Ortner syndrome - Symptoms, Causes, Treatment & Prevention

```html

Overview

Ortner syndrome, also called cardiovocal syndrome, is a rare form of left recurrent laryngeal nerve (RLN) palsy that results from cardiovascular disease. The nerve is compressed or irritated by enlarged cardiac structures, abnormal aortic arches, pulmonary hypertension, or congenital heart defects, leading to hoarseness or loss of voice.

Although the exact prevalence is unknown because it is often mis‑diagnosed as an isolated vocal‑cord problem, case series suggest it accounts for < 1 % of all adult vocal‑cord pareses. It can affect both men and women, most commonly adults over 40 years, but has been reported in children with congenital heart disease.

Key points:

• First described by Norwegian thoracic surgeon Harold Ortner in 1897.
• It is a secondary (i.e., “compressive”) nerve palsy, not a primary laryngeal disorder.
• Underlying heart or great‑vessel disease is the driver; treat the cardiovascular problem to improve voice.

Symptoms

The clinical picture reflects dysfunction of the left RLN, which supplies motor innervation to all intrinsic laryngeal muscles except the cricothyroid. Common symptoms include:

• Hoarseness or breathy voice – the most frequent presenting complaint; may be gradual.
• Voice fatigue – the voice worsens after prolonged talking.
• Reduced vocal pitch range – especially loss of lower pitches.
• Difficulty swallowing (dysphagia) – occurs when the vocal cord cannot close fully, allowing food to enter the airway.
• Coughing or choking episodes – especially with liquids.
• Throat irritation or sensation of a “lump” (globus).
• Stridor or noisy breathing – rare, only when the paralysis is severe.
• Chest discomfort or dyspnea – often present because the underlying cardiovascular disease (e.g., pulmonary hypertension) produces its own respiratory symptoms.
• Facial flushing or syncope – if severe aortic compression compromises blood flow.

Symptoms may be unilateral (most common) because only the left RLN loops under the aortic arch. In rare bilateral cases, voice loss can be profound and may require urgent airway protection.

Causes and Risk Factors

Ortner syndrome is a mechanical or ischemic injury to the left RLN secondary to cardiovascular pathology. The most common etiologies are:

Cardiovascular Causes

• Left atrial enlargement – often from chronic mitral stenosis or severe mitral regurgitation.
• Aortic arch aneurysm or dissection – expands the arch and compresses the nerve.
• Pulmonary hypertension – enlarges the pulmonary artery, which lies near the nerve.
• Congenital heart defects – e.g., patent ductus arteriosus, ventricular septal defect with left‑to‑right shunt.
• Severe left‑sided heart failure – causes cardiac silhouettes to press against the trachea/esophagus.
• Thoracic aortic coarctation – can distort the arch.

Non‑cardiovascular Causes (less common)

• Thoracic tumors (e.g., lung carcinoma, mediastinal mass) that directly invade or compress the nerve.
• Post‑surgical injury after cardiac or aortic surgery.
• Trauma to the mediastinum.

Risk Factors

• Age > 40 years (degenerative valvular disease becomes more common).
• History of rheumatic fever leading to mitral stenosis.
• Hypertension, diabetes, or hyperlipidemia that accelerate atherosclerotic aortic disease.
• Congenital heart disease that persists into adulthood.
• Chronic smoking – increases risk of aortic aneurysm and lung tumors that can mimic Ortner.

Diagnosis

Because hoarseness is a common complaint, the diagnosis of Ortner syndrome relies on a high index of suspicion and a systematic work‑up to identify the cardiovascular source.

Step‑by‑step Diagnostic Approach

1. History and Physical Examination – Document voice changes, associated cardiac or respiratory symptoms, and risk factors.
2. Laryngoscopic Evaluation – Flexible or rigid laryngoscopy visualizes unilateral vocal‑cord paralysis on the left side. It rules out primary laryngeal lesions.
3. Imaging of the Thorax:
   • Chest X‑ray* – May reveal cardiomegaly, aortic knob enlargement, or pulmonary artery dilation.*
   • Contrast‑enhanced CT or MRI of the neck/chest* – Gold standard for detecting compression of the left RLN by vascular structures.*
4. Echocardiography – Transthoracic (TTE) or transesophageal (TEE) echo assesses valve disease, atrial size, and pulmonary pressures.
5. Cardiac Catheterization or MRI Angiography – Used when an aortic aneurysm or dissection is suspected.
6. Pulmonary Function Tests (PFTs) – Helpful if dyspnea is prominent, to differentiate airway obstruction from nerve palsy.

Diagnostic criteria (adapted from Mayo Clinic & Cleveland Clinic guidelines) require:

• Documented left‑sided RLN palsy, and
• Radiologic evidence of a cardiovascular abnormality adjacent to the nerve that can plausibly cause compression.

Treatment Options

Treatment is two‑pronged: address the underlying cardiovascular pathology and manage the vocal‑cord dysfunction.

1. Treating the Cardiovascular Cause

• Medical management – For pulmonary hypertension: endothelin receptor antagonists, phosphodiesterase‑5 inhibitors, or prostacyclin analogues (e.g., bosentan, sildenafil) as recommended by the WHO Pulmonary Hypertension Guidelines.
• Valve repair/replacement – Mitral stenosis or severe regurgitation often require surgical or percutaneous (e.g., MitraClip) intervention.
• Aortic repair – Endovascular stent‑graft placement for aneurysm/dissection; open surgical repair when anatomy dictates.
• Congenital defect closure – Device closure of PDA or VSD reduces left‑sided pressure overload.
• Heart failure optimization – ACE inhibitors, beta‑blockers, diuretics, and lifestyle changes can shrink cardiac chambers over time.

2. Voice and Laryngeal Management

• Voice therapy – Conducted by a speech‑language pathologist; exercises improve breath support and compensatory phonation.
• Medialization procedures – Injection laryngoplasty (e.g., hyaluronic acid, calcium hydroxylapatite) or arytenoid adduction to improve vocal‑cord closure.
• Reinnervation surgery – Ansa cervicalis–RLN anastomosis can restore motor function, especially when the underlying cardiac disease is cured.
• Temporary tracheostomy – Rare, reserved for bilateral paralysis with airway compromise.

3. Lifestyle and Supportive Measures

• Hydration and humidified air to keep the vocal folds moist.
• Avoidance of vocal strain (e.g., shouting, prolonged phone use).
• Smoking cessation – essential for both cardiovascular health and vocal‑cord healing.

Living with Ortner Syndrome

Even after successful cardiac treatment, the nerve may take months to recover, and some patients retain a permanent vocal‑cord deficit. Practical strategies include:

• Regular follow‑up with cardiology and ENT – Allows monitoring of both heart status and voice progression.
• Speech‑therapy schedule – 1–2 sessions per week for the first 3 months, then as needed.
• Voice‑preserving habits:
  1. Warm‑up your voice before long conversations.
  2. Speak from the diaphragm rather than the throat.
  3. Use a microphone or amplification devices when speaking in groups.
• Nutrition – Soft‑food diet while dysphagia improves; avoid dry, crunchy foods that provoke coughing.
• Hydration – Aim for at least 8 cups of water daily; consider electrolyte‑balanced drinks if on diuretics.
• Stress management – Anxiety can worsen voice strain; practice breathing exercises, yoga, or mindfulness.

Prevention

Because Ortner syndrome is secondary to cardiac disease, primary prevention targets the cardiovascular system:

• Control blood pressure, cholesterol, and blood glucose within guideline‑recommended ranges.
• Maintain a heart‑healthy diet rich in fruits, vegetables, whole grains, and lean proteins (e.g., Mediterranean diet).
• Exercise regularly – at least 150 minutes of moderate aerobic activity per week.
• Quit smoking and limit alcohol intake.
• Promptly treat rheumatic fever to avoid mitral stenosis.
• Screen for and repair congenital heart defects early in life.
• Annual cardiac check‑ups for individuals with known valvular disease or aortic abnormalities.

Complications

If left untreated, Ortner syndrome can lead to:

• Persistent dysphonia – Social isolation, occupational limitations, and reduced quality of life.
• aspiration pneumonia – Due to compromised airway protection during swallowing.
• Airway obstruction – Rare but possible in bilateral RLN paralysis.
• Progressive cardiac disease – The underlying cardiovascular pathology may deteriorate without appropriate management.
• Psychological effects – Depression and anxiety secondary to voice loss.

When to Seek Emergency Care

---

Sources: Mayo Clinic. “Recurrent Laryngeal Nerve Paralysis.”; Cleveland Clinic. “Ortner’s Cardiovocal Syndrome.”; American Heart Association. “Guidelines for the Management of Valvular Heart Disease.”; WHO. “Pulmonary Hypertension Fact Sheet.”; National Institutes of Health. “Aortic Aneurysm and Dissection.”; Journal of Voice. 2022;36(4):555‑564.

```