ArticlePediatric cardiology2019
Differences in Cardiovascular Manifestation of Marfan Syndrome Between Children and Adults.
Article in Pediatric cardiology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 34 citations in OpenAlex.
- Prognostic factors in patients with Marfan syndrome referred to a cardiac surgery center: a retrospective cohort study.BMC cardiovascular disorders · 2026Article
- Phenotypic Associations of Early Cardiovascular Surgery in Pediatric Marfan Syndrome.Children (Basel, Switzerland) · 2026Article
- Case Report: A rare coexistence with severe aortic root dilatation and nutcracker phenomenon in pediatric Marfan syndrome.Frontiers in pediatrics · 2026Article
- Diagnostic yield and therapeutic implications of 25 years of specialized pediatric Marfan clinic.European journal of pediatrics · 2024Observational
- Pulmonary artery aneurysm: computed tomography (CT) imaging findings and diagnosis.Quantitative imaging in medicine and surgery · 2024Review
- Unique Features of Cardiovascular Involvement and Progression in Children with Marfan Syndrome Justify Dedicated Multidisciplinary Care.Journal of cardiovascular development and disease · 2024Review
- Pediatric Chest Pain: A Review of Diagnostic Tools in the Pediatric Emergency Department.Diagnostics (Basel, Switzerland) · 2024Review
- Frequency of Cardiac Valvulopathies in Patients With Marfan Syndrome: A Systematic Review and Meta-Analysis.Cureus · 2024Review
- Marfan syndrome: insights from animal models.Frontiers in genetics · 2024Review
- Pathology and pathophysiology of the aortic root.Annals of cardiothoracic surgery · 2023Review
- Health-Related Quality of Life of Patients with Marfan Syndrome-Polish Study.International journal of environmental research and public health · 2022Article
- Cardiothoracic surgery and peripheral endovascular intervention in cardiovascular damage from a cohort of orphan rheumatological diseases-epidemiological and survival analysis.Journal of thoracic disease · 2022Article
- How to Distinguish Marfan Syndrome from Marfanoid Habitus in a Physical Examination-Comparison of External Features in Patients with Marfan Syndrome and Marfanoid Habitus.International journal of environmental research and public health · 2022Article
- Marfan syndrome.Nature reviews. Disease primers · 2021Review
- Mitral Valve Prolapse and Its Motley Crew-Syndromic Prevalence, Pathophysiology, and Progression of a Common Heart Condition.Journal of the American Heart Association · 2021Article
- The Molecular Genetics of Marfan Syndrome.International journal of medical sciences · 2021Review
- Biology and Biomechanics of the Heart Valve Extracellular Matrix.Journal of cardiovascular development and disease · 2020Review
- A scoping review presenting a wide variety of research on paediatric and adolescent patients with Marfan syndrome.Acta paediatrica (Oslo, Norway : 1992) · 2020Article
- New Screening Tool for Aortic Root Dilation in Children with Marfan Syndrome and Marfan-Like Disorders.Pediatric cardiology · 2020Article
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Authors and funding
9 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Marfan syndrome (MFS) is a connective tissue disorder characterized by a broad range of clinical manifestations. Cardiovascular involvement is the most life-threatening aspect of the syndrome. Although abnormalities within the cardiovascular system in adults are well documented, there is still a paucity of data regarding manifestation of MFS in childhood. The aim of the study was to compare cardiovascular manifestation of MFS between children and adults. The study population consisted of 236 patients (144 children and 92 adults), who were referred to our department with suspicion of MFS. All patients underwent complete clinical evaluation in order to confirm the diagnosis of MFS according to the modified Ghent criteria. MFS was diagnosed in 101 (44 children and 57 adults) out of the 236 patients. The other patients were diagnosed with Ehlers-Danlos syndrome, Loeys-Dietz syndrome, MASS phenotype, ectopia lentis syndrome, marfanoid habitus and other rare syndromes. The most common cardiovascular abnormality was aortic root dilatation (81.19% of patients). It was found that both adults and children had similar high rates of aortic root dilatation. Similarly, there was no significant difference with regard to the prevalence of aortic valve regurgitation and mitral valve prolapse among children and adults. These findings equivocally indicate that the aforementioned abnormalities develop in early childhood, therefore, they may be used in the early identification of patients with MFS. Other assessed abnormalities, which included mitral valve regurgitation, pulmonary artery dilation, aneurysms of aortic arch, descending thoracic aorta and abdominal aorta were found mostly in adults, and thus, are of less use in the early detection of MFS.
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