Frequency of Ventricular Arrhythmias and Other Rhythm Abnormalities in Children and Young Adults With the Marfan Syndrome

Douglas Y. Mah, Lynn A. Sleeper, Jane Crosson, Richard J. Czosek, Barry A. Love, Brian W. McCrindle, Laura Muiño-Mosquera, Aaron K. Olson, Thomas A. Pilcher, Elif Seda Selamet Tierney, Maully J. Shah, Stephanie B. Wechsler, Luciana T. Young, Ronald V. Lacro

Research output: Contribution to journalArticle

Abstract

Patients with the Marfan syndrome (MFS) are at risk for sudden death. The contribution of arrhythmias is unclear. This study examines the prevalence of arrhythmias in children with the MFS and their relation to clinical and/or echocardiographic factors. Data from the Pediatric Heart Network randomized trial of atenolol versus losartan in MFS were analyzed (6 months to 25 years old, aortic root diameter z-score > 3.0, no previous aortic surgery and/or dissection). Baseline 24-hour ambulatory electrocardiographic monitoring was performed. Significant ventricular ectopy (VE) and supraventricular ectopy (SVE) were defined as ≥10 VE or SVE/hour, or the presence of high-grade ectopy. Three-year composite clinical outcome of death, aortic dissection, or aortic root replacement was analyzed. There were 274 analyzable monitors on unique patients from 11 centers. Twenty subjects (7%) had significant VE, 13 (5%) significant SVE; of these, 2 (1%) had both. None had sustained ventricular or supraventricular tachycardia. VE was independently associated with increasing number of major Ghent criteria (odds ratio [OR] = 2.13/each additional criterion, p = 0.03) and greater left ventricular end-diastolic dimension z-score (OR = 1.47/each 1 unit increase in z-score, p = 0.01). SVE was independently associated with greater aortic sinotubular junction diameter z-score (OR = 1.56/each 1 unit increase in z-score, p = 0.03). The composite clinical outcome (14 events) was not related to VE or SVE (p ≥ 0.3), but was independently related to heart rate variability (higher triangular index). In conclusion, in this cohort, VE and SVE were rare. VE was related to larger BSA-adjusted left ventricular size. Routine ambulatory electrocardiographic monitoring may be useful for risk stratification in select MFS patients.

Original languageEnglish (US)
JournalAmerican Journal of Cardiology
DOIs
StateAccepted/In press - Jan 1 2018

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Marfan Syndrome
Cardiac Arrhythmias
Young Adult
Ambulatory Electrocardiography
Odds Ratio
Dissection
Supraventricular Tachycardia
Atenolol
Losartan
Ventricular Tachycardia
Sudden Death
Cross-Sectional Studies
Heart Rate
Pediatrics

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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Frequency of Ventricular Arrhythmias and Other Rhythm Abnormalities in Children and Young Adults With the Marfan Syndrome. / Mah, Douglas Y.; Sleeper, Lynn A.; Crosson, Jane; Czosek, Richard J.; Love, Barry A.; McCrindle, Brian W.; Muiño-Mosquera, Laura; Olson, Aaron K.; Pilcher, Thomas A.; Tierney, Elif Seda Selamet; Shah, Maully J.; Wechsler, Stephanie B.; Young, Luciana T.; Lacro, Ronald V.

In: American Journal of Cardiology, 01.01.2018.

Research output: Contribution to journalArticle

Mah, DY, Sleeper, LA, Crosson, J, Czosek, RJ, Love, BA, McCrindle, BW, Muiño-Mosquera, L, Olson, AK, Pilcher, TA, Tierney, ESS, Shah, MJ, Wechsler, SB, Young, LT & Lacro, RV 2018, 'Frequency of Ventricular Arrhythmias and Other Rhythm Abnormalities in Children and Young Adults With the Marfan Syndrome', American Journal of Cardiology. https://doi.org/10.1016/j.amjcard.2018.07.006
Mah, Douglas Y. ; Sleeper, Lynn A. ; Crosson, Jane ; Czosek, Richard J. ; Love, Barry A. ; McCrindle, Brian W. ; Muiño-Mosquera, Laura ; Olson, Aaron K. ; Pilcher, Thomas A. ; Tierney, Elif Seda Selamet ; Shah, Maully J. ; Wechsler, Stephanie B. ; Young, Luciana T. ; Lacro, Ronald V. / Frequency of Ventricular Arrhythmias and Other Rhythm Abnormalities in Children and Young Adults With the Marfan Syndrome. In: American Journal of Cardiology. 2018.
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AU - Crosson, Jane

AU - Czosek, Richard J.

AU - Love, Barry A.

AU - McCrindle, Brian W.

AU - Muiño-Mosquera, Laura

AU - Olson, Aaron K.

AU - Pilcher, Thomas A.

AU - Tierney, Elif Seda Selamet

AU - Shah, Maully J.

AU - Wechsler, Stephanie B.

AU - Young, Luciana T.

AU - Lacro, Ronald V.

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