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Aortic dissection in a young woman with Turner's syndrome.

Aortic dissection is an extremely rare occurrence often associated with fatal consequences. Among women suffering from Turner's syndrome, a mosaic of cardiovascular anomalies, some congenital, are often reported. Among these abnormalities, the conjunction of dilatation of the aorta with hypertension may lead to aortic dissection. A high level of clinical follow-up is necessary on a lifetime basis in order to diagnose such patients on time, which will allow preventive surgical intervention. Successive echocardiographic surveillance of these patients is recommended in addition to aggressive antihypertensive therapy in order to minimize potential morbidity and mortality as much as possible. If aortic dissection is diagnosed on time, surgical intervention can be lifesaving. In this communication, we report on a patient whose diagnosis was missed and consequently expired due to severe aortic dissection.

Journal Article↗

Transient paraplegia caused by acute aortic dissection--case report.

A case of acute aortic dissection (AAD) presenting as sudden, transient paraplegia and severe back pain is reported. The patient was a 66-year-old male with a 10-year-history of hypertension. The pain characteristically migrated from the back to the neck and then returned to the back. He showed complete transverse myelopathy at the level of the 9th thoracic cord. Computed tomography disclosed internal displacement of aortic intimal calcifications, without abnormalities in the spinal canal, and myelography showed no spinal canal block or stenosis. Electrocardiography and chest x-ray indicated nonspecific changes of high amplitudes and mild cardiomegaly, respectively. Together, these findings suggested acute aortic dissection with spinal cord ischemia. The initial systolic blood pressure of 220 mmHg was lowered with medication, and the pain was controlled with morphine. He recovered fully and was discharged 80 days after the onset of symptoms, with no neurological deficits. AAD carries a very poor prognosis unless treated immediately. Therefore, it is very important to promptly differentiate this disorder from spinal vascular conditions that also produce back pain and paraparesis.

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Accuracy of biplane and multiplane transesophageal echocardiography in diagnosis of typical acute aortic dissection and intramural hematoma.

OBJECTIVES: The purpose of this study was to evaluate the diagnostic accuracy of biplane and multiplane transesophageal echocardiography in patients with suspected aortic dissection, including intramural hematoma. BACKGROUND: Transesophageal echocardiography is a useful technique for rapid bedside evaluation of patients with suspected acute aortic dissection. The sensitivity of transesophageal echocardiography is high, but the diagnostic accuracy of biplane and multiplane transesophageal echocardiography for dissection and intramural hematoma is less well defined. METHODS: We studied 112 consecutive patients at a major referral center who had undergone biplane or multiplane transesophageal echocardiography to identify aortic dissection. The presence, absence and type of aortic dissection (type A or B, typical dissection or intramural hematoma) were confirmed by operation or autopsy in 60 patients and by other imaging techniques in all. The accuracy of transesophageal echocardiography for ancillary findings of aortic dissection (intimal flap, fenestration and thrombosis) was assessed in the 60 patients with available surgical data. RESULTS: Of the 112 patients, aortic dissection was present in 49 (44%); 10 of these had intramural hematoma (5 with and 5 without involvement of the ascending aorta). Of the remaining 63 patients without dissection, 33 (29%) had aortic aneurysm and 30 (27%) had neither dissection nor aneurysm. The overall sensitivity and specificity of transesophageal echocardiography for the presence of dissection were 98% and 95%, respectively. The specificity for type A and type B dissection was 97% and 99%, respectively. The sensitivity and specificity for intramural hematoma was 90% and 99%, respectively. The accuracy of transesophageal echocardiography for diagnosis of acute significant aortic regurgitation and pericardial tamponade was 100%. CONCLUSIONS: Biplane and multiplane transesophageal echocardiography are highly accurate for prospective identification of the presence and site of aortic dissection, its ancillary findings and major complications in a large series of patients with varied aortic pathology. Intramural hematoma carries a high complication rate and should be treated identically with aortic dissection.

Acute Disease↗

[A case of extended surgery in acute aortic dissection by perfusion into extra anatomic bypass].

Total aortic arch replacement creates various problems involving surgical techniques, myocardial protection and perfusion methods. The most important problem during this procedure is cerebral ischemia. We developed a technique to prevent this problem by bypass grafting through the right axillo-left external carotid-left axillary artery and perfusing the brain continuously through the side branch attached to this bypass graft. A 62-year-old man with acute aortic dissection underwent ascending aorta and total aortic arch replacement using this alterative perfusion method. No cerebral dysfunction was seen in this case. The advantages of this method are as follows: there is no time limit required even for total aortic arch replacement; adequate and reliable anastomosis is possible; the dissecting aortic branches are not touched directly; it is possible to prevent cerebral infarction caused by mural thrombus and pieces of atherosclerotic plaque by clamping the aorta and aortic arch branches; and it is possible to prevent air embolism.

Aortic Dissection↗

[CT evaluation of aortic dissection treated surgically].

A group of 23 patients with aortic dissection submitted for surgical operation was studied by CT (type I-10, type II-7, Type III-6 patients, according to DeBakey classification). In 9 (56.3%) patients with aortic dissection, type I and III, followed up for 3,5-10 years after surgical intervention the signs of aortic dissection were still visible, and 7 (43.7%) patients after 3-9 years developed thrombotic changes of various intensity in the false aortic lumen. In 2 cases a small external leakage and aneurysmal dilatation of the aorta around the implanted prosthesis were found. Five patients with type II dissection showed no signs of the dissection and other complications, whereas two patients developed aneurysmal dilatation of the aorta around the prosthesis.

Adult↗

Leaking giant aneurysm of the aortic root due to cystic medial necrosis with pericardial tamponade mimicking type-A aortic dissection.

The diagnosis of acute type-A aortic dissection is predominantly based on the demonstration of an intimal tear or a dissection membrane. We describe another pathogenetic mechanism in a patient with the typical features of acute aortic dissection with pericardial tamponade, and a giant aneurysm of the ascending aorta. However, no dissection membrane, rupture site, or intimal tear could be demonstrated by transesophageal echocardiography, intraoperatively, or histologically. The histological work-up showed an extreme form of cystic medial necrosis with intramural hemorrhages consistent with a leaking aneurysm. Hence, in a patient with a symptomatic aneurysm of the aortic root and pericardial tamponade, obvious intimal dissection or rupture does not always have to be present echocardiographically or intraoperatively. A different presentation can occur in the setting of an extreme medial necrosis, where blood leaks through the aortic wall causing intramural hemorrhages with intimal leaks invisible to the surgeon's or echocardiographer's eye. This process is clinically indistinguishable from type-A aortic dissection.

Aortic Dissection↗

Recurrent transient ischemic attacks as the initial presenting manifestation of type A aortic dissection.

A case is reported of an 84-year-old woman with recurrent episodes of aphasia and right-sided weakness with spontaneous complete resolution associated with hypotension. She subsequently developed lower gastrointestinal bleeding, ischemic toes, and anuria without associated chest pain. An emergent transesophageal echocardiography showed a type A aortic dissection with aortic valve insufficiency and tamponade. Emergent thoracotomy was performed, which confirmed a severe acute type A aortic dissection. This is a reported case of acute painless aortic dissection presenting initially as recurrent transient ischemic attacks. The etiology of focal neurologic deficits should be crucially determined by an experienced clinician prior to thrombolytic administration. This case illustrates the importance of aortic disease in the etiology of acute stroke syndromes.

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Aortic dissection: pathophysiology, diagnosis, and acute care management.

Acute aortic dissection is a life-threatening illness requiring early diagnosis and treatment. Management of this disease incorporates not only emergency department personnel, but also the medical, surgical, and intensive care specialties. If left untreated, the mortality rate of acute aortic disease exceeds 50% within 48 hours and 80% within 2 weeks, with a 5-year survival rate of 19%. The most common cause of death in untreated acute aortic dissection, regardless of etiology, is aortic rupture. In this article, the author reviews: the incidence and pathology; etiology; clinical manifestations; diagnostic workup; patient care management.

Acute Disease↗

Role of aortic root motion in the pathogenesis of aortic dissection.

BACKGROUND: The downward movement of the aortic root during the cardiac cycle may be responsible for producing the circumferential tear observed in aortic dissections. METHODS AND RESULTS: Contrast injections were investigated in 40 cardiac patients, and a finite element model of the aortic root, arch, and branches of the arch was built to assess the influence of aortic root displacement and pressure on the aortic wall stress. The axial displacement of the aortic root ranged from 0 to 14 mm. It was increased in patients with aortic insufficiency (22+/-13% of the sino-tubular junction diameter versus 12+/-9%) and reduced in patients with hypokinesis of the left ventricle (10+/-9% of sino-tubular junction versus 17+/-12%). The largest stress increase due to aortic root displacement was found approximately 2 cm above the sino-tubular junction, where the longitudinal stress increased by 50% to 0.32 Nmm(-2) when 8.9-mm axial displacement was applied in addition to 120-mm Hg luminal pressure. A similar result was observed when the pressure load was increased to 180 mm Hg without axial displacement. CONCLUSIONS: Both aortic root displacement and hypertension significantly increase the longitudinal stress in the ascending aorta. For patients with hypertension who are at risk of dissection, aortic root movement may be monitored as an important risk factor.

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[Endografting for refractory aortic dissection and dissecting aneurysm].

OBJECTIVE: To explore the safety and feasibility of endograft repair in refractory aortic dissection and dissecting aneurysm. METHODS: The clinical data of 13 refractory cases of aortic dissection and dissecting aneurysm, 11 males and 2 females, aged 52 (38-82), out of the 68 consecutive patients with aortic dissection and dissecting aneurysm who underwent endograft repair from Jan 2001 to Oct 2004, were analyzed. Nine of the 13 cases were diagnoses as with aortic dissection and dissecting aneurysm of Stanford type A, 3 of which had tears in the ascending aorta (DeBakey type I), 3 had tears in aortic arch, and 3 had tears on the distal aortic arch with ascending aorta involved; and the other four out of the 13 cases were diagnosed as Stanford type B, one with Marfan's syndrome. Eight of the 13 cases had more than 2 entries, 3 of which had suffered from shock and hemathorax due to rupture preoperatively. Follow-up ranging 2 months to 3 years was carried out after the operation. RESULTS: Transluminal placement of stentgratf was technically successful in all patients. Three patients with DeBakey type I dissections received stent-grafts introduced through the left common carotid or right femoral artery, of which one case died from gastrointestinal hemorrhage 1 month postoperatively. For the patients with tears in aortic arch preliminary Y type bypass from ascending aorta to left common carotid artery and left subclivian artery was performed in 2 cases, and carotid-carotid artery bypass was performed in one case, and then stent-grafts were deployed through right femoral artery. The 3 cases with tears in the distal arch and ruptured aneurysm, received stent-grafts implantation through the femoral artery emergently, and closed thoracic drainage and anti-shock treatment, one of which died from another aneurysm rupture 27 hours postoperatively. Out of the 4 cases with Stanford type B dissection with multiple tear entries, 3 underwent multiple stent-grafts repair and 1 underwent emergent abdominal-bilateral iliac arteries Y-type graft bypass due to rupture of iliac dissecting aneurysm. During the follow-up CT, MRI and color Doppler sonography showed that all 11 patients remained healthy with the former tears well closed and thrombosis in the false lumen. CONCLUSION: Endograft repair for refractory aortic dissecting aneurysm is feasible and technically successful, especially for the patients with tears in the ascending aorta or aortic arch.

Adult↗

Imaging of aortic dissection by helical computed tomography (CT).

Aortic dissection is the most frequent cause of aortic emergency, and its outcome is still frequently fatal. The management of this pathology has changed with the development of endovascular means. Nowadays, imaging modalities are helpful in management decision-making by providing information such as identification of entry tears along the aorta and involvement of the visceral branches of the abdominal aorta. Multi-slice CT scanning now appears to be the modality of choice for complete examination of the entire aorta. We review the parameters of image acquisition and contrast injection; appearances on CT of acute and chronic dissection are illustrated. Diagnostic pitfalls in CT imaging of acute dissection are discussed. Imaging of the post-surgical aorta and of chronic dissection is outlined. Intra-mural hematoma and penetrating aortic ulcer are subtypes of aortic dissection, and their appearances on CT scanning are also presented.

Aortic Dissection↗

Pulmonary artery obstruction caused by thoracic aortic dissection: a case with unique pathological findings.

Pulmonary artery obstruction is a rare complication of acute thoracic aortic dissection. A 74-year-old woman was admitted to hospital with respiratory distress. Computed tomography scan showed right pulmonary artery occlusion and a thoracic aortic dissection of the Stanford A type. Lung perfusion scan revealed a defect in the entire right lung field. These findings mimicked acute pulmonary thromboembolism accompanying aortic dissection. On the other hand, pulmonary angiography revealed a round smooth defect of the right pulmonary artery, indicating an extrinsic compression. The patient finally died of cardiac tamponade. Autopsy disclosed that the right pulmonary artery was compressed by a hematoma in the adventitial space around the pulmonary artery. Such a mechanism of pulmonary artery obstruction caused by acute aortic dissection is unique, and is distinct from that caused by chronic non-dissecting aortic aneurysms, which themselves compress the pulmonary arteries. This complication inevitably follows aortic rupture; therefore, emergency operation to repair the dissected aorta must be performed to avoid the following catastrophic event.

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[Could apoptosis be contributed to the occurrence of aortic dissection?].

This clinical study was conducted to determine whether apoptosis is contributed to the occurrence of aortic dissection. The subjects comprised 11 patients who underwent Stanford type A aortic dissection and 4 autopsy cases, being the control group. The occurrence of apoptosis was determined by the TUNEL assay using an aortic wall specimen, and the distribution of macrophages and h-MMP-9 was examined by immunohistological staining. Apoptotic cells were observed in the aortic specimens for all of the 11 patients who underwent Standford type A aortic dissection, but not in any of the 4 autopsy cases. Moreover, apoptotic cells were present in large numbers on the surface of the false lumen strongly in 8 patients who underwent surgery within 1 month after aortic dissection, but not in 3 who underwent treatment after than 1 month. Those phenomena were also observed in the immunohistological staining of CD 68 and h-MMP-9. These findings indicate that apoptosis could be contributed to the occurrence of aortic dissection.

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Durability of aortic valve preservation and root reconstruction in acute type A aortic dissection.

BACKGROUND: The aim of this study was to determine the durability of aortic valve preservation and root reconstruction in type A aortic dissection with involvement of the aortic root. METHODS: From November 1976 to February 1999, 246 patients underwent surgical treatment for acute type A aortic dissection at our institution. In 121 patients (49%), all with acute type A dissection and aortic root involvement, the aortic valve was preserved and one or more of the sinuses of Valsalva were reconstructed. The mean age of this group was 59 +/- 11 years and 70 (58%) were men. Thirty patients (25%) were operated in cardiogenic shock. Criteria for aortic root reconstruction were technical feasibility and surgeon preference. Techniques used for reconstruction were valve resuspension in all patients and additional reinforcement of the aortic root with Teflon (L.R. Bard, Tempe, AZ) felt (n = 21), gelatin-resorcinol-formaldehyde-glue (GRF-glue, Fii, Saint-Just-Malmont, France) (n = 103), or fibrinous glue (Tissu-col, Immuno AG, Vienna, Austria) (n = 5). Mean follow-up was 43.5 +/- 46 months. RESULTS: The operative mortality was 21.5% (n = 26). Actuarial survival was 72% +/- 4%, 64% +/- 5%, and 53% +/- 6% at 1, 5, and 10 years, respectively. Median aortic regurgitation in patients with retained native aortic valve at follow-up was 1+. All root reoperations included aortic valve replacement (n = 12). Freedom from aortic root reoperation was 95% +/- 2% at 1 year, 89% +/- 4% at 5 years, and 69% +/- 9% at 10 years. The incidence of aortic root reoperation was 23%, 11%, and 40%, respectively, when Teflon felt, GRF-glue, and fibrinous glue were used for root reconstruction. Multivariate Cox proportional hazard analysis revealed the use of fibrinous glue (RR = 8.7; p = 0.03) as well as the presence of an aortic valve annulus more than 27 mm (RR = 4.2; p = 0.04) as independent risk factors for aortic root reoperation. CONCLUSIONS: Aortic valve preservation in acute type A dissection provides relatively durable results. The use of fibrinous glue for root reconstruction seems to compromise the long-term durability of the repair compared with Teflon felt and GRF-glue. A dilated aortic annulus requires a more extensive root procedure.

Actuarial Analysis↗

Retrograde aortic dissection with bilateral renal artery occlusion after repair of infrarenal aortic aneurysm.

After aortic aneurysmectomy, early postoperative anuric renal failure developed when a retrograde aortic dissection occluded both renal arteries in a 71-year-old woman. Preoperative renal function was retrieved by use of prompt angiography and reconstruction. We are aware of no other reports describing retrograde aortic and bilateral renal artery dissection after aortic aneurysm repair. The case is presented to call attention to this unusual complication and to provide a discussion of acute postoperative renal failure and recommendations for the management of the described complication.

Acute Kidney Injury↗

Emergency endovascular stent-grafting for life-threatening acute type B aortic dissections.

BACKGROUND: There is still a considerable controversy regarding optimal treatment for patients with acute type B aortic dissection. Patients with complicated disease are particularly challenging for cardiovascular treatment. Early surgery for acute dissections of the descending aorta with life-threatening complications is known to carry a high mortality. Endovascular stent grafting is developing as an alternative treatment mainly for chronic stages of type B aortic dissection. It is not clear whether endovascular stent grafting is safe and effective in emergency treatment of acute type B aortic dissection. METHODS: In 10 patients (7 men, 3 women; mean age, 59.2 years; range, 46 to 65 years), endovascular stent grafting was performed within 11.0 +/- 5.9 hours (range, 4 to 24 hours) of diagnosis of complications. Indications for acute intervention included contained rupture, hematothorax, life-threatening malperfusion, and refractory pain. Using a retrograde endovascular route after surgical exposure of the femoral artery, self-expanding stent prostheses consisting of polyester-covered Nitinol (Talent, World Medical; mean diameter, 40 +/- 4 mm; length, 10 cm) were placed into the descending aorta distal to the subclavian artery. Before discharge and on follow-up visits, imaging of the aorta was performed using computed tomography. RESULTS: In 9 of 10 patients (90%), the primary entry could be completely occluded with the endovascular stent. Early mortality was 20% (2 of 10): 1 patient died after disruption of the intimal layer distal to the stent, and 1 patient died in hemorrhagic shock after surgical fenestration of the abdominal aorta for persistent malperfusion. Three patients (30%) required consecutive surgical treatment: indications included acute development of retrograde type A aortic dissection, acute stent dislocation by fractured wires and secondary leakage, and late formation of an aneurysm of the descending aorta 6 months after endovascular stent grafting. There were no surgical or late deaths. CONCLUSIONS: Our experience provides some evidence that early mortality of life-threatening acute type B aortic dissection may be reduced by emergency endovascular stent grafting and that this form of treatment is a promising therapeutic option. Refinements, especially in stent design and application, may further improve the prognosis of patients in the life-threatening situation of complicated acute type B aortic dissection.

Acute Disease↗

CT findings in thoracic aortic dissection.

Computed tomography (CT) findings in 11 cases of aortic dissection were correlated with aortographic, surgical, or postmortem findings. In another two patients, the CT findings were considered diagnostic of aortic dissection but no further workup was performed. All the CT examinations were made before and after bolus injection of contrast. Many of the familiar angiographic signs were appreciated on CT, such as a thickened aortic wall, septum between two opacifying lumens, differential time density between the two lumens, and compression of the true lumen. No false-positive or false-negative studies were encountered. This experience suggests that CT is a reliable, noninvasive method for the diagnosis of aortic dissection.

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