Related Experiment Video
Updated: May 14, 2026

Echocardiographic Assessment Using Subxiphoid-Only Examination for Hypotensive Patients
Published on: April 18, 2025
Spontaneous intracranial hypotension in patients with bicuspid aortic valve
Wouter I Schievink1, Sharo S Raissi
1Department of Neurosurgery, Cedars-Sinai Medical Center, Los Angeles, California, USA. schievinkw@cshs.org
Insights
Patients with bicuspid aortic valve (BAV), a connective tissue disorder, may have a higher risk of spontaneous intracranial hypotension. This condition can cause headaches and is often misdiagnosed.
Area of Science:
- Neurology
- Cardiology
- Genetics
Background:
- Spontaneous intracranial hypotension (SIH) is a headache cause often linked to connective tissue disorders.
- Bicuspid aortic valve (BAV) is a common connective tissue disorder associated with various headaches.
Observation:
- The study investigated the prevalence of BAV in SIH patients.
- A control group of patients with non-SIH headaches was also analyzed.
Findings:
- Bicuspid aortic valve (BAV) was found in 1.1% of SIH patients (3 of 273).
- No BAV cases were identified in the control group of 506 patients.
Implications:
- BAV may indicate an increased risk for developing SIH.
- A shared neural crest cell origin for aortic valve and spinal dura may explain the association between BAV and SIH.
Background And Aim Of The Study:
Spontaneous intracranial hypotension, an important but frequently misdiagnosed cause of headache, is often associated with the presence of an underlying systemic connective tissue disorder. Bicuspid aortic valve (BAV) is a relatively common systemic connective tissue disorder that has been associated with other causes of headache, such as intracranial aneurysm and cervico-cephalic arterial dissection. The study aim was to assess the frequency of BAV among patients with spontaneous intracranial hypotension.
Methods:
The medical records of a consecutive group of patients with spontaneous intracranial hypotension were reviewed for the presence of BAV. The control population consisted of a group of patients evaluated for face pain or headache not due to spontaneous intracranial hypotension.
Results:
The presence of a BAV was confirmed in three of 273 patients (1.1%) with spontaneous intracranial hypotension, but in none of 506 controls (p = 0.04).
Conclusion:
Patients with BAV may be at an increased risk of spontaneous intracranial hypotension. The aortic valvular cusps, as well as the spinal dura, are derived from cells that originate in the neural crest; hence, a disorder of neural crest cell migration could explain the findings of BAV and spontaneous intracranial hypotension in the presently reported patients.
More Related Videos
Related Concept Videos
Measurement of Blood Pressure
Increased Intracranial Pressure l: Introduction
Aortic Regurgitation II: Clinical Features and Diagnostic Tests
Alterations in Blood Pressure
Hypertension (High blood pressure)
Hypertension occurs when blood pressure readings consistently exceed the normal range. It is diagnosed when systolic blood pressure (the top number, indicating pressure while the heart beats)...
Aortic Regurgitation I: Introduction
Increased Intracranial Pressure ll: Pathophysiology
