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Netherlands Heart Journal
Gepubliceerd in:

01-11-2008 | review article

The distal aorta in the Marfan syndrome

Auteur: B. J. M. Mulder

Gepubliceerd in: Netherlands Heart Journal | Uitgave 11/2008

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Extract

The leading cause of premature death in patients with Marfan syndrome is aortic dissection due to progressive dilation of the aorta. The abnormal fibrillin protein which results from the genetic defect that causes the disease gives rise to pathology of the aortic wall, especially of the proximal aorta. Elective repair of the aortic root has greatly improved survival of patients with Marfan syndrome. It has been recommended to replace the aortic root before the aortic diameter exceeds 50 to 55 mm.1-3 Adherence to these guidelines may lead to a substantial reduction of type A dissections in Marfan syndrome.4,5 Measurement of aortic diameters have, therefore, become a strongly recommended strategy of management (figures 1 and 2). Asymmetric dilation of the aortic root may go unnoticed as in the majority of Marfan patients the largest aortic diameter (from left to right coronary cusp) might not be detected with the standard echocardiography measurements in the long-axis view (right to noncoronary cusp).6 …
vorige artikel Restenosis begets restenosis: implications for stent selection
volgende artikel A rare combination of coronary anomalies
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Metagegevens
Titel
The distal aorta in the Marfan syndrome
Auteur
B. J. M. Mulder
Publicatiedatum
01-11-2008
Uitgeverij
Bohn Stafleu van Loghum
Gepubliceerd in
Netherlands Heart Journal / Uitgave 11/2008
Print ISSN: 1568-5888
Elektronisch ISSN: 1876-6250
DOI
https://doi.org/10.1007/BF03086183

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