Evidence map›Paper›PMID 39465402›Full record

ArticlePediatric cardiology2025

Mitral Annular Disjunction Associated with Ventricular Dilation in Pediatric Marfan Syndrome: A Cardiovascular Magnetic Resonance Study.

Ryan S Bishop, Tam T Doan, Natalie K Craik, Sara B Stephens, Alejandra Iturralde Chavez, Shagun Sachdeva, Justin D Weigand

Abstract read
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In one paragraph

Article in Pediatric cardiology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

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1citing papers in PubMed
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1 · What the graph read from it

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2 · The registry

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3 · Its place in the literature

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1 citing paper in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

7 authors.

Ryan S BishopDivision of Cardiology, Texas Children's Hospital, Baylor College of Medicine, 6651 Main Street MC-E1920, Houston, TX, 77030, USA. ryan.bishop@bcm.edu.ORCID http://orcid.org/0000-0003-3117-6044
Tam T DoanDivision of Cardiology, Texas Children's Hospital, Baylor College of Medicine, 6651 Main Street MC-E1920, Houston, TX, 77030, USA.
Natalie K CraikDivision of Cardiology, Texas Children's Hospital, Baylor College of Medicine, 6651 Main Street MC-E1920, Houston, TX, 77030, USA.
Sara B StephensDivision of Cardiology, Texas Children's Hospital, Baylor College of Medicine, 6651 Main Street MC-E1920, Houston, TX, 77030, USA.
Alejandra Iturralde ChavezDivision of Cardiology, Texas Children's Hospital, Baylor College of Medicine, 6651 Main Street MC-E1920, Houston, TX, 77030, USA.
Shagun SachdevaDivision of Cardiology, Texas Children's Hospital, Baylor College of Medicine, 6651 Main Street MC-E1920, Houston, TX, 77030, USA.
Justin D WeigandDivision of Cardiology, Texas Children's Hospital, Baylor College of Medicine, 6651 Main Street MC-E1920, Houston, TX, 77030, USA.

Funding

Evaluation of an Imaging Biomarker in Children and Young Adults with AortopathyK23HL127266 · NHLBI · BAYLOR COLLEGE OF MEDICINE · PI MORRIS, SHAINE ALAINE · 2015 to 2019
$761k
NHLBI NIH HHS K23HL127266
6 · The paper itself

Abstract

backgroundMitral annular disjunction (MAD) has increasingly been recognized as a marker for adverse cardiovascular events in Marfan syndrome (MFS). As recent adult data links MFS with left ventricular (LV) dilation and reduced ejection fraction (LVEF), we hypothesized that MAD may be associated with LV dilation in pediatric MFS patients.

methodsA retrospective analysis was performed among MFS patients < 19 years old at initial cardiac MRI (CMR). MAD and mitral valve prolapse (MVP) were assessed by CMR or most proximate echo. CMR-derived left ventricular end-diastolic (LVEDV) and end-systolic (LVESV) volumes were measured. Indexed volumes, absolute and indexed z-scores, and LVEF were calculated. The combined volume load from mitral and aortic regurgitation was indexed to LV stroke volume, allowing exclusion of patients with greater than mild volume load or prior MV intervention. MAD association with LV volumes and z-scores was then assessed.

resultsForty-two patients were analyzed (median age 13.5 years old, IQR [10.9, 15.3]). MAD was present in 28 patients (66.7%), and MVP was present in 13 patients (31.0%). Absolute LVEDV z-score was > 2 in 35.7% of patients, LVESV z-score was > 2 in 42.9%, and LVEF was < 55% in 45.2%. In multivariable analysis including MVP, MAD remained independently associated with elevated absolute LVESV z-score > 2 (RR 3.88, 95% CI 1.02-14.69, p = 0.046).

conclusionMAD was associated with CMR-derived volume-load-independent LV dilation among pediatric MFS patients. Prospective studies are needed to further understand this association and its relationship with LV dilation over time.

Indexed as

Heart VentriclesMagnetic Resonance Imaging, CineMarfan SyndromeMitral ValveMitral Valve ProlapseAdolescentChildFemaleHumansMaleRetrospective StudiesStroke VolumeCardiomyopathyMarfan syndromeMitral annular disjunctionMitral valve prolapse

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.