Biophysical properties of the normal-sized aorta in patients with Marfan syndrome: evaluation with MR flow mapping

PURPOSE: To investigate the feasibility of magnetic resonance (MR) flow mapping in the assessment of aortic biophysical properties in patients with Marfan syndrome and to detect differences in biophysical properties in the normal-sized aorta distal to the aortic root between these patients and matched control subjects. MATERIALS AND METHODS: Seventy-eight patients with Marfan syndrome with aortic root dilatation and 23 matched control subjects underwent MR flow mapping in four locations in the normal-sized aorta (1, ascending aorta; 2, thoracic descending aorta; 3, descending aorta at the level of the diaphragm; and 4, abdominal descending aorta). Distensibility at each location and flow wave velocity between locations were calculated. RESULTS: Compared with the control subjects, patients with Marfan syndrome had decreased aortic distensibility at three of the four locations (levels 1, 2, and 4; P <.05) and increased flow wave velocity between all locations (P <.05) in the aorta. In patients with Marfan syndrome, flow wave velocity was also significantly increased along the entire aortic tract beyond the aortic root (from level 1 to level 4). CONCLUSION: MR imaging reveals abnormal biophysical properties of the normal-sized aorta in patients with Marfan syndrome. Monitoring of these properties is relevant for evaluating disease progression and treatment options.     

The thoracic aorta studied by MR imaging

Thirty-three patients with a variety of disorders of the thoracic aorta (aneurysm, dissection, Marfan syndrome, coarctation/pseudocoarctation, L-transposition, and Takayasu disease) were evaluated with magnetic resonance (MR) imaging. MR imaging delineated the presence and extent of thoracic aortic aneurysms and showed the relationship of the aneurysm to arch vessels; it also demonstrated intimal flaps and individual lumina in types A and B aortic dissection. Dilation of the ascending aorta in Marfan syndrome and focal narrowing of the aorta in coarctation were well visualized. The anteroposterior and side-to-side relationships of the aorta and pulmonary artery in L-transposition were demonstrated, as were aortic wall thickening and branch vessel narrowing in Takayasu arteritis. Initial experience suggests that MR imaging may provide a noninvasive method for evaluating thoracic aortic disease. Limitations include inferior spatial resolution, occasional difficulty in imaging the entire region of interest in one section, lack of signal from calcifications, and inability to monitor critically ill patients.     

马凡综合征主动脉病变MRI诊断的研究

目的：评价ＭＲＩ对马凡综合征主动脉病变诊断的价值。材料与方法：分析了２０例马凡综合征主动脉病变的ＭＲＩ所见，并与１１例非马凡综合征所致的升主动脉扩张或动脉瘤进行对照研究。结果：主动脉窦和近段升主动脉瘤样扩张是马凡综合征最重要而常见的心血管病变，“瘤体”与正常段或轻度扩张的主动脉分界清楚是颇具特征性的马凡综合征心血管病变，继发于主动脉瓣狭窄和（或）关闭不全的升主动脉扩张大多呈普遍轻度扩张，而主动脉窦大多无明确扩张，其ＭＲＩ所见与马凡综合征升主动脉病变明显不同。结论：ＭＲＩ在马凡综合征主动脉病变的诊断中有优良的价值。     

Case report 582

A patient with Marfan syndrome presented with enlargement of lumbosacral spinal canal with a bulky meningocele, and a fusiform aneurysm of the ascending aorta. In Marfan syndrome, the presence of a meningocele is extremely rare, but 60% of the patients develop cardio-vascular complications (a frequent cause of death). MRI is the imaging technique of choice for the diagnosis of meningocele and aortic aneurysm.     

Marfan syndrome: evaluation with MR imaging versus CT

Twenty-five patients with Marfan syndrome underwent computed tomography (CT) and magnetic resonance (MR) imaging. MR images were interpreted in blinded fashion and then the results were compared with findings from CT scans. MR imaging was found to be equivalent to CT in the depiction of aortic, dural, and hip abnormalities in patients who had not undergone surgery. MR imaging was superior to CT in evaluation of postoperative patients because the artifact produced by Bjork-Shiley or St. Jude valves precludes adequate evaluation of the aortic root on CT scans, while producing only a small inferior field distortion, a "pseudo-ventricular septal defect," on MR images. The absence of radiation exposure is another significant advantage for the relatively young Marfan syndrome population, who require serial studies. MR imaging is the modality of choice for evaluation and follow-up of patients with Marfan syndrome and offers an appropriate means of screening their kindred.     

Aortic dissection: atypical patterns seen at MR imaging

Aortic dissection is typically manifested by an intimal flap separating true and false lumens. Atypical patterns lacking an intimal flap can occur, however, and are less well recognized. In this study, the authors review their experience with atypical patterns at magnetic resonance (MR) imaging. They evaluated the T1-weighted and gradient-echo MR images of the entire aorta in 64 patients with aortic dissection. In nine patients (14%) aortic wall thickening was the only sign of dissection in the thorax. In three of these cases, an intimal flap was found in the abdominal aorta; the other six patients had no flap in any region of the aorta, and this led to false-negative angiographic results in three cases. Aortic wall thickening as an atypical sign of dissection was seen more frequently in acute (29%) than in chronic (7%) dissection and was seen in only patients without Marfan syndrome. Two patients with atypical acute dissection at initial examination developed a clearly identifiable intimal flap at follow-up. Aortic wall thickening may represent the only sign of aortic dissection. In some cases, imaging the entire aorta can enable recognition of dissection in atypical cases.     

Real-time MR imaging of aortic flow: influence of breathing on left ventricular stroke volume in chronic obstructive pulmonary disease

PURPOSE: To assess real-time changes of left ventricular stroke volume (SV) in relation to the breathing pattern in healthy subjects and in patients with chronic obstructive pulmonary disease (COPD). MATERIALS AND METHODS: Real-time magnetic resonance (MR) imaging flow measurements were performed in the ascending aorta of 10 healthy volunteers and nine patients with severe COPD. Breathing maneuvers were registered with an abdominal pressure belt, which was synchronized to the electrocardiographic signal and the flow measurement. Healthy subjects performed normal breathing, deep breathing, and the Valsalva maneuver. Patients with COPD performed spontaneous breathing. Paired two-tailed Student t tests were used in healthy volunteers to assess significant SV differences between normal breathing and deep breathing or the Valsalva maneuver. The results of measurements in the patients with COPD were compared with the results during normal breathing in healthy subjects with the unpaired two-tailed Student t test. RESULTS: In healthy subjects, SV decreased during inspiration and increased during expiration (r2 = 0.78, P <.05). When compared with the SV during normal breathing, mean SV did not change during deep breathing but declined during the Valsalva maneuver (P <.05). The difference between minimal and maximal SVs (ie, SV range) increased because of deep breathing or the Valsalva maneuver (P <.05). In normal and deep breathing, velocity of SV elevation and velocity of SV decrease were equal (which resulted in a ratio of 1), whereas during the Valsalva maneuver, this value increased (P <.05). Spontaneous breathing in COPD resulted in SV changes (P <.05) similar to those observed in healthy subjects who performed the Valsalva maneuver. CONCLUSION: Real-time MR imaging of aortic flow reveals physiologic flow alterations, which are dependent on variations in the breathing pattern.     

马凡综合征升主动脉病变17例影像分析

本文对17例马凡综合征患者的血管造影、CT 和MR 进行了分析,并对马凡综合征升主动脉瘤同其他原因引起的升主动脉瘤进行了比较,分别测量马凡综合征患者和其他原因引起的升主动脉瘤的窦直径、升中径、弓前径、弓中径、降上径,发现马凡综合征患者的升主动脉瘤最大直径均位于主动脉窦部,窦横径与弓前径的比值均≥1.78;而非马凡综合征患者的这一比值均明显<1.78。本文马凡综合征患者中有82%(14/17)合并主动脉瓣关闭不全。     

Aortic compliance in patients with aortic regurgitation: evaluation with magnetic resonance imaging

PURPOSE: To assess by means of cine magnetic resonance imaging (MRI) aortic compliance before and after aortic valve replacement (AVR with SJM valve) in patients with aortic regurgitation (AR). MATERIALS AND METHODS: Two groups (healthy controls and patients with severe isolated AR) of 10 subjects each were included in this study. Cine MRI was performed at three locations of the aorta, and aortic compliance was calculated by dividing the maximum change in the aortic area by pulse pressure. RESULTS: Cine MRI is useful to assess abnormalities of aortic compliance in patients with AR. Compared with the control group, aortic compliance in the AR group was significantly less in the ascending aorta (p<0.05), decreasing in order of aortic location. After AVR, aortic compliance improved for all locations. CONCLUSION: Cine MRI enables assessment of aortic biophysical properties such as a compliance for evaluating the progression of AR and the efficacy of treatment.     

MR相位对比法和形态体积分析法评价心室功能的比较研究

目的探讨相位对比法MR和形态体积分析法MR成像技术在评价心室功能中的临床应用价值。方法应用相位对比法MR(phasecontrastmagneticresonanceimaging,PCMRI)和形态体积分析法电影MRI(stereologicalcinemagneticresonanceimaging,SCMRI)分别对12例健康成年人和46例心脏病患者进行检查。首先应用SCMRI行心脏左、右室短轴电影成像,分别描记左、右室心内、外膜界面,测出左右心室舒张末期容量(EDV)、收缩末期容量(ESV)、每搏输出量(SV)、射血分数(EF)等指标;再应用PCMRI和流量分析软件分别在主动脉瓣上和肺动脉瓣上水平测得1个心动周期内的前向血流,获得SV值,并将2种方法的测量结果进行比较;同时对正常志愿者与患者组、正常志愿者组左、右两侧心室,以及PCMRI法在主动脉瓣上和肺动脉瓣上水平测得的左右心室SV均值进行比较。结果(1)PCMRI与SCMRI法所测正常志愿者左、右心室的SV相关良好,相关系数分别为090和087,2种方法测得的SV均值差异无统计学意义(P>005)。(2)患者组2种MRI测量方法所得左室SV的相关系数为085,SV均值比较差异无统计学意义(P>005),应用PCMRI法在主动脉瓣上水平测得正常人与患者组的SV均值,差异有统计学意义(P<005)。结论PCMRI与SCMRI法相比,对心室每搏输出量(SV)测量准确,相关性好,重复性高,加     

Aortic annulus and ascending aorta: Comparison of preoperative and periooperative measurement in patients with aortic stenosis

Background

Precise determination of the aortic annulus size constitutes an integral part of the preoperative evaluation prior to aortic valve replacement. It enables the estimation of the size of prosthesis to be implanted. Knowledge of the size of the ascending aorta is required in the preoperative analysis and monitoring of its dilation enables the precise timing of the operation. Our goal was to compare the precision of measurement of the aortic annulus and ascending aorta using magnetic resonance (MR), multidetector-row computed tomography (MDCT), transthoracic echocardiography (TTE), and transoesophageal echocardiography (TEE) in patients with degenerative aortic stenosis.

Methods and results

A total of 15 patients scheduled to have aortic valve replacement were enrolled into this prospective study. TTE was performed in all patients and was supplemented with TEE, CT and MR in the majority of patients. The values obtained were compared with perioperative measurements. For the measurement of aortic annulus, MR was found to be the most precise technique, followed by MDCT, TTE, and TEE. For the measurement of ascending aorta, MR again was found to be the most precise technique, followed by MDCT, TEE, and TTE.

Conclusion

In our study, magnetic resonance was found to be the most precise technique for the measurement of aortic annulus and ascending aorta in patients with severe degenerative aortic stenosis.     

Quantitative assessment of dural ectasia as a marker for Marfan syndrome

PURPOSE: To establish normal values for lumbosacral dural sac dimensions with magnetic resonance (MR) imaging and to use these values to assess the sensitivity and specificity of dural ectasia as a marker for Marfan syndrome. MATERIALS AND METHODS: MR imaging was performed to measure dural sac diameter (DSD) from L1 through S1 in 44 adult patients with Marfan syndrome and in 44 matched control subjects. DSD values were corrected for vertebral body size, yielding dural sac ratios (DSRs). The control subjects served to establish the upper limit of normal DSR values at the L1 through S1 levels. RESULTS: Cutoff values for normal DSRs for L1 through S1 were 0.64, 0.55, 0.47, 0.48, 0.48, and 0.57. Significant DSR differences were shown at all levels between patients with Marfan syndrome and control subjects (P <.001 at all levels). At L1 through S1, the sensitivity of dural ectasia as a marker for Marfan syndrome was 45%-77%, and the specificity was 95% or greater. By combining levels L3 and S1, dural ectasia as a marker for Marfan syndrome yielded a sensitivity of 95% (42 of 44 patients) and a specificity of 98% (43 of 44). The presence of dural ectasia excelled, compared with the presence of other Marfan syndrome manifestations in the patient population. CONCLUSION: Abnormal DSR values at L3 or S1 can be used to identify Marfan syndrome with 95% sensitivity and 98% specificity.     

Thoracic aorta: comparison of single-dose breath-hold and double-dose non-breath-hold gadolinium-enhanced three-dimensional MR angiography.

OBJECTIVE: The purpose of this study was to compare single-dose (0.1 mmol/kg) breath-hold gadolinium-enhanced three-dimensional (3D) MR angiography and double-dose (0.2 mmol/kg) non-breath-hold 3D MR angiography for evaluation of thoracic aortic disease. MATERIALS AND METHODS: Twenty-five patients referred for MR evaluation of the thoracic aorta underwent non-breath-hold gadolinium-enhanced 3D MR angiography on a 1.5-T scanner with standard gradients (TR/TE, 21/6; flip angle, 30 degrees) during slow infusion of a double dose of gadopentetate dimeglumine using a body coil. Subsequently, the same patients underwent breath-hold MR imaging with high-performance gradients (TR/TE, 5/2; flip angle, 30 degrees-50 degrees), a timing examination, and power injection of a single dose of gadolinium. For both studies, quantitative signal-to-noise measurements were obtained for the ascending thoracic, descending thoracic, and abdominal aorta. Three observers retrospectively evaluated each examination for degree of enhancement of the aorta, pulmonary arteries, and systemic veins; motion artifacts; and overall image quality. RESULTS: Single-dose breath-hold gadolinium-enhanced 3D MR angiography showed greater signal-to-noise ratio, fewer motion artifacts, and better overall image quality (p < .05) than the non-breath-hold double-dose technique. The single-dose technique also showed significantly better qualitative enhancement of the aortic root and ascending aorta (p < .05) and less enhancement of the pulmonary arteries, renal veins, and left internal jugular vein (p < .05). CONCLUSION: Optimized single-dose breath-hold gadolinium-enhanced 3D MR angiography is superior to double-dose non-breath-hold 3D MR angiography for evaluation of thoracic aortic disease.     

MR angiography of the thoracic aorta with an electrocardiographically triggered breath-hold contrast-enhanced sequence.

An electrocardiographically (ECG) triggered breath-hold contrast material-enhanced magnetic resonance (MR) angiography sequence has been developed for imaging the thoracic aorta. A three-dimensional (3D) gradient-echo sequence is used with a contrast material bolus. Forty-nine patients with various aortic abnormalities and five healthy volunteers underwent imaging with the sequence. All studies were performed in a single breath hold. ECG-triggered breath-hold contrast-enhanced MR angiography was tolerated in 48 of the 49 patients. The images demonstrated no respiratory motion artifacts and diminished pulsation artifacts. The cardiac chambers, aortic root, ascending and descending aorta, aortic arch, proximal arch vessels, and proximal coronary arteries were clearly demonstrated and not obscured by ghost artifacts. The 3D data set allowed excellent multiplanar reformation, permitting orthogonal or oblique views of the vascular anatomy. A variety of congenital and acquired abnormalities were clearly identified. When this sequence is used, it is important to evaluate both the maximum-intensity projection and source images. Delayed imaging should be performed to detect late filling. In conjunction with cine MR and T1-weighted spin-echo imaging, ECG-triggered breath-hold contrast-enhanced MR angiography should be considered the technique of choice for imaging the thoracic aorta.     

Infected aortic aneurysms: imaging findings

PURPOSE: To determine the imaging characteristics of infected aortic aneurysms. MATERIALS AND METHODS: Review of records of patients with surgical and/or microbiologic proof of infected aortic aneurysm obtained over a 25-year period revealed 31 aneurysms in 29 patients. This study included 21 men and eight women (mean age, 70 years). One radiologist reviewed 28 computed tomographic (CT) studies (22 patients underwent CT once and three patients underwent CT twice), 12 arteriograms (12 patients underwent arteriography once), eight nuclear medicine studies (six patients underwent nuclear medicine imaging once and one patient underwent nuclear medicine imaging twice), and three magnetic resonance (MR) studies (three patients underwent MR imaging once). Features evaluated included aneurysm size, shape, and location; branch involvement; aortic wall calcification; gas; radiotracer uptake on nuclear medicine studies; and periaortic and associated findings. The location of infected aortic aneurysms was compared with that of arteriosclerotic aneurysms. RESULTS: Aneurysms were located in the ascending aorta (n = 2, 6%), descending thoracic aorta (n = 7, 23%), thoracoabdominal aorta (n = 6, 19%), paravisceral aorta (n = 2, 6%), juxtarenal aorta (n = 3, 10%), infrarenal aorta (n = 10, 32%), and renal artery (n = 1, 3%). Two patients had two infected aortic aneurysms. CT revealed 25 saccular (93%) and two fusiform (7%) aneurysms with a mean diameter at initial discovery of 5.4 cm (range, 1-11 cm). Paraaortic soft-tissue mass, stranding, and/or fluid was present in 13 (48%) of 27 aneurysms, and early periaortic edema with rapid aneurysm progression and development was present in three (100%) patients with sequential studies. Other findings included adjacent vertebral body destruction with psoas muscle abscess (n = 1, 4%), kidney infarct (n = 1, 4%), absence of calcification in the aortic wall (n = 2, 7%), and periaortic gas (n = 2, 7%). Angiography showed 13 saccular aneurysms with lobulated contour in 10 (77%). Nuclear medicine imaging showed increased activity consistent with infection in six (86%) of seven aneurysms. MR imaging showed three saccular aneurysms. Adjacent abnormal vertebral body marrow signal intensity was seen in one (33%) of three patients. CONCLUSION: Saccular aneurysms (especially those with lobulated contour) with rapid expansion or development and adjacent mass, stranding, and/or fluid in an unusual location are highly suspicious for an infected aneurysm.     

Aortic dissection: magnetic resonance imaging

Fifteen patients with suspected or known aortic dissection were imaged with magnetic resonance (MR). Thirteen of these patients were eventually shown to have dissection. In most instances the diagnosis was established by aortography and/or computed tomography (CT) prior to the MR study. Surgical proof (6/13) and/or aortographic proof (10/13) were available in 11/13 patients with aortic dissection. MR demonstrated the intimal flap and determined whether the dissection was type A or type B. In addition, MR: differentiated between the true and false lumens; determined the origins of the celiac, superior mesenteric, and renal arteries from the true or false lumen in the cases where the dissection extended into the abdominal aorta (8/12); allowed post-surgical surveillance of the dissection; and identified aortoannular ectasia in the three patients who had Marfan syndrome. In addition to the 13 cases with dissection, there were two cases in whom the diagnosis of dissection was excluded by MR. Our early experience suggests that MR can serve as the initial imaging test in clinically suspected cases of aortic dissection and that the information provided by MR is sufficient to manage many cases. Additionally, MR obviates the use of iodinated contrast media.     

Breathhold time‐resolved three‐directional MR velocity mapping of aortic flow in patients after aortic valve‐sparing surgery

Purpose

To evaluate the utility of breathhold time‐resolved three‐directional MR velocity mapping for quantifying the restoration of normal flow patterns in patients after aortic valve‐sparing surgery.

Materials and Methods

Breathhold time‐resolved three‐directional MR velocity mapping was performed on 13 patients with aortic valve‐sparing surgery. Ten healthy volunteers and 12 patients with ascending aortic aneurysm underwent the same MR examination for comparison. Aortic laminar flow, turbulent flow, and the presence of vortical flow in the sinuses of Valsalva were semiquantitatively assessed and statistically compared between the three groups of subjects.

Results

The average score of laminar flow in the ascending aorta for patients with surgery was not significantly different from that of volunteers (P = 0.210), but was significantly greater than that of patients with aneurysm (P < 0.01). The average score of turbulent flow in patients with surgery was significantly smaller than that of patients with aneurysm (P < 0.01). The presence of systolic vortical flow in the sinuses of Valsalva for patients with surgery was not significantly different from that of healthy volunteers (P = 0.405) and patients with aneurysm (P = 0.238).

Conclusion

Breathhold time‐resolved three‐directional MR velocity mapping allows for quantifying flow patterns in the aortic root and ascending aorta. Normal laminar flow in the ascending aorta and vortical flow in the sinuses of Valsalva can be restored in patients after aortic valve‐sparing surgery. J. Magn. Reson. Imaging 2009;29:569–575. © 2009 Wiley‐Liss, Inc.     

64排CT主动脉血管造影个体化对比剂用量的可行性研究

目的:探讨体质量小于60kg的患者在主动脉CT造影中减少对比剂用量的可行性。方法:60例行主动脉CTA患者按体质量分为A、B组:A组体质量≥60kg,注射浓度370mgI/mL非离子型对比剂60mL后用40mL生理盐水冲洗,注射速率均为5mL/s;B组为60kg患者,注射同种对比剂,剂量按1mL/kg体质量计算,后用40mL生理盐水冲洗,注射速率均为5mL/s。扫描延迟时间采用自动触发技术,测量升主动脉、T7及L2水平降主动脉、主动脉分叉处CT值;各由1名高级和中级职称影像诊断医师对血管强化程度、重建血管清晰度进行评价,并由另一影像医师比较两者的评价是否存在差异。结果:A组在升主动脉、T7及L2水平降主动脉、主动脉分叉处测得平均CT值为383.19HU,B组为391.58HU,2组差异无统计学意义。A组和B组患者图像重建后血管清晰度差异无统计学意义。结论:在不影响图像质量前提下,体质量小于60kg患者可以使用少于60mL剂量的对比剂行胸腹主动脉造影。     

特殊类型主动脉夹层的电子束CT表现及诊断

目的探讨表现特殊的主动脉夹层的电子束CT(EBCT)影像特征及诊断.材料和方法20例表现特殊的主动脉夹层,其中不典型夹层13例,三腔以上夹层动脉瘤3例,合并升主动脉壁内血肿的Stanford B型夹层2例,动脉瘤样夹层1例,外伤性主动脉夹层1例.结果主动脉不典型夹层为主动脉壁新月形或环形的低密度血肿包绕,常可见穿透性溃疡或钙化内移等征象;三腔以上夹层动脉瘤有2～3个内膜片,3～4个腔,瘤体管径较大;合并升主动脉壁内血肿的B型夹层见升主动脉管壁低密度新月形或环形增厚,降主动脉则见内膜片及真假两腔形成;动脉瘤样主动脉夹层见降主动脉局限性瘤样扩张,破口大,内膜片不易发现.1例外伤性夹层于主动脉弓峡部及降部起始见破裂内膜片.结论特殊类型的主动脉夹层表现各具特征,EBCT可清晰显示,是极适用于胸部急症的快速、无创的检查方法.     

Atypical presentation of dissection of the ascending aorta in young men with cystic medial necrosis: MR findings

Dissection of the ascending aorta is usually associated with severe chest and/or back pain. We describe three young men, with pathologically proven cystic medial necrosis, who presented with atypical clinical symptoms and ascending aortic dissection diagnosed by MR imaging and surgery. Patients with cystic medial necrosis and aortic dissection may not present with a classic acute chest pain syndrome.