Arrhythmias & electrophysiologyCohort study
Ventricular Arrhythmias in Marfan Syndrome: Prevalence and Clinical Correlates in a Multicenter Cohort
- retrospective multicenter cohort study
- 3 Mayo Clinic sites
- manual chart review
Authors' conclusion
In adults with Marfan syndrome, sustained VT/VF occurs in nearly 1 in 10 patients and is associated with a broader structural-electrical phenotype. Late gadolinium enhancement provided additional risk signal among patients undergoing cardiac magnetic resonance.
Study context
- Population
- Adults with confirmed Marfan syndromeadults with confirmed Marfan syndrome
- Participants
- 783 adults
- Exposure
- Associations of clinical, arrhythmic, echocardiographic, cardiac magnetic resonance and surgical features with sustained ventricular tachycardia (VT) or ventricular fibrillation (VF)
Main finding
As reported in the abstract
Sustained VT/VF occurrence · 12.7±7.6 years
Sustained VT/VF
74 patients (9.5%)
Observational data: associations, not effects.
Further findings
Independent associations with sustained VT/VF (primary multivariable model)
| Comparison | odds ratio (95% CI) | P value | |
|---|---|---|---|
| nonsustained VT · primary multivariable model in the full cohort | 12.11 (6.43–22.79) | P < 0.001 | |
| history of at least 1 cardiac surgery · primary multivariable model in the full cohort | 3.59 (1.65–7.84) | P = 0.001 | |
| moderate-severe mitral regurgitation · primary multivariable model in the full cohort | 2.00 (1.07–3.75) | P = 0.030 | |
| 0.050.21520Odds ratio (log scale) |
| Outcome | odds ratio (per 1% increase) (95% CI) | P value | |
|---|---|---|---|
| Independent associations with sustained VT/VF (primary multivariable model) — left ventricular ejection fraction · primary multivariable model in the full cohort | 0.95 (0.93–0.97) | P < 0.001 | |
| 0.512Odds ratio (log scale) |
- Independent associations with sustained VT/VF (primary multivariable model): lower left ventricular ejection fraction associated with sustained VT/VF
Univariable associations with sustained VT/VF
- Sustained VT/VF clustered with an adverse structural-electrical phenotype including atrial arrhythmias, ventricular remodeling, mitral valve disease, cardiac surgery history, and late gadolinium enhancement
- univariable logistic regression
Cardiac magnetic resonance sensitivity model
- Nonsustained VT, lower left ventricular ejection fraction, and late gadolinium enhancement remained associated with sustained VT/VF
- prespecified exploratory
Original publication
Ventricular Arrhythmias in Marfan Syndrome: Prevalence and Clinical Correlates in a Multicenter Cohort. Circ Arrhythm Electrophysiol. Published 2 October 2026. doi:10.1161/circep.126.014967
Summarised from the published abstract; every number is checked against it