☆ 4.7 Editorial Material

Marfan Syndrome Expanding the Cardiovascular Phenotype?

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY (2021)

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Article Clinical Neurology

Intracranial Aneurysms Are Associated With Marfan Syndrome Single Cohort Retrospective Study in 118 Patients Using Brain Imaging

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Summary: This study found a high prevalence of IAs in patients with MFS, with a mean age in the IA group significantly higher and a higher female predominance. All IAs were unruptured with larger diameters, more frequently located in the vertebrobasilar artery and with fusiform morphology in MFS patients compared to controls.

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Article Cardiac & Cardiovascular Systems

Coronary Artery Aneurysms in Patients With Marfan Syndrome: Frequent, Progressive, and Relevant

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Summary: This retrospective cohort study of 109 adult patients with Marfan syndrome found that coronary artery aneurysms (CAAs) are common in these patients and are associated with a more severe aortic phenotype and longer follow-up after aortic-root replacement. The study suggests that regular monitoring of coronary artery size, especially after aortic-root replacement and in patients with severe aortic phenotypes, is necessary. Large multicentre studies are needed to determine the most appropriate surveillance plan.

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Article Genetics & Heredity

Clinical relevance of genotype-phenotype correlations beyond vascular events in a cohort study of 1500 Marfan syndrome patients with FBN1 pathogenic variants

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Summary: This study revealed the existence of FBN1 genotype-phenotype correlations for both aortic and extra-aortic features, which can aid in optimal risk stratification of patients, genetic counseling, and personalized medicine. It also contributes additional data to the overall understanding of the role of fibrillin-1 in various organs.

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Article Cardiac & Cardiovascular Systems

Aortic Branch Aneurysms and Vascular Risk in Patients With Marfan Syndrome

Angela Lopez-Sainz et al.

Summary: This study revealed that aortic branch aneurysms are present in approximately one-quarter of patients with MFS, are associated with patient age and aortic dilation, and independently predict the need for aortic surgery.

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY (2021)

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The genetics of aortopathies: Hereditary thoracic aortic aneurysms and dissections

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AMERICAN JOURNAL OF MEDICAL GENETICS PART C-SEMINARS IN MEDICAL GENETICS (2020)

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Optimising Aortic Endovascular Repair in Patients with Marfan Syndrome

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EUROPEAN JOURNAL OF VASCULAR AND ENDOVASCULAR SURGERY (2020)

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Increased visceral arterial tortuosity in Marfan syndrome

Bence Agg et al.

ORPHANET JOURNAL OF RARE DISEASES (2020)

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Update on Clinical Trials of Losartan With and Without β-Blockers to Block Aneurysm Growth in Patients With Marfan Syndrome A Review

Marion A. Hofmann Bowman et al.

JAMA CARDIOLOGY (2019)

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Marfan syndrome: improved clinical history results in expanded natural history

Reed E. Pyeritz

GENETICS IN MEDICINE (2019)

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The fate of nonaortic arterial segments in Marfan patients

Florian S. Schoenhoff et al.

JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY (2019)

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Article Surgery

Endovascular thoracic aortic repair in confirmed or suspected genetically triggered thoracic aortic dissection

Sherene Shalhub et al.

JOURNAL OF VASCULAR SURGERY (2018)

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Editorial Material Cardiac & Cardiovascular Systems

Marfan Syndrome Always Evolving

Guillaume Jondeau et al.

CIRCULATION-CARDIOVASCULAR GENETICS (2017)

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Systematic review and meta-analysis of surgical outcomes in Marfan patients undergoing aortic root surgery by composite-valve graft or valve sparing root replacement

Campbell D. Flynn et al.

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Repair of Multiple Subclavian and Axillary Artery Aneurysms in a 58-Year-Old Man with Marfan Syndrome

Ahmet Dolapoglu et al.

TEXAS HEART INSTITUTE JOURNAL (2016)

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Article Cardiac & Cardiovascular Systems

Repair of Multiple Subclavian and Axillary Artery Aneurysms in a 58-Year-Old Man with Marfan Syndrome

Ahmet Dolapoglu et al.

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Article Medicine, General & Internal

Atenolol versus Losartan in Children and Young Adults with Marfan's Syndrome

R. V. Lacro et al.

NEW ENGLAND JOURNAL OF MEDICINE (2014)

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Article Genetics & Heredity

The revised Ghent nosology for the Marfan syndrome

Bart L. Loeys et al.

JOURNAL OF MEDICAL GENETICS (2010)

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Article Genetics & Heredity

Abdominal visceral findings in patients with Marfan syndrome

Kira Chow et al.

GENETICS IN MEDICINE (2007)

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Article Genetics & Heredity

Characterization of the symptoms associated with dural ectasia in the Marfan patient

JRH Foran et al.

AMERICAN JOURNAL OF MEDICAL GENETICS PART A (2005)

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Article Clinical Neurology

Atlantoaxial rotatory subluxation in patients with Marfan syndrome - A report of three cases

A Herzka et al.

SPINE (2000)

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