International Journal of Cardiac Imaging 8: 249-254, 1992. 9 1992 KluwerAcademic Publishers. Printedin the Netherlands. Detection of aortic dissection by magnetic resonance imaging in adults with Marfan's syndrome J.H.Z. Bfinki1, L.C. Meiners 1'*, J.O. Barentsz 2 & T.D. Witkamp 1 1 University Hospital, 3508 GA Utrecht, The Netherlands; 2 St. Radboud Hospital, Nijmegen, The Netherlands (* corresponding attthor) Accepted 12 June 1992 Key words: Marfan, MRI, aortic dilatation, dissection, symptomatic, asymptomatic Abstract Marfan's syndrome is a connective tissue disorder, which mainly affects the cardiovascular system, together with ocular, skeletal and connective tissue. The cardiovascular manifestations in particular determine the mortality of this disorder. We studied 13 patients, suffering of Marfan's syndrome, with suspected aortic dilatation by means of magnetic resonance imaging (MRI). Aneurysmal dilatation of the aortic root and dissection were the two major findings in our study. Two of the five patients with dissections presented without relevant symptoms in the medical history nor at the time of the MRI examination. Abbreviations: AD - descending aorta, LAO - left anterior oblique, MRI- magnetic resonance imaging, PA - prearch, SV - sinus Valsalvae, SD - standard deviation Introduction Marfan's syndrome has been known as a clinical entity since 1896 [1]. It is an autosomal dominant heritable disease of connective tissue with variable manifestations. Pyeritz and McKusick described these manifestations and redefined the diagnostic criteria [2]. The true incidence of the syndrome in all its variations may be about one per 10,000 peo- ple, without racial or ethnic predilection. Family history has its value for detection in those who have few or subtle features of the Marfan syndrome. No family history of Marfan's syndrome is found in at the most 25% of the patients. Cardiovascular, ocular, skeletal and connective tissue disorders are the most important findings. However, dermal, central nervous system and pleural manifestations are also described [2]. The presence of ocular and skeletal abnormal- ities or other noncardiac findings, although essen- tial in making the diagnosis, are not specifically associated with a poor prognosis. The unfavorable prognosis of Marfan's syndrome concerning life expectancy is most commonly associated with the group of cardiovascular manifestations [3]. The methods for detecting these cardiovascular complications were the usual invasive and non- invasive imaging modalities of cardiology and ra- diology, viz echocardiography and angiography. These methods, although useful, are limited in the work-up of a more extensive visualization of the aorta and in repeatability in case of the invasive methods, which involve contrast media and ioniz- ing radiation. It seemed worthwhile to use the new imaging method of magnetic resonance imaging (MRI) for detection and follow-up of aortic involvement in Marfan's syndrome. For this reason a group of