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Hyperventilation during rest and exercise in orthostatic intolerance and Spiky-Leaky Syndrome
Amir Hashemizad1, Jerriel Dela Cruz1, Aditya Narayan1
1Heart of the Valley Pediatric Cardiology, Pleasanton, CA, United States.
Frontiers in Neurology
|May 2, 2025
Summary
Patients with Spiky-Leaky Syndrome (SLS) and orthostatic intolerance dysautonomia syndromes (OIDS) show reduced exercise capacity and lower end-tidal CO2 levels. These metabolic changes may indicate sympathetic hyperactivity and reduced ventricular preload in OIDS and SLS.
Area of Science:
- Cardiology
- Neurology
- Pulmonology
Background:
- Orthostatic intolerance dysautonomia syndromes (OIDS), including postural orthostatic tachycardia syndrome (POTS), are linked to sympathetic hyperactivity and daytime hyperventilation.
- A subset of OIDS patients, termed Spiky-Leaky Syndrome (SLS), exhibit craniocervical instability, mast cell activation syndrome, hypermobility spectrum disorder, and cerebrospinal fluid leaks.
- Daytime hyperventilation in OIDS and SLS is hypothesized to stem from sympathetic hyperactivity.
Purpose of the Study:
- To compare metabolic and exercise parameters in young patients with SLS, OIDS, and healthy controls.
- To identify potential exercise-based markers for POTS physiology and sympathetic hyperactivity in OIDS and SLS.
Main Methods:
- Retrospective review of clinical metabolic exercise data from 323 individuals (44 SLS, 210 OIDS, 53 controls).
- Assessment of metabolic parameters at rest, anaerobic threshold (AT), and maximal oxygen consumption (VO2max).
- Measured parameters included end-tidal CO2 (ETCO2), end-tidal O2 (ETO2), peak oxygen pulse, total work performed, and oxygen uptake efficiency slope (OUESp).
Main Results:
- VO2max, AT, peak oxygen pulse, total work, and OUESp were significantly reduced in OIDS and further diminished in SLS compared to controls.
- End-tidal CO2 (ETCO2) levels were significantly lower at rest, AT, and VO2max in OIDS and SLS patients, persisting throughout exercise.
- End-tidal O2 (ETO2) showed a reciprocal, increasing trend in OIDS and SLS patients compared to controls.
Conclusions:
- Patients with OIDS and particularly SLS demonstrate reduced metabolic exercise parameters, including decreased peak oxygen pulse and ETCO2.
- These findings suggest reduced ventricular preload and chronic hyperventilation in OIDS and SLS.
- Exercise parameters like decreased ETCO2 may serve as indicators of POTS physiology and sympathetic hyperactivity in the pathophysiology of SLS.
Keywords:
Ehlers-Danlos syndromePOTSSpiky-Leaky Syndromedysautonomiaend-tidal CO2exercise stress testinghypermobility spectrum disordermast cell activation syndromeMore Related Videos
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