Does a patient with Ehlers-Danlos syndrome (EDS) need an Echocardiogram (ECHO) in the Emergency Room (ER)?

Echocardiography in Patients with Ehlers-Danlos Syndrome in the Emergency Room

Patients with Ehlers-Danlos syndrome (EDS) do not routinely need an echocardiogram in the Emergency Room unless they present with specific cardiovascular symptoms or hemodynamic instability.

Indications for Emergency Echocardiography in EDS Patients

Emergency echocardiography should be performed in EDS patients in the following situations:

Recommended:

• Patients presenting with:
  • Chest pain with suspected aortic dissection or cardiac involvement 1, 2
  • Hemodynamic instability (hypotension, tachycardia) 1
  • Signs of heart failure 1
  • Abnormal cardiac auscultation findings 1
  • Abnormal ECG tracings or recurrent arrhythmias 1
  • Trauma with suspected cardiac involvement 1

Not Recommended:

• Routine screening in asymptomatic EDS patients 1
• EDS patients with minor chest complaints without concerning symptoms 1
• Stable patients with normal vital signs and no cardiac symptoms 1

Cardiovascular Manifestations in EDS

The prevalence of cardiovascular abnormalities in EDS varies by subtype:

• Vascular EDS (Type IV): Highest risk for serious vascular complications

  • Arterial dissection and rupture
  • Aortic root dilation
  • Annual imaging of the entire aorta is recommended 1

• Hypermobile EDS and Classical EDS:

  • Mitral valve prolapse: 6-15% of patients 3, 4, 2
  • Aortic root dilation: 15-20% of patients 5, 2
  • Tricuspid valve prolapse: Less common 4
  • Extra-aortic arterial involvement (rare): Cervical artery dissection, spontaneous coronary artery dissection 2

Decision Algorithm for Emergency Echocardiography in EDS

1. Emergent Echocardiography (perform immediately):

   • Suspected aortic dissection or rupture
   • Hemodynamic instability with tachycardia or hypotension
   • Chest trauma with cardiac involvement
   • Suspected cardiac tamponade

2. Urgent Echocardiography (perform during ER visit):

   • Chest pain with abnormal ECG or cardiac biomarkers
   • New heart murmur or change in previously documented murmur
   • Signs of heart failure
   • Unexplained persistent arrhythmias

3. Deferred Echocardiography (schedule as outpatient):

   • Stable EDS patient without acute cardiac symptoms
   • Routine surveillance in known EDS
   • Minor chest complaints with normal vital signs

Type of Echocardiography

• Transthoracic echocardiography (TTE) is the first-line imaging modality in most cases
• Transesophageal echocardiography (TEE) may be necessary if:
  • TTE is non-diagnostic 1
  • Suspected aortic dissection requires better visualization 1
  • Suspected endocarditis with negative TTE 6

Important Caveats

• Earlier studies may have overestimated the prevalence of cardiac abnormalities in EDS 3
• More recent evidence suggests cardiovascular manifestations in EDS are typically mild, especially in females 2
• Cardiac symptoms (atypical chest pain, palpitations, dyspnea) are more common in EDS patients than actual structural abnormalities 3
• The decision for emergency echocardiography should be based on clinical presentation rather than the EDS diagnosis alone

Conclusion

While EDS patients have an increased risk of certain cardiovascular abnormalities, emergency echocardiography should be reserved for those presenting with concerning cardiac symptoms, hemodynamic instability, or signs of acute cardiac pathology. Asymptomatic EDS patients or those with minor complaints do not require emergency echocardiography and can have this imaging scheduled on an outpatient basis.