The Prevalence of Cardiac Symptoms in Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorder: A Pilot Study

OBJECTIVES: The authors compared palpitations, presyncope, syncope, and postural orthostatic tachycardia syndrome (POTS) prevalence among patients with hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorder (HSD). METHODS: A retrospective analysis of patients referred for cardiac evaluation at the New York Institute of Technology Ehlers-Danlos Syndrome/Hypermobility Treatment Center between January 2019 and November 2023 was conducted. hEDS and HSD patients underwent cardiac evaluation and were stratified by median age into younger (<35 years) and older (≥35 years) groups. Symptom prevalence was compared between hypermobile patients overall and by age cohort. Data were presented as mean ± SD; P < .05 was considered statistically significant. RESULTS: One hundred-nine patients (75 hEDS, 34 HSD) underwent cardiac evaluation: 75 hEDS (71 females/4 males; age 33.9 + 11.6 years) and 34 HSD (29 females/ 5 males; age 40.0 + 13.8 years). POTS (48% vs 23.5%, P = .016) was significantly more prevalent in the hEDS group; palpitations, presyncope, and syncope were common in both groups. No significant differences were observed in Cohort 1 (age less than 35 years). In Cohort 2 (age ≥35 years), presyncope (100% vs 82.6%, P = .04) differed; palpitations, syncope, and POTS were similar. POTS was prevalent in patients younger than 35 years with hEDS (58.3% vs 29.6%, P = .022) and HSD (45.5% vs 13.0%, P = .042). CONCLUSIONS: Patients with h EDS exhibited greater symptoms than HSD patients, and symptoms were particularly more pronounced with age. POTS prevalence decreased with age in both groups. Larger prospective studies are needed to explore underlying mechanisms.