ArticleOrphanet journal of rare diseases2020
Myocardial disease and ventricular arrhythmia in Marfan syndrome: a prospective study.
Article in Orphanet journal of rare diseases, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 30 citations in OpenAlex.
- Prognostic factors in patients with Marfan syndrome referred to a cardiac surgery center: a retrospective cohort study.BMC cardiovascular disorders · 2026Article
- Systematic Disruption of Zebrafish Fibrillin Genes Identifies a Translational Zebrafish Model for Marfan Syndrome.JACC. Basic to translational science · 2026Article
- Intrinsic cardiomyopathy in pediatric Marfan syndrome: predictive factors and risk assessments.Pediatric research · 2025Article
- Ventricular dispersion and repolarization in Marfan syndrome: A clinical analysis.Heart rhythm O2 · 2025Article
- Marfan syndrome cardiomyocytes show excess of titin isoform N2BA and extended sarcomeric M-band.The Journal of general physiology · 2025Article
- Advances in Cardiovascular Multimodality Imaging in Patients with Marfan Syndrome.Diagnostics (Basel, Switzerland) · 2025Review
- Management of aortic disease in children with FBN1-related Marfan syndrome.European heart journal · 2024Review
- Marfan Syndrome, Hypertrophic Cardiomyopathy And Long QT A Rare Association Causing Sudden Death.Arquivos brasileiros de cardiologia · 2024Article
- Is Marfan Syndrome Associated with Primary Structural Changes in the Left Atrium?Diagnostics (Basel, Switzerland) · 2023Article
- Cardiovascular Magnetic Resonance Imaging Patterns in Rare Cardiovascular Diseases.Journal of clinical medicine · 2022Review
- Cardiac Complications in Marfan Syndrome: A Review.Cureus · 2022Review
- Sleep apnea predicts cardiovascular death in patients with Marfan syndrome: a cohort study.The EPMA journal · 2022Article
- Cardiomyopathies in Children and Systemic Disorders When Is It Useful to Look beyond the Heart?Journal of cardiovascular development and disease · 2022Review
- Association of Mitral Annular Disjunction With Cardiovascular Outcomes Among Patients With Marfan Syndrome.JAMA cardiology · 2021Article
- Non-aortic cardiovascular disease in Marfan syndrome: a nationwide epidemiological study.Clinical research in cardiology : official journal of the German Cardiac Society · 2021Article
- Cardiomyopathy in Genetic Aortic Diseases.Frontiers in pediatrics · 2021Review
- An Overview of Investigational and Experimental Drug Treatment Strategies for Marfan Syndrome.Journal of experimental pharmacology · 2021Review
- Myocardial Function, Heart Failure and Arrhythmia in Marfan Syndrome: A Systematic Literature Review.Diagnostics (Basel, Switzerland) · 2020Review
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Authors and funding
9 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAortic root dilatation and-dissection and mitral valve prolapse are established cardiovascular manifestations in Marfan syndrome (MFS). Heart failure and arrhythmic sudden cardiac death have emerged as additional causes of morbidity and mortality.
methodsTo characterize myocardial dysfunction and arrhythmia in MFS we conducted a prospective longitudinal case-control study including 86 patients with MFS (55.8% women, mean age 36.3 yr-range 13-70 yr-) and 40 age-and sex-matched healthy controls. Cardiac ultrasound, resting and ambulatory ECG (AECG) and NT-proBNP measurements were performed in all subjects at baseline. Additionally, patients with MFS underwent 2 extra evaluations during 30 ± 7 months follow-up. To study primary versus secondary myocardial involvement, patients with MFS were divided in 2 groups: without previous surgery and normal/mild valvular function (MFS-1; N = 55) and with previous surgery or valvular dysfunction (MFS-2; N = 31).
resultsCompared to controls, patients in MFS-1 showed mild myocardial disease reflected in a larger left ventricular end-diastolic diameter (LVEDD), lower TAPSE and higher amount of (supra) ventricular extrasystoles [(S)VES]. Patients in MFS-2 were more severely affected. Seven patients (five in MFS-2) presented decreased LV ejection fraction. Twenty patients (twelve in MFS-2) had non-sustained ventricular tachycardia (NSVT) in at least one AECG. Larger LVEDD and higher amount of VES were independently associated with NSVT.
conclusionOur study shows mild but significant myocardial involvement in patients with MFS. Patients with previous surgery or valvular dysfunction are more severely affected. Evaluation of myocardial function with echocardiography and AECG should be considered in all patients with MFS, especially in those with valvular disease and a history of cardiac surgery.
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