Takayasu's aortitis with dissection in systemic lupus erythematosus.

A forty-seven-year-old Japanese woman under treatment for systemic lupus erythematosus (SLE), complained of severe back pain. Chest X-ray and MRI showed an aneurysmal dilatation of the ascending aorta. Subsequently an aortic replacement was performed. Microscopically, the resected aorta showed Takayasu's aortitis with chronic dissection. Both aortitis and dissection are rare events in SLE patients. To our knowledge, this is the first report of Takayasu's aortitis with dissection in a patient with SLE.     

Thrombosed dissection of the ascending aorta complicating extravasation.

This report presents 2 patients with thrombosed dissection of the ascending aorta complicating extravasation. The first case was an 85-year-old male admitted with shock due to cardiac tamponade. Plain computed tomography (CT) demonstrated a dilated ascending aorta without clear evidence of aortic dissection. The second case was a 77-year-old female presenting with shock, in whom an enhanced CT scan demonstrated a dilated ascending aorta and periaortic effusion. However, dissection of the distal ascending aorta was not identified in either case before emergency surgery. In case 1, soon after the bloody pericardial effusion was decompressed during the operation, bleeding from the ascending aorta occurred. A small intimal tear was found in the distal ascending aorta, and in each case the pseudolumen was filled with fresh thrombus. The ascending aorta was replaced. Each patient had an uneventful postoperative recovery. Based on this experience, it is suggested that patients with thrombosed ascending aortic dissection complicating extravasation should undergo early graft replacement.     

Acute type A aortic dissection in a patient with systemic lupus erythematosus

The case of a 27-year-old Japanese woman with type A acute aortic dissection who had been diagnosed with systemic lupus erythematosus (SLE) is presented. The patient also had aortic regurgitation due to non-infective endocarditis and systemic hypertension, and had been maintained on steroid therapy for 15 years. Her twin sister was also diagnosed with SLE. The patient was admitted to emergency due to severe back pain. A chest x-ray showed enlargement of the upper mediastinum. Echocardiography revealed a thickened and deformed aortic valve with aortic regurgitation and dissection of the ascending aorta, but pericardial effusion was not found. Computed tomography demonstrated aortic dissection extending from the ascending aorta to the abdominal aorta. Graft replacement of the ascending aorta and proximal aortic arch was performed under hypothermic circulatory arrest with retrograde cerebral perfusion. The patient recovered uneventfully. Aortic dissection complicated with SLE is extremely rare, and this is only the 15th case reported in the English or Japanese literature.     

Ascending aortic aneurysm with aortic valve incompetence--surgical management

Aneurysm of ascending aorta with aortic valve incompetence is a formidable pathological entity having a relentless course. Etiological factors include syphilitic aortitis, dissection of aorta, Marfan's syndrome and similar conditions. With the advance in operative technique and extra corporeal circulation, now it is possible to treat these conditions by surgery. Surgical intervention includes replacement of entire ascending aorta along with aortic valve by an artificial valve conduit. Basically, two procedures are described, namely, the conventional method and Bentall's operation, both involving a great technique challenge. Our experience with both these techniques is being described in four consecutive cases in the last one year.     

Spontaneous rupture of the ascending aorta. A surgically treated case

Rupture of the ascending aorta may follow thoracic trauma or complicate an aortic aneurysm or acute dissection. It is otherwise extremely rare. The authors report a case of spontaneous rupture of the ascending aorta occurring in a patient with a pre-existing incomplete rupture of the ascending aorta, and treated surgically. The clinical presentation was of acute dissection with pericardial effusion. This diagnosis was excluded by aortography with multiple views which showed abnormalities of the aortic wall: an abnormal notch, continuity of the internal wall and extravasation of the contrast medium. These abnormalities are often minimal but should be recognised and surgery proposed as this is the only chance of a favourable outcome.     

Spontaneous rupture of the ascending aorta: case report and review.

Spontaneous nontraumatic rupture of the ascending aorta occurred in a hypertensive patient. The clinical findings suggested acute aortic dissection, and echocardiography showed a large pericardial effusion. Computed tomography scanning did not indicate aortic dissection, but aortography in 3 projections revealed an area of intimal disruption similar to the niche of an ulcer. The patient underwent replacement of the ascending aorta and proximal aortic arch, and the postoperative course was uneventful.     

Aortitis

Inflammatory or noninfectious aortitis may be idiopathic or it may be part of a systemic autoimmune disease, such as Takayasu's arteritis, Beh?et's disease, or giant cell arteritis. At the acute stage, there is thickening of the aortic wall with dilatation of the aorta, more commonly in the thoracic aorta. If it involves the aortic root, there may be annuloaortic ectasia or aortic regurgitation. At a later stage, there may be aneurysmal dilatation of the aorta and rarely dissection or rupture of the aorta. In Takayasu's arteritis, stenosing lesions can occur as well as aneurysmal dilatation of the aorta or arteries. Stenosing lesions may be treated with angioplasty with or without stenting, whereas aneurysmal dilatation of the aorta is treated by aneurys-mectomy with arterial reconstruction or conduit. Severe aortic regurgitation may require aortic valve surgery with or without replacement of the ascending aorta. Irrespective of the interventional procedure undertaken as appropriate for the lesion, control of inflammation with steroid therapy with or without other immunosuppressive agents is of paramount importance. Otherwise, prosthetic valve or graft dehiscence may occur after aortic surgery, and restenosis rate is also higher after percutaneous transluminal angioplasty or stenting.     

Coronary artery aneurysm associated with aortitis syndrome diagnosed pre- and intraoperatively

Aneurysm of the coronary artery associated with aortitis syndrome is extremely rare. This is a case report of a left coronary artery aneurysm associated with aortitis syndrome. The patient was a 47-year-old woman who complained of palpitation and dizziness on exertion. Aortogram revealed occlusion of the arch vessels, infrarenal aortic stenosis, and a fusiform aneurysm of the left coronary artery. There was neither an intraluminal thrombus nor occlusive changes in the coronary arteries. Severe systolic hypertension nearing 300 mmHg was present in the ascending aorta. Severe hypertension and direct extension of inflammation to the coronary artery seemed to be important factors for the formation of the aneurysm. To resolve the severe hypertension, a bypass operation between the ascending aorta and the abdominal aorta distal to the stenosis was performed. The postoperative course was uneventful and blood pressure was reduced.     

Aortite et complications aortiques de l’artérite à cellules géantes (maladie de Horton)

Aortitis is a serious complication of giant cell arteritis (GCA), because of the risk of aortic aneurism, rupture, or dissection. Aortitis is present either at presentation or, more frequently, occurs as a delayed complication, typically as an aortic aneurism of the ascending part of the aorta. An aortic aneurism may occur in up to 10% of patients. Aortitis is sometimes associated to arteritis of the supra-aortic vessels. Risk factors for aortitis remain unknown. Recent clinical studies indicate that prevalence of aortitis was initially under-estimated. Imaging studies show signs of infra-clinical aortitis in 20 to 65% of cases at diagnosis. Using ultrasonography, thickening of the vascular wall with an hypoechoic halo around the abdominal aorta is suggestive of abdominal aortitis. Positron emission tomography shows a metabolic hypersignal of the aorta in about 50% of patients with giant cell arteritis. Aortic computed tomographic (CT) scan visualizes aneurysmal dilatations, ectasia or focal or concentric parietal thickenings. When present at the time of diagnosis of GCA, these findings seem to be associated with frequent relapses and perhaps with a higher long-term vascular mortality rate. Therefore, we recommend the screening of aortitis lesions at GCA diagnosis by an aortic CT-scan and follow-up. Therapeutic trials should be conducted to try to improve the treatment of aortitis in GCA.     

Immunoglobulin G4-Related Aortitis and Severe Aortic Valve Stenosis Treated With Transcatheter Aortic Valve Replacement and Immunosuppression

IgG4-related disease is an immune-mediated fibro-inflammatory disorder with multisystemic involvement. Aortitis and peri-aortitis are the most common cardiovascular manifestations of the disease. We present the case of a 65-year-old man with symptomatic severe aortic stenosis and concomitant IgG4 aortitis. The diagnosis was confirmed by IgG4 serum levels, positive positron emission computed tomography (PET-CT) scans, and pathology from mediastinal dissection. Surgical aortic valve replacement (SAVR) was unfeasible owing to extensive mediastinal fibrosis, and transcatheter aortic valve replacement (TAVR) was successfully performed. As ascending aorta access for SAVR in IgG4 aortitis with long-run fibrosis entails a high risk of mortality, TAVR could be considered in certain suitable patients.     

Diagnostic and surgical management of patients with aneurysms of the thoracic aorta with various causes. Echocardiography and contrast enhanced computed tomography in prophylactic replacement of the ascending aorta

Sixty eight patients with aneurysms of the thoracic aorta were studied. Forty one had aortic dissection, 24 had dilatation only, and three had transverse aortic rupture. Sixteen had Marfan's syndrome; 17 had hypertension; and in eight there were other causal factors. In 17 the cause of the aneurysm was unknown. Histological examination did not help to establish the cause of aneurysm. Echocardiography failed to detect dissection of the ascending aorta in four (21%) out of 19 cases studied. The mortality rate in the whole series was 26%. Early (operative and hospital) and late deaths occurred in 20% and 6% of patients respectively. The early mortality rate was 40% in the 24 emergency cases of dissection of the ascending aorta, 9% in patients operated on for dilatation of the ascending and transverse aorta without dissection, and 8% in patients with chronic dissection of the ascending aorta who had elective operation. Early and late mortality rates were no higher in patients with Marfan's disease than in any of the other groups. It is suggested that contrast enhanced computer tomography should be performed in all patients with pronounced aortic root dilatation and in patients with Marfan's disease with symptoms which suggest dissection, even if they have only slight aortic root dilatation. Preventive replacement of the ascending aorta should be considered in more patients to reduce the number of emergency operations, in which the mortality rate is high. There is no definite limit of aortic root dilatation above which preventive replacement of the ascending aorta should be routinely considered.     

Diagnostic and surgical management of patients with aneurysms of the thoracic aorta with various causes. Echocardiography and contrast enhanced computed tomography in prophylactic replacement of the ascending aorta.

Sixty eight patients with aneurysms of the thoracic aorta were studied. Forty one had aortic dissection, 24 had dilatation only, and three had transverse aortic rupture. Sixteen had Marfan's syndrome; 17 had hypertension; and in eight there were other causal factors. In 17 the cause of the aneurysm was unknown. Histological examination did not help to establish the cause of aneurysm. Echocardiography failed to detect dissection of the ascending aorta in four (21%) out of 19 cases studied. The mortality rate in the whole series was 26%. Early (operative and hospital) and late deaths occurred in 20% and 6% of patients respectively. The early mortality rate was 40% in the 24 emergency cases of dissection of the ascending aorta, 9% in patients operated on for dilatation of the ascending and transverse aorta without dissection, and 8% in patients with chronic dissection of the ascending aorta who had elective operation. Early and late mortality rates were no higher in patients with Marfan's disease than in any of the other groups. It is suggested that contrast enhanced computer tomography should be performed in all patients with pronounced aortic root dilatation and in patients with Marfan's disease with symptoms which suggest dissection, even if they have only slight aortic root dilatation. Preventive replacement of the ascending aorta should be considered in more patients to reduce the number of emergency operations, in which the mortality rate is high. There is no definite limit of aortic root dilatation above which preventive replacement of the ascending aorta should be routinely considered.     

Takayasu's arteritis revealed by aortic regurgitation with ascending aortic aneurysm (About 2 cases)

Aortic regurgitation caused by non-specific aortitis is relatively rare, and is now considered as an important risk factor related to mortality. Aortic valve replacement surgery is the only curative treatment. Aneurismal dilatation of the ascending thoracic aorta associated with aortic regurgitation is a rare involvement in Takayasu, there are many difficult problems in surgical treatment of this lesion, because of its inflammatory nature, so steroid therapy before and after surgery is therefore vital. We report the cases of tow young Moroccans women (32-35 years-old) with an ascending aortic aneurism associated to aortic insufficiency. The subsequent evaluation of the entire aorta, demonstrated the presence of multiple steno-occlusive lesions. Aortic valve replacement was performed associated with graft replacement of the ascending aorta without coronary artery reimplantation. Histopathological examination of the ascending aorta and aortic valve, showed findings in favour Takayasu's arteritis.     

Aortic dissection

Aortic dissection is a life-threatening cardiovascular emergency requiring immediate diagnosis and treatment. It is mainly associated with hypertension and the Marfan syndrome. Diagnosis has been revolutionized by the use of transesophageal echocardiography (TEE), which allows for rapid and safe assessment of this condition. Echocardiographic hallmarks of aortic dissection ar the presence of a dissection membrane separating a true from a false lumen, rupture sites in the membrane with to-and-from flow, aortic regurgitation, and pericardial effusion. Dissection of the ascending aorta, which has an extremely high mortality and necessitates emergency surgery, is diagnosed quickly and accurately by TEE.     

Aortic valve replacement and ascending aorta replacement in ankylosing spondylitis: report of three surgical cases and review of the literature

Out of 887 consecutive patients who underwent aortic valve replacement between January 1976 and December 1981 at Hannover Medical School Hospital, 3 patients had severe aortic valve insufficiency associated with ankylosing spondylitis (Morbus Bechterew). One of them had huge aneurysmatic dilatation of the ascending aorta and successfully underwent replacement of the ascending aorta by a vascular prosthesis. Microscopical examination of the resected aortic wall showed characteristic findings of aortitis in ankylosing spondylitis. The 3 patients are in good clinical condition at 5 and 6 months, and 2 1/2 years, respectively, after uneventful surgery. It is concluded that aortic valve replacement in patients with ankylosing spondylitis can be performed feasibly and clinical results have been satisfactory. The risk of aneurysmatic dilatation of the ascending aorta resulting from aortitis associated with ankylosing spondylitis is emphasized.     

Predictors of in-hospital mortality in patients with acute aortic dissection

BACKGROUND: If acute aortic dissection is a highly lethal disease. There were few reports addressing predictors of in-hospital mortality of this disease in southern Taiwan. METHODS: If from January 1, 1989, to December 31, 2001, patients with acute aortic dissection were enrolled. Patient demographics, history, clinical characteristics, and laboratory examinations were reviewed. Univariate testing followed by logistic regression analysis was performed to identify the predictors of in-hospital mortality. RESULTS: If in total, 198 (146 male) patients with mean age of 60.7+/-11.6 years were enrolled. The in-hospital mortality rate was 34.8% in overall patients, 58.8% for type A dissection, and 14.8% for type B dissection. There were five independent predictors of in-hospital mortality: presence of hypertension [odds ratio (OR)=0.09, 95% confidence interval (CI)=0.02-0.36, p<0.001], type A dissection (OR=8.26, 95% CI=3.44-19.60, p<0.001), probable extravasation (pericardial effusion in type A dissection or left side pleural effusion in patients with involvement of descending thoracic aorta) (OR=2.70, 95% CI=1.14-6.41, p=0.024), visible intimal flap in ascending aorta in trans-thoracic echocardiography (OR=4.46, 95% CI=1.58-12.60, p=0.005), and acute renal deterioration (OR=3.85, 95% CI=1.36-10.87, p=0.011). CONCLUSIONS: If acute aortic dissection, especially type A, is with high mortality in southern Taiwan. There are five independent predictors of in-hospital mortality found in current analysis. Our result may remind doctors to find out their patients at high risk. Trans-thoracic echocardiography is a useful tool to find out patients at high risk because it is easily performed to check if there is pericardial effusion or visible intimal flap in ascending aorta.     

IgG4‐related systemic disease and lymphoplasmacytic aortitis

We describe herein a patient who developed a dissection of the ascending aorta in the setting of IgG4‐related systemic disease, linking IgG4‐related systemic disease with a newly‐recognized subset of noninfectious aortitis. At the time of aortic surgery, a transmural lymphoplasmacytic infiltrate was detected in the patient's aorta, with a principal focus of inflammation within the media. Immunohistochemical studies demonstrated that >50% of the plasma cells in the lesion stained for IgG4. By in situ hybridization, the plasma cells showed polytypic staining for kappa and lambda light chains, consistent with a polyclonal plasma cell infiltrate. Serologic evaluation revealed that the patient's IgG4 levels were elevated nearly 10‐fold. Four years before aortic surgery, the patient had undergone a mediastinal lymph node biopsy. Reexamination of the lymph node revealed features consistent with IgG4‐related systemic disease, which had not been recognized at the time of the original biopsy. Glucocorticoid therapy for the IgG4‐related systemic disease yielded a prompt response. Recognition that IgG4‐related systemic disease can involve the ascending as well as the descending abdominal aorta indicates the need for a change in the way idiopathic aortitis is regarded. This case offers new potential considerations for short‐ and long‐term management of noninfectious aortitis, because of the frequent good response of IgG4‐related systemic disease to glucocorticoid treatment without additional therapy. Treatment of the aortitis may prevent progression of the IgG4‐related systemic disease to involvement of other organs. IgG4‐related systemic disease should be considered in all patients with aortitis judged to be of unknown etiology.     

Painless left hemorrhagic pleural effusion: An unusual presentation of dissecting ascending aortic aneurysm

Aortic dissection is a catastrophic event that is commonly associated with severe pain, massive hemorrhage, and high mortality. In this report, we present the case of a 31-year-old man who presented with painless, hemorrhagic left pleural effusion. Further investigation revealed a 9-cm dissecting ascending aortic aneurysm that was thought to be due to a congenitally bicuspid aortic valve. We suggest that ascending aortic aneurysm be included in the differential diagnosis of hemorrhagic pleural effusion, even in the absence of the classic features of aortic dissection, such as chest pain, advanced age, or history of hypertension.     

Hématome intramural de l’aorte thoracique : traitement médical ou chirurgie ?

Intramural hematoma (IMH) of the aorta is an uncommon entity. This disease shares many characteristics with acute aortic dissection. Treatment of IMH remains controversial. We report the case of a 58 years old man with hypertension disease who was admitted in emergency department with suspicion of acute aortic dissection. Transoesophageal echocardiography showed IMH involving the descending aorta which spred afterwards to the ascending aorta. Patient was treated medically and echocardiographic follow-up showed that aortic hematoma remains stable. Two years later, patient is alive and the last TEE reveals disappearance of hematoma in ascending aorta and decrease of it in descending aorta.     

Diagnostic goals in aortic dissection. Value of transthoracic and transesophageal echocardiography]

The combination of different ultrasound techniques like transthoracic, suprasternal, subcostal and transesophageal echocardiography have a high sensitivity and specificity in the diagnosis of aortic dissection. The limitation of this combined ultrasound technique is related to the visualization of the ascending part of the aortic arch which, cause of the interposition of the trachea, can not be visualized completely. The beginning or the end of a dissection in this part of the aorta may be misinterpreted. False negative results are rare. False positive results due to artefacts resulting from reverberations in an ectatic ascending aorta have to be taken into account. The most important diagnostic aims in acute or chronic aortic dissection can be described: 1. confirmation of the diagnosis by visualization of the intima membrane, 2. the differentiation of the true and false lumen depending on visualization of spontaneous echocardiographic contrast thrombus formation, slow or reduced reversed flow, systolic diameter reduction and signs of entry jet into the false lumen, 3. detection of intimal tear, demonstrating communication by two-dimensional or color Doppler echocardiography, 4. determination of the extent of the dissection with classification according to DeBakey type I, II and III or Stanford A and B with differentiation to communicating or non-communicating dissection, antegrade or retrograde dissection limited to the descending aorta or expanding to the ascending aorta, 5. detection of wall motion abnormalities as a sign of preexisting coronary artery disease or myocardial ischemia due to ostium occlusion by an intimal flap, coronary artery rupture or collapse of the true lumen during diastole, 6. detection and grading of aortic insufficiency, 7. detection of side branch involvement by suprasternal, subcostal and abdominal sonography, which will gain the information which side can be chosen for cannulation or catheterization at the femoral artery, 8. detection of pericardial pleural effusion and mediastinal hematoma as a sign of emergency as rupture can occur within minutes. Without surgical intervention have be performed. Based on these informations, surgery can be performed in all acute situations in type A dissection without further investigations. This decision is particularly important in patients with signs of emergency like pericardial or pleural effusion or mediastinal hematoma.