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PubMed2006

Clinical and epidemiological description of aortic dissection in Turner's syndrome.

BACKGROUND: Women with Turner's syndrome have an increased risk of congenital cardiac malformations, ischaemic heart disease, hypertension and stroke. Aortic dissection seems to occur with increased frequency. AIM: To describe in more detail aortic dissection as encountered in Turner's syndrome, giving attention to clinical, histological and epidemiological aspects. MATERIALS AND METHODS: Based on a retrospective study, we describe the clinical, karyotypic, and epidemiological aspects of aortic dissection as encountered in cases of Turner's syndrome seen in Denmark and Sweden. RESULTS: The median age at onset of aortic dissection in 18 women was 35 years, ranging from 18 to 61 years. Fourteen of 18 women had a 45,X karyotype, while 2 patients had 45,X/45,XY, and 2 had the 45,X/46,X+r(X) complement, respectively. Echocardiography was performed in 10 of 18 patients before their acute illness, and showed signs of congenital cardiac disease, with either bifoliate aortic valves, dilation of the aortic root, or previous aortic coarctation evident in most patients. In 5 patients evidence of a bifoliate aortic valve was conclusive. Hypertension was present in 5 of 18 patients, while 10 of the patients died from aortic dissection, of so-called type A in 6, type B in 3, while in the final case the origin of dissection could not be determined. Biochemical analysis showed altered ratio between type I and type III collagen. Histology showed cystic medial necrosis in 3 of 7 cases. We estimated an incidence of dissection of 36 per 100,000 Turner's syndrome years, compared with an incidence of 6 per 100,000 in the general population, and a cumulated rate of incidence of 14, 73, 78, and 50 per 100,000 among 0-19, 20-29, 30-39, and 40+ year olds, respectively. CONCLUSION: Aortic dissection is extremely common in the setting of Turner's syndrome, and occurs early in life. Patients with Turner's syndrome should be offered a protocol for clinical follow-up similar to that provided for patients with Marfan syndrome, and each clinic should embrace a programme for follow-up.

Adolescent↗
PubMed1990

Surgical treatment of arch-type dissecting aortic aneurysms.

We have proposed calling dissecting aortic aneurysm with entry of the dissection in the aortic arch "arch-type". We described the procedures and results of surgery for 12 cases of this disorder. The dissection was localized (type a) in six cases, and extended (type b) in the other six. Seven cases were treated by patch or direct closure of the entry, with one operative death and two later deaths. Five cases were treated by reconstruction of the ascending aorta and the aortic arch with a prosthetic graft, with two operative deaths and one later death. Efforts must be directed towards improving the outcome of the latter operation.

Adult↗
PubMed2002

Stented elephant trunk procedure combined with ascending aorta and arch replacement for acute type A aortic dissection.

OBJECTIVES: Despite steadily improving outcomes, surgery for acute type A aortic dissection has several unresolved problems such as expansion of the residual false lumen in the descending aorta. We performed transaortic stented graft implantation into the descending aorta combined with the ascending aorta and aortic arch replacement for acute type A aortic dissection. We review the efficacy and outcomes of this procedure with respect to the residual false lumen and postoperative neurologic complications we encountered. METHODS: Nine consecutive patients with acute type A aortic dissection underwent this procedure. The stented elephant trunk graft was implanted through the aortic arch under hypothermic circulatory arrest. The stented graft was 15 cm long in six patients, and 10 cm long in three patients. Enhanced computed tomography (CT) was performed 1 month after surgery and once each year after discharge to evaluate the postoperative time course of the residual false lumen. RESULTS: Cardiopulmonary bypass (CPB) time was quite long because of slow cooling and re-warming [352+/-92 (mean+/-SD) min], and average lower-body arrest time was 54+/-10min. The intima in one patient was injured at the time of implantation, and a small leak was created. One patient died of multiorgan failure postoperatively. One patient suffered cerebral injury, and two suffered spinal cord injury perioperatively. Average follow-up time was 40.4 months (range, 13-66 months). One patient died of cerebral infarction during follow up, and the other seven survived and remain well. Postoperative enhanced CT scans showed that the dissected descending aortas attached to the stented grafts and the aortas near the stented grafts returned to normal. In one patient with no re-entry, the false lumen completely closed with thrombi and the entire aorta returned to normal. The diameter of the descending aorta decreased or did not change in six of the seven patients (85.8%) and increased by only 2mm in one of them (14.2%) during follow up. CONCLUSIONS: Implantation of a stented elephant trunk into the descending aorta combined with replacement of the ascending aorta and total arch for acute type A aortic dissection is effective in closing the residual false lumen of the descending aorta and in preventing expansion of the descending aorta. However, further technical modifications, such as using a short stented elephant trunk, eliminating aortic clamping, shortening CPB and spinal cord ischemic time, and reconstruction of left subclavian artery, are needed to prevent neurologic complications.

Acute Disease↗
PubMed2002

Transluminal stent-graft placements for the treatments of acute onset and chronic aortic dissections.

BACKGROUND: Transluminal stent-graft placement (TSGP) for aortic dissection is a relatively new procedure. We performed TSGPs to seal the primary entry site to treat and prevent complications of aortic dissection. The early to mid-term outcomes were analyzed. METHODS AND RESULTS: Thirty-seven patients with a primary intimal tear in descending aorta underwent TSPG. TSGP was performed in 16 acute onset dissections (AOD) with dissection-related complications instead of emergency surgery. Eight AOD without complications were treated to prevent aneurysmal enlargement. Thirteen chronic dissections were treated to prevent rupture. TSGP was technically successful in all cases. One patient with prehospital rupture died. The hospital mortality rate was of 2.7% overall, 6.3% in AOD with complications, 0% in AOD without complications and in chronic dissections. One persistent endoleak required open surgery, and 1 intimal tear was caused by the stent-graft, necessitating an additional TSGP. The primary success rate was 94.4% overall. After hospital discharge, no patient died or suffered aortic rupture during an average follow-up of 24.5 months. New intimal tears caused by the stent-grafts and a secondary endoleak developed in 3 AOD patients. One open procedure and 2 additional TSGPs were performed. Actuarial survival rate and cardiovascular event-free rate at 2 years are 97.3% and 78.3% overall, 93.8% and 48.0% in AOD with complications, 100% and 87.5% in AOD without complications, and both 100% in chronic dissections. CONCLUSIONS: TSGP is a reasonable treatment option for aortic dissection. However, delayed intimal tear formations caused by the stent-graft is a problem that requires further investigation.

Acute Disease↗
PubMed1988

Painless aortic dissection presenting as spinal cord ischemia.

A 67-year-old woman developed a transient paraplegia as the initial manifestation of acute aortic dissection. Chest or abdominal pain was not present. Magnetic resonance imaging of the thorax demonstrated dissection of the aortic root, aortic arch, and descending aorta as well as pericardial effusion. Ultrasonography of the abdomen revealed an intimal flap in the region of the artery of Adamkiewicz. During aortic dissection, temporary obstruction of spinal arteries may result in transient paraplegia. Painless aortic dissection should be considered in patients who present with transient myelopathy.

Aged↗
PubMed1995

Aortic dissection: rupture into right ventricle and right pulmonary artery.

Rupture of an acute ascending aortic dissection into a surrounding cardiac chamber or pulmonary artery is an uncommon occurrence, and is often only diagnoses post mortem. Although fistulization (aortopulmonary and aorta-right atrial) after acute aortic dissection has been well documented in the literature, acute aortic dissection fistulizing into both the right ventricle and pulmonary artery has not. We report on a 75-year-old woman who presented with an acute ascending aortic dissection with both aortopulmonary and aorta-right ventricular fistulas who underwent repair and had long-term survival.

Aged↗
PubMed1994

[Dissecting aortic aneurysms that occurred in aged brothers without Marfan syndrome].

The occurrence of familial dissecting aortic aneurysms without Marfan syndrome is rare. This report describes two aged siblings who underwent successful surgery for this disease. Neither patient had the clinical features of Marfan syndrome, and both had histories of systemic hypertension. Case 1: A 76-year-old man had dissecting aortic aneurysm, DeBakey type IIIa, which was localized and had multiple lumina with thrombi. Case 2: A 71-year-old man (younger brother of case 1) had retrograde dissecting aortic aneurysm, DeBakey type IIIa. Histological examinations of the aortae showed cystic medial necrosis in both cases, and also showed atherosclerotic changes in case 1. These two siblings had no familial history of cardiovascular incidents and were much older than previously reported cases. Therefore it may be possible that the dissecting aortic aneurysms of these two cases occurred coincidently rather than due to hereditary factors.

Aged↗
PubMed1996

[Retrograde aortic dissection during cardiopulmonary bypass a case report successfully treated by contralateral femoral cannulation].

Retrograde aortic dissection is the most critical complication associated with femoral perfusion. It may be related to the trauma of femoral cannulation per se or to the jet of retrograde perfusion. We report the successful repair of a retrograde aortic dissection by composite graft replacement of the dissected ascending aorta using contralateral femoral cannulation. A 66-year-old man presented with aortic regurgitation due to annuloaortic ectasia. At the operation, the right common femoral artery was cannulated, and cardiopulmonary bypass was initiated. Within a few minutes after beginning the bypass, we recognized a retrograde dissection showing a tense bluish bulge in the ascending aorta. Bleeding was detected from the dissected aortic root beside the right coronary ostium. The bypass was interrupted, and contralateral femoral cannulation was performed immediately to re-establish the bypass. Composite graft replacement of the aortic root and ascending aorta was successfully performed under hypothermic circulatory arrest using additional retrograde cerebral perfusion. Pathological findings of the aortic wall revealed cystic medionecrosis. When a retrograde aortic dissection occurs, it is essential to establish antegrade flow. If it is impossible to cannulate at the ascending aorta for any reason, a contralateral femoral cannulation can be used successfully to re-establish the cardiopulmonary bypass because of the easy access.

Aged↗
PubMed2006

Endovascular repair of acute type B aortic dissection: early and mid-term results.

OBJECTIVE: To evaluate early and mid-term results of endovascular repair of acute type B aortic dissection by stent graft. METHODS: From June 2001 to May 2005, 63 patients with acute type B aortic dissection underwent stent graft implantation. The study included 59 men and 4 women with an average age of 50.4 +/- 11.4 years (range, 31-80 years). Four patients underwent stent-graft implantation in the acute phase. Fifty-nine patients with acute type B dissection underwent stent-graft implantation 2 weeks after the onset of dissection. All patients were followed up from 1 to 47 months (average, 11.7 +/- 10.6 months). The clinical data of the patients were analyzed. RESULTS: The primary tear was incompletely sealed in three cases. The incidence of incomplete seal was 4.8%. Ascending aortic dissection occurred in three cases. One occurred during operation. The other two occurred at 1 day and 10 months after stent-graft implantation. Two patients died within 30 days after operation. One died of rupture of the ascending aortic dissection. The other one died of acute renal failure. The 1-month mortality was 3.2%. Four patients underwent a second stent-graft implantation before discharge. One year after stent-graft implantation, complete thrombosis of the false lumen in the thoracic aorta was achieved in 98.4% of patients, and the maximum diameter of the descending aorta decreased 11.2% +/- 7.3%. Three patients died within the follow-up time. Mortality during the follow-up period was 4.8%. One patient died of peptic ulcer hemorrhage. Another one died of rupture of the ascending aortic dissection. The third one died of unknown reasons. The actuarial survival curve by the Kaplan-Meier method showed a 4-year survival rate of 89.4%. CONCLUSIONS: Early and mid-term results showed that endovascular repair was effective in treatment of acute type B aortic dissection. With the enrichment of doctors' experience and refinement of the device, better results are expected in the future.

Acute Disease↗
PubMed2000

Epidemiology and clinicopathology of aortic dissection.

STUDY OBJECTIVES: To determine the incidence and mortality as well as to analyze the clinical and pathologic changes of aortic dissection. DESIGN AND SETTING: A population-based longitudinal study over 27 years on a study population of 106,500, including 66 hospitalized and 18 nonhospitalized consecutively observed patients. MEASURES: Analysis of data from the medical, surgical, and autopsy records of patients with aortic dissection. RESULTS: Altogether, 86 cases of aortic dissection were found in 84 patients, corresponding to a 2.9/100,000/yr incidence. Sixty-six of the 84 patients (79%) were admitted to the hospital, and 18 patients (21%) died before admission. Their ages ranged from 36 to 97 years, with a mean of 65. 7 years. The male/female ratio was 1.55 to 1. A total of 22.7% of the hospitalized patients died within the first 6 h, 33.3% within 12 h, 50% within 24 h, and 68.2% within the first 2 days after admission. Six patients were operated on, with a perioperative mortality of two of six patients and a 5-year survival of two of six patients. All patients who were not operated on died. Pain was the most frequent initial symptom. Every patient had some kind of cardiovascular and respiratory sign. Neurologic symptoms occurred in 28 of 66 patients (42%). Five patients presented with clinical pictures of acute abdomen and two with acute renal failure. Trunk arteries were affected in 33 of the 80 autopsied cases (41%), and rupture of aorta was seen in 69 cases (86%). In five cases, spontaneous healing of dissection was also found. The ratio of proximal/distal dissections was 5.1 to 1. All 18 prehospital cases were acute. Fifty-nine cases (89.4%) were acute at admission, and 7 cases (10.6%) were chronic dissections. Hypertension and advanced age were the major predisposing factors. CONCLUSION: Aortic dissection was the initial clinical impression in only 13 of the 84 patients (15%). Thus, 85% of the patients did not receive immediate appropriate medical treatment. For this reason, these late-recognized and/or unrecognized cases may be regarded as an untreated or symptomatically treated group, whose course may resemble the natural course of aortic dissection.

Adult↗
PubMed1991

Decrease in sulphated glycosaminoglycans in aortic dissection--possible role in the pathogenesis.

STUDY OBJECTIVE: The aim was to investigate alterations in sulphated glycosaminoglycans in aortic dissection. DESIGN: Aortic fragments were taken from 10 patients within the first 3 d after onset of symptoms of aortic dissection and from nine age matched patients with no aortic disease. Sulphated glycosaminoglycans were analysed and quantified by agarose gel electrophoresis and densitometry after degradation with specific enzymes. MEASUREMENTS AND MAIN RESULTS: The amount of chondroitin sulphate was similar (7.14 v 7.60 mg.g-1 of dry tissue, n = 10, p greater than 0.5) in patients with dissection and in the control group. Total sulphated glycosaminoglycan content was decreased (11.51 v 14.26 mg.g-1 of dry tissue, n = 10, p less than 0.001). This difference was due to heparan sulphate (1.79 v 2.48 mg.g-1 of dry tissue, n = 10, p less than 0.05) and mainly to dermatan sulphate (2.58 v 4.18 mg.g-1 of dry tissue, n = 10, p less than 0.001). The ratio of 6-/4-sulphated disaccharides after chondroitinase ABC digestion was increased in the affected group. No correlation between these biochemical results and a histological evaluation of mucoid content was found. On the other hand, a significant increase in chondroitin sulphate could be observed related to aging. CONCLUSIONS: The diminution in sulphated glycosaminoglycans and its possible relationship with fat, collagen, and other extracellular matrix molecules could lead to a weakness in the aortic wall related to the dissection.

Adult↗
PubMed1995

Aortic dissection in giant-cell arteritis.

Twenty-three cases of aortic dissection in patients with giant-cell arteritis are reviewed and an additional case is reported. Forty-six percent presented catastrophically with aortic dissection and no prior history of giant cell arteritis. Eighty percent died within 2 weeks of the event; four patients had successful surgical grafts. There was diffuse involvement of the aorta with giant cells in 89%, but dissecting tears occurred primarily in the proximal aorta in 85% of cases. The majority of cases with a preceding history of giant cell arteritis were on low doses of steroid or on no treatment at the time of dissection, and the median erythrocyte sedimentation rate of these patients was 62 mm/h (range 21-98). Evidence of some form of hypertension, whether acute or chronic, mild or severe, was found in 77% of patients. Inadequate treatment of giant-cell arteritis and underlying hypertension (treated or untreated) are potential factors leading to aortic dissection in these patients.

Aged↗
PubMed2004

Emergent endovascular stent-graft placement to treat ruptured Stanford type B acute aortic dissection.

Uncomplicated Stanford type B acute aortic dissection, for which medical treatments are effective in most cases, is associated with a better prognosis than Stanford type A dissection. However, ruptured Stanford type B dissection still is associated with high mortality, because of the risks of open surgery and the complications of the disease. We report successful stent-graft placement in two patients with acute type B dissection with rupture, and discuss the advantages of stent-graft placement to treat ruptured acute type B dissection.

Aortic Dissection↗
PubMed2005

Serum heart-type fatty acid-binding protein concentration positively correlates with the length of aortic dissection.

BACKGROUND: Patients with aortic dissection (AD) often demonstrate positive heart-type fatty acid-binding protein (H-FABP), but its significance is unclear. METHODS AND RESULTS: In 63 of 64 consecutive AD patients, the serum H-FABP concentration was measured and the H-FABP positive rate calculated (cutoff value: 6.2 ng/ml) for each of following factors: (1) with or without dissection of the ascending aorta; and (2) a thrombosed false lumen; (3) length score; (4) presence of shock; (5) malperfusion of limbs; (6) ST elevation and/or depression on electrocardiogram; and (7) renal dysfunction. In total 36 AD patients had a positive H-FABP test. Statistically significant differences in the H-FABP positive rate were observed between those with and without ascending AD (76.7% vs 39.4%, p = 0.003), and in the length score (p < 0.001). Multivariate logistic regression test demonstrated that the most significant factor was the length score (odds ratio: 2.239 (95% confidence interval: 1.119-4.481), p = 0.023). Moreover, a significant correlation was observed between length score and absolute H-FABP value (r = 0.420, p = 0.001). CONCLUSIONS: In patients with AD, an increased serum H-FABP concentration is caused by the protein being released not only from the cardiac muscle but also from skeletal muscle, or possibly the aortic wall. Physicians using H-FABP for detection of myocardial injury need to be aware that patients with a long or ascending AD will show an elevation of H-FABP.

Adult↗
PubMedDate not supplied

Spontaneous resolution of a descending aortic dissection. Report of a case.

The natural history of an aortic dissection is either endothelialization of the false lumen forming a so-called double-barrelled aorta, or thrombosis of the sack leading to fibrosis and scarring. Complete healing of an aortic dissection is extremely rare, and has to our knowledge only been reported once in vivo. Here we report a second case of spontaneous resolution of an aortic dissection, disclosed by contrast medium enhanced computed tomography.

Aged↗
PubMed2003

Stanford type A aortic dissection which ruptured into the left atrium: report of a case.

A Stanford type A aortic dissection ruptures usually into the pericardial space or the mediastinal space. We herein report the rare surgical case of a Stanford type A aortic dissection which ruptured into the left atrium. The patient had a previous history of mitral valve replacement. The time and the cause of the aortic dissection was unclear. At operation, adhesions around the proximal aorta and between the aortic root and the left atrial roof were confirmed to be one of the causes for this rare form. A fistula to the cardiac cavity following an aortic dissection may occur in any patient, especially in those with a history of previous cardiac surgery.

Aged↗
PubMed1995

[Diagnostic accuracy of transesophageal echocardiography in the diagnosis of aortic dissection: comparison with computerized axial tomography].

This study was designed to assess the sensibility, specificity and diagnostic accuracy of transesophageal echocardiography (TEE) and X-ray contrast enhanced computed tomography (CT) in the diagnosis of aortic dissection and its complications. Fifty patients with clinically suspected aortic dissection were examined. Imaging results were validated in each case by intraoperative and/or autopsy findings and/or the results of cineangiography. The Stanford and DeBakey classifications were used to differentiate the dissection type; the patients were also subdivided by TEE according to a modified DeBakey classification. The sensibility of TEE to detect aortic dissection was 100%, significantly higher (p < 0.05) than that of CT for type A dissections (77.2%). The two imaging procedures did not statistically differ (NS) in the detection of type B dissection (CT sensibility 87.5%). The specificity of TEE for the detection of type A aortic dissection was 94%; it was not significantly higher (NS) than that of CT (CT specificity 86.6%). Both TEE and CT had no false negative findings in the diagnosis of type B aortic dissection (100%; TEE vs CT, NS). TEE was reliable in the correct identification of the primary entry site in the ascending aorta (80%), the arch (62.5%) and descending aorta (71.4%), and also in the involvement of coronary arteries (62.5%), and aortic arch branch vessels (71.4%); CT scanning was not effective in detecting any of these complications. Aortic regurgitation was accurately identified by TEE in each case. Both TEE and CT scanning correctly identified thrombosis of the false lumen and pericardial effusion. Intraoperative TEE documented in all patients postrepair persistence of the intimal flap in aortic segments that were not operated; flow in the false lumen was detected in 46.6% of the patients; in 26.6% of them secondary tears, not seen before surgical treatment, were detected. In conclusion, TEE allows a bedside, safe and accurate diagnosis and classification of aortic dissection. It also provides the diagnostic information necessary for the therapeutical decision making. Intraoperative TEE allows improvement in preoperatory diagnosis and gives important information for the management of the patient immediately after cardiopulmonary bypass and in the follow-up.

Adult↗