Ortner's syndrome

Overview

Ortner's syndrome, also known as cardiovocal syndrome, is a rare condition characterized by hoarseness of voice due to paralysis of the left recurrent laryngeal nerve (RLN), caused by cardiovascular pathology. The syndrome was first described in 1897 by Austrian physician Norbert Ortner in a patient with severe mitral stenosis and left atrial enlargement. The left RLN loops under the aortic arch and is particularly susceptible to compression by enlarged cardiovascular structures, leading to vocal cord paralysis and associated symptoms.

Ortner's syndrome is not a primary laryngeal disorder but a consequence of extralaryngeal pathology, specifically affecting the nerve as it traverses the thoracic cavity. Prompt recognition of the syndrome is essential because the underlying cause is often a serious cardiovascular condition that requires medical or surgical intervention.

Causes

Ortner's syndrome occurs when the left recurrent laryngeal nerve is compressed, stretched, or otherwise affected by abnormal cardiovascular structures. The left RLN is more prone to involvement than the right due to its longer intrathoracic course and its location beneath the aortic arch near the ligamentum arteriosum.

Common Cardiovascular Causes

• Mitral stenosis: Leading to left atrial enlargement, historically the most common cause

• Aortic aneurysm: Particularly aneurysms of the aortic arch or descending thoracic aorta

• Pulmonary hypertension: Resulting in dilation of the pulmonary artery and compression of the RLN

• Left atrial enlargement: Due to various valvular heart diseases

• Patent ductus arteriosus (PDA): Especially if associated with aneurysmal changes

• Thoracic aortic dissection

• Vascular tumors or mediastinal masses

Symptoms

The hallmark symptom of Ortner’s syndrome is hoarseness of voice due to left vocal cord paralysis. Other symptoms may reflect the underlying cardiovascular condition or result from RLN dysfunction.

Voice and Airway Symptoms

• Persistent hoarseness

• Weak or breathy voice

• Vocal fatigue

• Difficulty speaking loudly or shouting

• Coughing or choking while eating (in severe cases)

Cardiopulmonary Symptoms

• Shortness of breath

• Fatigue or decreased exercise tolerance

• Orthopnea or paroxysmal nocturnal dyspnea (in heart failure)

• Palpitations or chest discomfort (if related to aneurysmal disease)

Diagnosis

Diagnosis of Ortner’s syndrome involves confirming vocal cord paralysis and identifying the cardiovascular etiology responsible for RLN impairment. A multidisciplinary approach, often involving ENT specialists, cardiologists, and radiologists, is required.

Clinical Evaluation

• History of hoarseness with or without cardiac symptoms

• Physical exam findings may include signs of heart failure or murmurs (e.g., from mitral stenosis)

ENT and Voice Assessment

• Flexible laryngoscopy: Reveals left vocal cord paralysis in the paramedian position

• Stroboscopy: May be used to assess vocal fold vibration and movement

Imaging Studies

• Chest X-ray: May show cardiomegaly or aortic dilation

• Echocardiography: To evaluate for mitral valve disease, left atrial size, or pulmonary hypertension

• CT or MRI of the chest: To identify thoracic aortic aneurysm, mediastinal mass, or vascular anomalies compressing the RLN

• Cardiac catheterization or CT angiography: In cases with suspected vascular pathology

Treatment

Treatment of Ortner’s syndrome focuses on addressing the underlying cardiovascular condition and managing the voice symptoms resulting from RLN paralysis.

Treatment of Underlying Cause

• Mitral valve repair or replacement: In patients with mitral stenosis or regurgitation

• Management of pulmonary hypertension: Using vasodilators, oxygen therapy, and diuretics

• Surgical repair of aortic aneurysm: If aneurysm is large or symptomatic

• Intervention for PDA or mediastinal tumors: As indicated by the cause

Voice Therapy and Airway Management

• Speech-language therapy: To improve vocal quality and strengthen the unaffected vocal cord

• Medialization procedures: Such as vocal cord injection or thyroplasty in patients with significant voice impairment

• Tracheostomy: Rarely needed, unless there is bilateral vocal cord paralysis or airway compromise

Prognosis

The prognosis of Ortner’s syndrome depends largely on the severity of the underlying cardiovascular disorder and the timeliness of diagnosis and treatment. In cases where the causative lesion is treatable or reversible, improvement in vocal cord function may occur, especially if nerve compression is relieved early.

However, prolonged nerve compression may result in irreversible nerve damage, necessitating permanent voice therapy or surgical intervention. Overall, with appropriate multidisciplinary management, many patients can achieve symptom relief and stabilization of cardiac and vocal function.