Cardiovascular Magnetic Resonance in Marfan syndrome

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Abstract

This review provides an overview of Marfan syndrome with an emphasis on cardiovascular complications and cardiovascular imaging. Both pre- and post-operative imaging is addressed with an explanation of surgical management. All relevant imaging modalities are discussed with a particular focus on cardiovascular MR.

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The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease.

P. Hagan, C. A. Nienaber, E. Isselbacher … (2000)

Acute aortic dissection is a life-threatening medical emergency associated with high rates of morbidity and mortality. Data are limited regarding the effect of recent imaging and therapeutic advances on patient care and outcomes in this setting. To assess the presentation, management, and outcomes of acute aortic dissection. Case series with patients enrolled between January 1996 and December 1998. Data were collected at presentation and by physician review of hospital records. The International Registry of Acute Aortic Dissection, consisting of 12 international referral centers. A total of 464 patients (mean age, 63 years; 65.3% male), 62.3% of whom had type A dissection. Presenting history, physical findings, management, and mortality, as assessed by history and physician review of hospital records. While sudden onset of severe sharp pain was the single most common presenting complaint, the clinical presentation was diverse. Classic physical findings such as aortic regurgitation and pulse deficit were noted in only 31.6% and 15.1% of patients, respectively, and initial chest radiograph and electrocardiogram were frequently not helpful (no abnormalities were noted in 12.4% and 31.3% of patients, respectively). Computed tomography was the initial imaging modality used in 61.1%. Overall in-hospital mortality was 27.4%. Mortality of patients with type A dissection managed surgically was 26%; among those not receiving surgery (typically because of advanced age and comorbidity), mortality was 58%. Mortality of patients with type B dissection treated medically was 10.7%. Surgery was performed in 20% of patients with type B dissection; mortality in this group was 31.4%. Acute aortic dissection presents with a wide range of manifestations, and classic findings are often absent. A high clinical index of suspicion is necessary. Despite recent advances, in-hospital mortality rates remain high. Our data support the need for continued improvement in prevention, diagnosis, and management of acute aortic dissection.

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2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with Thoracic Aortic Disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine.

Loren Hiratzka, George L. Bakris, Joshua A Beckman … (2010)

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ESC Guidelines for the management of grown-up congenital heart disease (new version 2010).

H Baumgartner, P Bonhoeffer, N. M. S. de Groot … (2010)

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Author and article information

Contributors

Helen Dormand

Raad H Mohiaddin

Journal

Journal ID (nlm-ta): J Cardiovasc Magn Reson

Journal ID (iso-abbrev): J Cardiovasc Magn Reson

Title: Journal of Cardiovascular Magnetic Resonance

Publisher: BioMed Central

ISSN (Print): 1097-6647

ISSN (Electronic): 1532-429X

Publication date Collection: 2013

Publication date (Electronic): 15 April 2013

Volume: 15

Issue: 1

Page: 33

Affiliations

[1 ]Manchester Heart Centre, Manchester Royal Infirmary, Oxford Road, Manchester, M13 9WL, UK

[2 ]Royal Brompton Hospital and National Heart & Lung Institute, Imperial College, Sydney Street, London SW3 6NP, UK

Article

Publisher ID: 1532-429X-15-33

DOI: 10.1186/1532-429X-15-33

PMC ID: 3651373

PubMed ID: 23587220

SO-VID: 9ebd7d97-608e-4fc8-b5f4-3d04d1469403

License:

This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Cardiovascular Magnetic Resonance in Marfan syndrome

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Abstract

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Cardiovascular Innovations and Applications

Most cited references 108

The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease.

ESC Guidelines for the management of grown-up congenital heart disease (new version 2010).

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