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

Saneeha Shahid1, Peter F Aziz1, Iqbal El Assaad1

  • 1Department of Pediatric Cardiology, Cleveland Clinic Children's, 9500 Euclid Avenue, Cleveland, OH, 44195, USA.

Pediatric Cardiology
|August 29, 2025
PubMed

Insights

Pediatric Marfan syndrome patients show high rates of ventricular ectopy. Mitral annular disjunction (MAD) and mitral valve prolapse (MVP) were not directly linked to complex ectopy, but MVP was present in all triplet and NSVT cases.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Genetics

Background:

  • Adult studies link mitral annular disjunction (MAD) and mitral valve prolapse (MVP) to ventricular arrhythmias.
  • Limited data exists on these associations in pediatric Marfan syndrome patients.
  • Marfan syndrome, a genetic disorder, affects connective tissue, including heart valves.

Purpose of the Study:

  • To assess the association between MAD and ventricular ectopy (VE), non-sustained ventricular tachycardia (NSVT), and ventricular tachycardia (VT) in pediatric Marfan patients.
  • To evaluate the association between MVP and VE, NSVT, and VT in this population.
  • To determine the co-occurrence of MAD and MVP in pediatric Marfan syndrome.

Main Methods:

  • Retrospective single-center study (January 2001 - January 2022).
  • Included pediatric patients (≤21 years) with Marfan syndrome and cardiac rhythm monitoring.
  • Analyzed data for MAD, MVP, VE, NSVT, and VT.

Main Results:

  • High prevalence of complex VE (50%) including couplets, triplets, and NSVT in pediatric Marfan patients.
  • MAD and MVP were not directly associated with complex VE.
  • All patients with triplets and NSVT had MVP (mostly bileaflet); MAD associated with bileaflet MVP, which correlated with increased VE.

Conclusions:

  • Pediatric Marfan syndrome patients exhibit a high prevalence of complex ventricular ectopy.
  • While not directly linked to complex arrhythmias, mitral valve abnormalities (MVP, MAD) are common and associated with increased ectopy.
  • Further investigation into the role of MVP and MAD in pediatric Marfan syndrome arrhythmias is warranted.
Keywords:
MarfanMitral annular disjunctionMitral valve prolapseVentricular ectopy

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