, but factors such as tumors of the mediastinum or even mediastinal lymphadenopathy due to tuberculosis cannot be ruled out. To do this, other tests might be required. These are generally invasive and carry an element of risk. A plain CT scan or MRI of the chest might not be sufficient for diagnosis, and a contrast CT or angiography might be needed. A limiting factor for any of these is a renal compromise as is common in many patients suffering from heart disease. In some cases, an endoscopically guided transesophageal ultrasound study might also be of use, especially in doubtful cases of an aortic aneurysm.  In other cases, the cause might be pulmonary thrombo embolism with hypertension,  requiring additional tests such as perfusion scans for confirmation.
The fourth challenge lies in the management of these patients. Patients diagnosed early and/or those who are younger in age can be managed optimally by surgical or nonsurgical means, with a resolution of hoarseness and the underlying heart condition.  Elderly patients and those with co-morbidities are more difficult to deal with, and renal, hepatic or neurological dysfunction could come in the way of corrective heart surgery. Treatment of the cardiac condition in these cases would revolve around medication to control hypertension and hyperlipidemia, with or without blood thinners. The hoarseness due to vocal cord paralysis is usually tackled with speech and voice therapy to enhance compensation by the opposite vocal cord.  In a selected number of cases, a vocal cord adduction or tightening procedure or even a medialization thyroplasty could be undertaken under local anesthesia. This should be attempted wherever possible because compensation by the opposite vocal cord might be inadequate and the patient would be at risk of aspiration, besides having a poorer quality of life due to impaired phonation.
The fifth and final challenge refers to the failure of treatment of the heart condition. Aneurysms arising from the suture line of a mitral valve repair have been reported.  This of course, is diagnosed and managed on similar lines as the erstwhile primary problem, that is, prompt diagnosis and surgical correction whenever possible. Atrial fibrillation could result from pulmonary hypertension and could indicate a worsening prognosis.  A vocal cord paralysis could also be a poor prognostic indicator of rupture of an aortic aneurysm,  more the reason for maintaining a high level of vigilance in all patients presenting with unexplained hoarseness of voice.
| Conclusion|| |
Otolaryngologists need to be highly alert regarding hoarseness as a symptom and try to increase awareness about the same in sister specialties.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Keesecker SE, Murry T, Sulica L. Patterns in the evaluation of hoarseness: Time to presentation, laryngeal visualization, and diagnostic accuracy. Laryngoscope 2015;125:667-73.
Fennessy BG, Sheahan P, McShane D. Cardiovascular hoarseness: An unusual presentation to otolaryngologists. J Laryngol Otol 2008;122:327-8.
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.
Mulpuru SK, Vasavada BC, Punukollu GK, Patel AG. Cardiovocal syndrome: A systematic review. Heart Lung Circ 2008;17:1-4.
Harano M, Tanemoto K, Kuinose M, Kanaoka Y, Kagawa S, Yoshioka T. A case of chronic traumatic dissecting aneurysm of the thoracic aorta. Kyobu Geka 1994;47:1023-5.
Heikkinen J, Milger K, Alejandre-Lafont E, Woitzik C, Litzlbauer D, Vogt JF, et al.
Cardiovocal Syndrome (Ortner′s Syndrome) associated with chronic thromboembolic pulmonary hypertension and giant pulmonary artery aneurysm: Case report and review of the literature. Case Rep Med 2012;2012:230736.
Kishan CV, Wongpraparut N, Adeleke K, Frechie P, Kotler MN. Ortner′s syndrome in association with mitral valve prolapse. Clin Cardiol 2000;23:295-7.
Rubens F, Goldstein W, Hickey N, Dennie C, Keon W. Hoarseness secondary to left atrial myxoma. Chest 1989;95:1139-40.
Lee SI, Pyun SB, Jang DH. Dysphagia and hoarseness associated with painless aortic dissection: A rare case of cardiovocal syndrome. Dysphagia 2006;21:129-32.
Albertini RE. Vocal cord paralysis associated with pulmonary emboli. Chest 1972;62:508-10.
Victoria L, Graham SM, Karnell MP, Hoffman HT. Vocal fold paralysis secondary to cardiac countershock (cardioversion). J Voice 1999;13:414-6.
Pai RK, Boyle NG, Child JS, Shivkumar K. Transient left recurrent laryngeal nerve palsy following catheter ablation of atrial fibrillation. Heart Rhythm 2005;2:182-4.
Tewari P, Aggarwal SK. Combined left-sided recurrent laryngeal and phrenic nerve palsy after coronary artery operation. Ann Thorac Surg 1996;61:1721-2.
Murty GE, Smith MC. Recurrent laryngeal nerve palsy following heart-lung transplantation: Three cases of vocal cord augmentation in the acute phase. J Laryngol Otol 1989;103:968-9.
Diefenbach WC. Left vocal-cord paralysis associated with hypertensive heart disease. N Engl J Med 1949;240:419.
Talley JD, Fowler K. Tetralogy of fallot (Eisenmenger type) with hypoplasia of dextraposed aorta. Am J Med Sci 1936;191:618-26.
Dolowitz DA, Lewis CS. Left vocal cord paralysis associated with cardiac disease. Am J Med 1948;4:856-62.
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.
Collins JS, Evangelista A, Nienaber CA, Bossone E, Fang J, Cooper JV, et al.
Differences in clinical presentation, management, and outcomes of acute type A aortic dissection in patients with and without previous cardiac surgery. Circulation 2004;110 11 Suppl 1:II237-42.
Monwarul Islam AK, Zaman S, Doza F. Ortner syndrome due to concomitant mitral stenosis and bronchiectasis. Korean Circ J 2012;42:507-10.
Stocker HH, Enterline HT. Cardio-vocal syndrome: Laryngeal paralysis in intrinsic heart disease. Am Heart J 1958;56:51-9.
Subramaniam V, Herle A, Mohammed N, Thahir M. Ortner′s syndrome: Case series and literature review. Braz J Otorhinolaryngol 2011;77:559-62.
Annema JT, Brahim JJ, Rabe KF. A rare cause of Ortner′s syndrome (cardiovocal hoarseness). Thorax 2004;59:636.
Mohamed AL, Zain MM. Hoarseness of voice in a patient with mitral stenosis and Ortner′s syndrome. Malays J Med Sci 2004;11:65-8.
Prada-Delgado OO, Barge-Caballero E. Ortner′s syndrome. N Engl J Med 2011;365:939.
Grout CJ, Simpson KA, Clements MR. Ortner′s syndrome: An unusual cause of cough. Br J Cardiol 2012;19:48-9.
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.