BS05. Orthostatic hypotension and syncope in patients with ehlers-danlos syndrome

Abstract

Introduction Postural tachycardia Syndrome (POTS) is a form of chronic orthostatic intolerance where the hallmark physiological trait is an excessive increase in heart rate upon standing. POTS is a final common pathway for a number of overlapping conditions, including lower body autonomic neuropathy, elevated sympathetic tone, mast cell activation, deconditioning, the presence of certain antibodies and hypovolemia. It also has similarities to chronic fatigue syndrome, Ehlers-Danlos Syndrome (EDS), vasovagal syncope and inappropriate sinus tachycardia. EDS is an heterogeneous disorder associated with inherited abnormalities of collagen structure. Patients develop hyper-extensibility, joint hypermobility and fragile connective tissue. Patients with EDS type III frequently have symptoms of autonomic dysregulation such as palpitations, lightheadedness, chest pain, presyncope and syncope that are also common in patients with POTS. Connective tissue abnormalities in EDS could lead to vascular laxity, predipose patients to orthostatic blood pooling in the lower extremities and orthostatic intolerance. These patients might have a peripheral neuropahty that could contribute to autonomic impairment. Methods Clinical records of patients diagnosed with Ehlers Danlos treated at Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran were reviewed retrospectively during a 10 year period (1987–2017). Results 29 patients were found to have a diagnosis of EDS. The female-to-male ratio was 3:1, while the mean age was 29. One patient died due to a ruptured aortic aneurysm. 9 (34.6%) patients had a history of presyncope, 14 patients (53%) had low blood pressure and 12 (46.15%) patients had heart rate values above 80 beats per minute. Mean systolic blood pressure was 107.2 ± 16, diastolic blood pressure 67.3 mmHg ± 10.8 with a mean heart rate of 80.1 ± 11.8. The most common Ehlers Danlos phenotype found was type III, which ocurred in 5 patients (40%). Type IV was found in 5 patients (26%), 3 patients (20%) presented with type VI and only two patients with type VII and type IX, respectively. We could not have the type of 14 patients. Conclusion Although is known that patients with the EDS may present with syncope or presyncopal episodes it is not known the percentage of patients who have dysautonomic cardiovascular symptoms and signs. We found that 53% of the patients have orthostatic intolerance, 34% had syncopal episodes and 46% could be diagnosed with POTS. One patient died due to a thoracic aortic aneurysm.