Association between Mitral Valve Pathology and Ventricular Ectopy in the Pediatric Marfan Population

Adult studies establish an association between mitral valve pathology, namely mitral annular disjunction (MAD) and mitral valve prolapse (MVP), and ventricular arrhythmias. Data in the pediatric Marfan population is limited. To assess the association between (1) MAD and ventricular ectopy (VE), non-sustained ventricular tachycardia (NSVT) and ventricular tachycardia (VT); (2) MVP and VE, NSVT and VT and (3) MAD and MVP in the pediatric Marfan population. We carried out a retrospective single center study from January 2001 to January 2022 including all patients with Marfan syndrome who were ≤ 21 years of age and had a cardiac rhythm monitor. Of the 32 patients included, 12 (38%) were female and 21 (66%) had a positive Fibrillin 1 variant. The mean age at echocardiogram was 13.5 ± 4.5 years and median duration of cardiac monitoring was 58 (32.5–190.5) hours. Sixteen (50%) had complex VE (couplets, triplets, and/or NSVT). Fourteen (44%) had couplets with median episodes per monitor of 2 (1–4), 1 (3%) being polymorphic and 6 (19%) with fast RR (R-R interval < 350 ms). Six (19%) had triplets with median episodes per monitor of 1 (1–1) and fast RR in 4 (13%). Four (13%) had NSVT. There is a high prevalence of complex VE in the pediatric Marfan population. MAD and MVP were not associated with complex VE however, all patients with triplets and NSVT had MVP, mostly bileaflet. MAD is positively associated with bileaflet MVP and bileaflet MVP is associated with more ventricular ectopy.