Importance of dural ectasia in phenotypic assessment of Marfan's syndrome.

BACKGROUND: Early identification of Marfan's syndrome is fundamental in the prevention of aortic dilatation, but the wide phenotypic expression of the disorder makes the clinical diagnosis very difficult. Dural ectasia has been classified as a major diagnostic criterion; however, its prevalence is not known. We aimed to identify the true prevalence of dural ectasia in Marfan's syndrome, and to investigate its relation to aortic pathology. METHODS: A magnetic-resonance-imaging (MRI) study of the thoracic aorta and of the lumbosacral spine was done in an inclusive series of 83 patients with Marfan's syndrome to assess the presence and degree of dural ectasia and aortic involvement; 12 patients were younger than 18 years. 100 individuals who underwent MRI of the lumbar spine for routine clinical indications represented the control group; none of them had any potential causes for dural ectasia. FINDINGS: Dural ectasia was identified in 76 (92%) patients and none of the control group. The severity of dural ectasia was related to age; the mean (SD) age of patients with mild dural ectasia was 26 years (14) whereas that of those with severe disease (meningocele) was 36 years (9) (p=0.038). 11 of 12 patients younger than 18 years had dural ectasia. No association was found between aortic dilatation and dural ectasia. INTERPRETATION: Dural ectasia is a highly characteristic sign of Marfan's syndrome, even at an early age.

Composite graft replacement of the ascending aorta: leakage detection with gadolinium-enhanced MR imaging.

PURPOSE: To assess the value of magnetic resonance (MR) imaging in the detection of postoperative complications after composite valve graft replacement. MATERIALS AND METHODS: Spin-echo and gradient-echo MR imaging was performed in 52 patients 1/2 to 200 months after composite graft replacement of the ascending aorta (22 for dissection, 30 for aneurysm). The prosthetic aortic segment, distal and proximal anastomoses, general morphologic characteristics, and diameter of the reimplanted coronary arteries were evaluated. In patients with abnormal perigraft thickening, additional spin-echo imaging was performed after injection of gadopentetate dimeglumine. RESULTS: Normal postoperative perigraft thickening (< or = 10 mm) was observed in 42 patients. Ten patients had abnormal periprosthetic thickening of 15-52 mm. Gadolinium-enhanced MR imaging demonstrated leakage in five of those 10 patients. The lack of enhancement excluded the presence of bleeding in the remaining five patients (three with chronic hematomas, one with infection, and one with granulation tissue). These findings were confirmed at surgery or with subsequent follow-up MR examinations. CONCLUSION: MR imaging was an optimal imaging modality for evaluating the morphologic characteristics of composite grafts and reimplanted coronary arteries. Gadolinium-enhanced MR imaging is a simple, accurate, and noninvasive method for detecting a leak, which necessitates urgent repeat surgery.

Long-term arterial patency after coronary reperfusion.

Coronary reocclusion is a frequent event after reperfusion and may be responsible for the deterioration of left ventricular function. It may occur early as well as in the chronic phase after hospital discharge. Current, evidence based, strategies to prevent reocclusion include antiplatelet and anticoagulant agents as well as the use of intracoronary stenting in those patients who are treated by PTCA. The combination of aspirin and ticlopidine adds on the results of stenting. Further treatments are currently investigated and may significantly improve the long-term coronary patency.

Evolution of post-traumatic aortic aneurysm in the subacute phase: magnetic resonance imaging follow-up as a support of the surgical timing.

OBJECTIVES: A recent surgical series documented that in traumatic aortic rupture (TAR) a surgical repair postponed to the treatment of associated lesions reduced operative and overall mortality. Nevertheless some isolated cases may develop to free rupture. Until now, no imaging follow-up studies of post-traumatic aortic lesions have been reported in the early stage. The aim of this study is to analyze the behaviour of traumatic aortic ruptures in the subacute phase, in order to detect the morphological characteristics of unstable post-traumatic aneurysms. METHODS: Twenty-five consecutive patients affected by traumatic aortic rupture (one intimal hemorrhage, 19 partial lesions and five circumferential lesions) were admitted to the department of cardiac surgery. Magnetic resonance imaging (MRI) was the imaging method used to confirm the diagnosis. No one was operated on during the acute phase. All patients were treated with beta-blockers and vasodilators as well as limited fluid administration. Delayed surgery was carried out in 18 patients at 243 days (+/-127), after the resolution of associated lesions. A scheduled MRI follow-up was performed at 7, 15 and 30 days and immediately before the operation. The parameters examined were increase of post-traumatic aneurysm, increase of periaortic hematoma and modification of the thoracic associated lesions. RESULTS: At 30 days a 3.0 +/- 3.7 mm median increase of the aneurysm was observed, while in the subsequent period the lesions became substantially stable, resulting in a 4.4 +/- 3.6 mm increment at the end of the follow-up. The circumferential lesions presented a higher increment with respect to the partial lesions. In three cases an augmentation of 6, 7 and 12 mm was detected and surgical repair was anticipated. In 13 cases a periadventitial hematoma surrounding the aortic aneurysm decreased through the time. One case of intimal hemorrhage healed spontaneously, with no aneurysm formation. Thoracic associated lesions (pleural and pericardial effusions, rib fractures, lung focal contusions and two cases of ARDS) resolved at 30-60 days. CONCLUSIONS: Despite common knowledge, considering TAR highly evolutive in the acute and subacute phase, this study demonstrated that this pathological entity is relatively stable if a proper pharmacological treatment is administrated. MRI follow-up is recommended in order to detect isolated cases of unstable aneurysm.