Dissecting aneurysm in a patient with Marfan's syndrome: recognition of extensive involvement of the aorta by two-dimensional echocardiography.
Abstract
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To determine the ability of two-dimensional echocardiography (2-D echo) to detect aneurysms and dissections of the descending thoracic aorta (DTA), we studied 15 patients, five with proved DTA dissections (group A) and 10 with proved aneurysms without dissection (group B), using 2-D echo in three recording positions: precordial, suprasternal and a modified apical. The DTA was visualized in 14 of 15 patients (93%); in each patient in group A, an intimal flap was recognized (in two patients only b...
Abstract The usefulness of two dimensional echocardiography in establishing the diagnosis of aortic dissection was evaluated. Forty-two patients were referred for study; 15 had a dissection and 27 did not. Two dimensional echocardiography detected the intimai flap in 12 of 15 patients with a dissection; the three false negative studies were in patients with a localized dissection. There was one false positive study in the 27 patients who did not have a dissection.
Abstract Two dimensional echocardiography was used to identify the descending thoracic aorta in 106 subjects. In 12 of these subjects, contrast injection techniques were used to identify this structure as it descended posteriorly adjacent to the atrioventricular groove. The course of the descending thoracic aorta was mapped using both the long axis and transverse axis views. The normal descending thoracic aorta (26 subjects) measures 10 ±1.4 mm / m 2 during diastole. Unusual M mode echocardiogra...
Anuloaortic ectasia: angiographic, hemodynamic and clinical comparison with aortic valve insufficiency.
Abstract Thirty-five patients with anglographically diagnosed anuloaortic ectasia were compared with 18 patients with isolated aortic valve insufficiency to study differences between the presentation, hemodynamic or anglographic findings and clinical course in the two groups. The maximal aortic root diameter in anuloaortic ectasia was 7.6 ± 2.4 cm (mean ± 1 standard deviation) versus 4.2 ± 0.6 in aortic insufficiency. Dissection was a common complication of anuloaortic ectasia (44 percent) but w...
Abstract Six patients with aortic root dissection proved by angiography, surgery or autopsy, and six patients with aortic root dilatation were studied by echocardiography. Echocardiography was diagnostic in five of six patients with dissection and suggestive In the sixth, disclosing anterior and posterior dissection in three, anterior dissection in one and posterior dissection in one. The recording of a double echo in the aorta was the diagnostic feature. Angiography was diagnostic in four of th...
Abstract Echocardiographic findings from 10 patients without clinical indications of aortic root dissection or aortic valve disease and from 1 patient with angiographic confirmation of aortic root dissection are reported and compared. Previously reported echocardiographic findings were confirmed in the patient with aortic root dissection. These include (1) a widened posterior or anterior aortic wall, or both; (2) parallel motion of the separated margins of the aortic root walls; and (3) aortic r...
Abstract The Marfan syndrome is a dominantly inherited disorder of connective tissue with multisystem involvement. The cardiac complications, particularly aortic dilatation, dissection and rupture and involvement of the aortic and mitral valves, lead to a greatly reduced life expectancy. This poor survival was demonstrated in a series of 257 patients with the Marfan syndrome. The average age at death for the 72 deceased patients was 32 years. Cardiac problems led to 52 of the 56 deaths of known ...
Cystic medionecrosis (CMN) characteristically affects the aorta. That it also may involve vessels in the lung is not widely appreciated. Two patients are presented in whom cystic medionecrosis of the pulmonary arteries accounted for dramatic clinical and pathologic findings. One had massive dilatation of both main pulmonary arteries together with a healed dissecting aneurysm extending from the aortic arch to the iliac bifurcation. The other had an enormous saccular aneurysm of the left main pulm...
The presence of cystic medial necrosis in the human ascending thoracic aorta was determined by histologic examination in a series of 250 necropsies. Cases of Marfan's syndrome, idiopathic dilatation of the aorta and dissecting aneurysm were not included. The severity of lesions of cystic medial necrosis were graded on the basis of 1 to 4 according to the amount of basophilic ground substance and fragmentation of elastic tissue. In most positive cases the lesion was of minimal (grade 1 or 2) seve...
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Abstract Unruptured aneurysms of the left sinus of Valsalva are extremely rare, and their clinical features are not well-documented in the literature. We describe an unusual case in which an unruptured aneurysm of the left sinus of Valsalva associated with previously undiagnosed Marfan syndrome caused extrinsic compression of the esophagus, resulting in dysphagia unrelated to the type or timing of meals. Our report focuses on the initial clinical presentation, as well as the diagnosis and manage...
Diagnosis of coronary artery dehiscence and pseudoaneurysm formation in postoperative Marfan patient by color flow Doppler echocardiography
The complementary role of magnetic resonance imaging, doppler echocardiography, and computed tomography in the diagnosis of dissecting thoracic aneurysms
Abstract Non-ECG gated MRI was compared with 2DE and/or CT scans in 10 patients with dissecting aneurysms proven by angiography and/or surgery. Patient ages ranged from 48 to 85 years (mean 69.6). Six had DeBakey type I dissections and four had DeBakey type III dissections. MRI was diagnostic for aortic dissection in nine cases and suggestive in the tenth. 2DE was diagnostic in six out of nine patients, suggestive in two patients, and nondiagnostic in one patient. CT was diagnostic in the three ...