Evidence map›Paper›PMID 40100519›Full record

ArticleQuality of life research : an international journal of quality of life aspects of treatment, care and rehabilitation2025

Family functioning and health-related quality of life in children and young adults with Marfan syndrome.

Lauren S Crafts, Lynn A Sleeper, Karen Uzark, Andrew M Atz, Timothy J Bradley, Elizabeth Cappella, Veronica Fettig, Georgeann Keh Teng Groh, Michelle S Hamstra, Christina Hartje-Dunn and 9 more

Abstract readMulticenter Study
PubMed Publisher
In one paragraph

Article in Quality of life research : an international journal of quality of life aspects of treatment, care and rehabilitation, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

What it found

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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

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3 · Its place in the literature

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0 citing papers in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

19 authors.

Lauren S CraftsDepartment of Cardiology, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA. lauren.crafts@cardio.chboston.org.ORCID http://orcid.org/0000-0002-4197-6718
Lynn A SleeperDepartment of Cardiology, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
Karen UzarkC.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI, USA.
Andrew M AtzMedical University of South Carolina, Charleston, SC, USA.
Timothy J BradleyUniversity of Saskatchewan, Saskatoon, SK, Canada.
Elizabeth CappellaAnn and Robert H. Lurie Children's Hospital, Chicago, IL, USA.
Veronica FettigIcahn School of Medicine at Mount Sinai, Mount Sinai Hospital, New York, NY, USA.
Georgeann Keh Teng GrohWashington University School of Medicine, St. Louis, MO, USA.
Michelle S HamstraThe Christ Hospital Health Network, Heart and Vascular Institute, Cincinnati, OH, USA.
Christina Hartje-DunnSeattle Children's Hospital, Seattle, WA, USA.
Jami C LevineDepartment of Cardiology, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
Ian LindsayThe University of Utah, Salt Lake City, UT, USA.
Aaron K OlsonSeattle Children's Hospital, Seattle, WA, USA.
Gretchen MacCarrickJohns Hopkins University School of Medicine, Baltimore, MD, USA.
Larry W MarkhamRiley Hospital at Indiana University Health, Indiana University School of Medicine, Indianapolis, IN, USA.
Tonia MorrisonChildren's Hospital of Philadelphia, Philadelphia, PA, USA.
Reed E PyeritzPerelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Seda TierneyLucile Packard Children's Hospital Stanford, Stanford University School of Medicine, Palo Alto, CA, USA.
Ronald V LacroDepartment of Cardiology, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.

Funding

Marfan Foundation Marfan Foundation
6 · The paper itself

Abstract

purposeEvaluate family functioning (FF) and associations with quality of life (QOL) in a large, multicenter cohort of children and young adults with Marfan syndrome (MFS) who participated in the Pediatric Heart Network (PHN) Marfan Trial.

methodsOf the 608 patients enrolled in the PHN Marfan Trial, 359 families completed one or more of the following: the General Functioning subscale of the Family Assessment Device (FAD), the Condition Management Ability module of the Family Management Measure (FaMM), and the Pediatric Quality of Life Inventory (PedsQL) at the final trial visit at three years. The correlations between FAD and FaMM scores and patient-related factors were examined. Linear regression was used to determine the relationship between FAD, FaMM, patient-related factors, and PedsQL.

results25% of families by self-report and 9% of families by parent-report had impaired functioning (FAD score > 2). FAD and FaMM were inversely correlated (R=-0.41, p < 0.001). Higher (worse) FAD scores were correlated with more patient-reported symptoms (R = 0.17, p = 0.003) and with lower QOL (R=-0.28, p < 0.001), independent of other factors typically related to QOL.

conclusionsIn patients with MFS, family dysfunction and more symptoms were independently associated with worse QOL. This suggests that interventions addressing family dysfunction and symptoms may positively impact QOL for patients and families living with MFS.

Indexed as

Family RelationsMarfan SyndromeQuality of LifeAdolescentAdultChildChild, PreschoolFemaleHumansMaleSurveys and QuestionnairesYoung Adult

Identifiers

PMID40100519

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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.