Author + information
- Douglas Mah,
- Lynn Sleeper,
- Jane Crosson,
- Richard Czosek,
- David Gamboa,
- Barry Love,
- Brian McCrindle,
- Laura Muino Mosquero,
- Aaron Olson,
- Maully Shah,
- Stephanie Wechsler,
- Luciana Young and
- Ronald Lacro
Background: Individuals with Marfan syndrome (MFS) are at risk for sudden death. The contribution of arrhythmias is unclear. Our aim was to determine the prevalence of arrhythmias in MFS subjects, and their relationship to clinical and echocardiographic factors.
Methods: Data from the Pediatric Heart Network (PHN) prospective multicenter randomized trial of atenolol vs. losartan in subjects with MFS were analyzed. The 608 randomized subjects were 6 months-25 years old with aortic root diameter z-score (ARz) >3, and no prior AR surgery or dissection. Baseline 24-hour Holter monitors were performed prior to study drug initiation. Significant ventricular ectopy (VE) and supraventricular ectopy (SVE) were defined as ≥10 VE or SVE/hour, or the presence of high-grade ectopy: couplets, triplets, non-sustained ventricular (VT) or supraventricular tachycardia (SVT). The clinical outcome analyzed was the composite of death, aortic dissection, or AR replacement. Age-adjusted multivariable analysis was performed.
Results: There were 274 analyzable Holters from 11 PHN centers. Mean±SD age was 11±6 years, similar to the 334 subjects with unavailable Holters. 20 subjects (7%) had significant VE, 13 (5%) had significant SVE, 2 (1%) had both. None had sustained VT or SVT. VE was independently associated with increasing number of major Ghent criteria (odds ratio [OR] 2.1 per each additional criterion met, p=0.03) and greater left ventricular end-diastolic dimension z-score (OR=1.5, p=0.01). SVE was independently associated with greater aortic sinotubular junction diameter z-score (OR=1.6, p=0.03). Palpitations were more common when SVE was present vs absent (46% vs. 14%, p=0.006) but not VE (p=0.7). Syncope was not associated with either. Over 3 years, there were no deaths, 2 AR dissections with surgeries, and 13 with AR surgery only. These events were not related to VE or SVE (p≥0.3).
Conclusions: This is the largest study evaluating the prevalence of arrhythmia in children and young adults with MFS. Overall, VE and SVE were rare, with none having VT or SVT. VE is related to larger BSA-adjusted left ventricular size and a greater number of major Ghent criteria. Routine Holters are useful only in select MFS patients.
Poster Hall, Hall C
Sunday, March 19, 2017, 9:45 a.m.-10:30 a.m.
Session Title: Congenital Heart Disease: Novel Insights in Pediatric Cardiology
Abstract Category: 10. Congenital Heart Disease: Pediatric
Presentation Number: 1271-017
- 2017 American College of Cardiology Foundation