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Genetic basis of thoracic aortic aneurysms and aortic dissections.

Ascending thoracic aortic aneurysms leading to type A dissections (TAAD) can occur in association with a genetic syndrome, such as Marfan syndrome (MFS), or as an autosomal dominant disorder in the absence of syndromic features, termed familial TAAD. Familial TAAD demonstrates genetic heterogeneity, and linkage studies have identified three TAAD loci at 5q13-14 (TAAD1), 11q23 (FAA1), and 3p24-25 (TAAD2). The underlying genetic heterogeneity of TAAD is reflected in the phenotypic variation associated with familial TAAD with respect to age of onset, progression, penetrance, and association with additional cardiac and vascular features. Recently, mutations in the TGFBR2 gene have been identified as the cause of disease linked to the TAAD2 locus, supporting the hypothesis that dysregulation of TGFbeta signaling is a mechanism leading to aneurysms and dissections. The recent identification of the TGFbeta pathway as a key target in the molecular pathogenesis of TAAD has opened new avenues for future genetic and therapeutic research.

Aortic Aneurysm↗

Ascending aortic dissection in weight lifters with cystic medial degeneration.

We report 4 cases of ascending aortic dissection in patients with long histories of weight lifting. In 2 of the patients, the initial symptoms of dissection developed while they were lifting weights. Two patients had a history of hypertension and 2 had previously used anabolic steroids. All 4 were successfully treated surgically. Histopathology showed aortic medial changes in all 4. We believe that the hemodynamic stresses of weight lifting, namely, a rapid increase in systemic arterial blood pressure without a decrease in total peripheral vascular resistance, in combination with aortic medial degeneration may have contributed to the development of the aortic dissection.

Adult↗

[Surgical treatment of acute aortic dissections--a clinical study using a ringed intraluminal graft].

From 1984 to 1994, surgery was performed using a ringed intraluminal graft (RIG) in 75 patients with acute aortic dissection (DeBakey's type I in 37 cases, type II in 10 cases and type III in 28 cases). The operative death rate was 24% for type I, 30% for type II and 21% for type III. The average time from onset to surgery was short (47 +/- 67 hours for type I, 34 +/- 36 hours for type II and 47 +/- 77 hours for type III). The outcome of these cases indicated that this technique was effective for saving the lives of patients in the acute early stage of aortic dissection. No characteristic complications developed after this surgery. The postoperative course of patients was followed by using CT scans, MRI, angiography. No patients developed aneurysmal formation in the ligated area or dislocation of the RIG. There were no deaths directly attributable to the RIG. Patients who were autopsied in the late postoperative period showed no aneurysm of the ligated area or necrosis of the aortic wall. In conclusion, RIG surgery effectively saved the life of patients with acute aortic dissection and the RIG could be used as prosthetic graft for long-term periods.

Acute Disease↗

Utility of intraoperative transesophageal echocardiography during endovascular stent-graft repair of acute thoracic aortic dissection.

This study was undertaken to evaluate the utility and efficacy of intraoperative transesophageal echocardiography (TEE) in the endovascular management of patients with acute type B aortic dissection. Twelve consecutive patients with acute type B aortic dissection underwent elective endoluminal stent-graft repair in the operating room under angiographic and TEE guidance. The follow-up protocol included spiral CT scanning before discharge from the hospital to assess thrombosis exclusion of the aortic false lumen, perfusion of branch vessels, and the absence of perigraft leak. No complications related to the use of TEE were encountered. TEE clearly demonstrated the presence and extent of the dissection flap in all patients. After the procedure a persistent perigraft leak or residual flow into the false lumen was diagnosed by TEE in 6 patients, whereas intraoperative angiography visualized only 3 cases. Postoperative CT scan confirmed these findings. One patient died 2 months later by aortic rupture secondary to a persistent perigraft leak not treated (8.3% mortality). No postoperative paraplegia or visceral ischaemia were registered, although an asymptomatic occlusion of left subclavian artery was observed. TEE monitoring detects perigraft leakage, identifies the entry tear, and accurately demonstrates sealing of the false lumen. TEE is essential in our practice as an adjuvant to fluoroscopy to achieve optimal results during endovascular stent-graft repair of acute thoracic aortic dissection.

Acute Disease↗

Modification of the elephant trunk procedure for treatment of acute aortic dissection.

The distal anastomosis constructed during repair of an aortic dissection is prone to bleeding, reentry, and neodissection. To protect the hemostasis, a modification of Borst's elephant trunk procedure has been devised. This "mini-trunk" protects the anastomosis with an internal buttress using the crease of an infolded graft and with a short flap of graft everted into the distal lumen preventing access of the blood stream to this newly constructed suture line.

Acute Disease↗

[A case of successful operation for chronic aortic dissection (DeBakey 1 type) complicated with cerebral infarction].

We report a successful operation for a patient of chronic aortic dissection complicated with cerebral infarction. A 60-year-old woman has been treated suffering from cerebral infarction for 7 years. She was referred to our hospital for abnormal chest X-ray and complaint of dry cough. The enhanced CT scan and aortogram demonstrated chronic aortic dissection (DeBakey I) extending from ascending aorta to the abdominal aortic bifurcation with occlusion of right common carotid artery and dilated ascending aorta and aortic arch. The onset of aortic dissection seemed to be, from a clinical point of view, coincident with the onset of cerebral infarction seven years ago. The replacement of the ascending aorta and aortic arch with woven Dacron velour graft were performed under hypothermic and selective cerebral perfusion. The postoperative course was uneventful although complicated with transient recurrence nerve palsy and she has been doing well.

Aortic Aneurysm, Thoracic↗

[Aortic dissecting aneurysms: clinical masks, differential diagnosis].

101 necropsies of aortic dissection (AD) made in 1991-2000 have been analysed. Among the deceased men prevailed (76.3%), 23(22.8%) of them were under 50 years of age. An accurate intravital diagnosis was made only in 40.6 patients. The most common clinical masks were the following: coronary, valvular, vascular, gastrointestinal, pulmonary, thromboembolic, mediastinal, cerebral, renal and anemic. Most often AD masks as acute myocardial infarction. The knowledge of encountered clinical masks of AD promoted improvement of early AD diagnosis to correct AD surgically and reduce lethality.

Adult↗

[Aortic dissection].

Most often revealed thanks to chest pain, acute aortic dissection must be suspected and diagnosed without losing anytime, because of their evolutive risks and their specific treatment in a specialised unit. Once the early management is over, survivers must be closely followed, specially for their blood pressure to be kept normal, and to screen for secondary complications that would require surgery. Owing to the improvement of early management and follow up, facilitated by the new non-invasive imaging techniques of the aorta, the severe prognosis of this disease has significantly improved.

Aortic Dissection↗

Risk factor analysis for proximal and distal reoperations after surgery for acute type A aortic dissection.

OBJECTIVE: This study was undertaken to determine significant risk factors for proximal or distal reoperations after surgical correction of acute type A aortic dissection. METHODS: Between 1980 and 2000, a total of 160 consecutive patients (mean age 57.5 +/- 13.3 years, 126 men) underwent surgery for acute type A aortic dissection. Proximal repair was performed by means of ascending aorta replacement with valve resuspension in 130 cases (81.3%), composite graft replacement in 19 cases (11.9%), separate aortic valve and ascending aorta replacement in 7 cases (4.4%), and aortic repair in 1 case (0.6%). Distal repair required arch replacement in 23 cases. Follow-up time averaged 4.51 +/- 5.6 years per patient. RESULTS: Survival estimates after initial operation were 66.1% +/- 3.8%, 57.7% +/- 4.2%, 52.2% +/- 4.6%, and 42.5% +/- 5.8% at 1, 5, 10, and 15 years, respectively. Thirty patients required 37 reoperations at a mean interval of 5.7 +/- 4.5 years after the initial operation. Freedoms from reoperation were 96.9% +/- 1.8%, 74.7% +/- 5.3%, 60.8% +/- 6.8%, and 39.3% +/- 9.1% at 1, 5, 10, and 15 years, respectively. Reoperations included procedures on the proximal aorta (aortic root or valve) in 21 cases and on the distal aorta or its side branches in 19 cases. Cox regression analysis distinguished severe preoperative aortic valve insufficiency as the only significant risk factors for proximal reoperation; younger patient age, more distal extent of dissection, and more recent operative date were found to be significant risk factors for distal reoperation. CONCLUSION: Patients with acute type A aortic dissection who have severe aortic valve insufficiency are at increased risk for proximal reoperation. These patients should benefit from a more aggressive proximal repair at initial operation. Distal extent of aortic resection at initial operation did not significantly influence the risk of distal reoperation.

Acute Disease↗

Diagnostic implications of elevated levels of smooth-muscle myosin heavy-chain protein in acute aortic dissection. The smooth muscle myosin heavy chain study.

BACKGROUND: A rapid 30-minute assay of circulating smooth-muscle myosin heavy-chain protein has been developed as a biochemical diagnostic tool for aortic dissection. OBJECTIVE: To determine the sensitivity and specificity of this assay. DESIGN: Cross-sectional study. SETTING: 8 major cardiovascular centers in Japan. PATIENTS: 95 patients with acute aortic dissection, 48 patients with acute myocardial infarction, and 131 healthy volunteers. MEASUREMENTS: Levels of circulating smooth-muscle myosin heavy-chain protein. RESULTS: Patients with acute aortic dissection who presented within 3 hours after onset had elevated levels of circulating smooth-muscle myosin heavy-chain protein. In these patients, the assay had a sensitivity of 90.9%, a specificity of 98% compared with healthy volunteers, and a specificity of 83% compared with patients who had acute myocardial infarction; the clinical decision limit was 2.5 microgram/L. All patients with proximal lesions had elevated levels of smooth-muscle myosin heavy-chain protein, and only patients with distal lesions had decreased levels (<2.5 microgram/L). CONCLUSIONS: Levels of smooth-muscle myosin heavy-chain protein can be used to diagnose aortic dissection soon after symptom onset. The assay had the greatest diagnostic value in patients with proximal lesions.

Aged↗

Creatine-kinase isoenzyme pattern in colonic infarction consequent to acute aortic dissection. A case report.

Bowel infarction can complicate acute aortic dissection (AAD); in that case early diagnosis, which decreases the high mortality, is often difficult. We report the case of one patient who underwent surgery for AAD and developed a colonic infarction, which was clinically manifest on the 4th postoperative day. However, bowel ischemia was suspected already 48 hours after surgery, on the basis of the suggestive CK pattern and the absence of myoglobin in plasma. Total CK activity reached the highest level only 48 hours after surgery (92,800 U/l); the peak was coincident with LDH, which increased proportionally less; CK-MM constituted 100% of total CK activity. The absence of myoglobin in plasma excluded the presence of rhabdomyolysis. We conclude that such laboratory findings suggest the occurrence of severe bowel ischemia.

Acute Disease↗

Acute aortic dissection provoked by sneeze: a case report.

The response of the abdominal viscera and the contraction of the intercostal muscles during the respiratory phase of sneezing increases intrathoracic pressure, which may lead to several complications. However, there are no reports in the literature concerning aortic dissection after sneezing. We report a patient in whom the development of dissection was secondary to sneezing, although hypertension was present as a risk factor, and we discuss the relationship between sneezing and aortic dissection. To our knowledge, this is the first report of aortic dissection provoked by sneezing in the literature.

Acute Disease↗

Superior pericardial recess simulating aortic dissection on computed tomography.

Computed tomography is frequently used in the initial workup of patients with suspected aortic dissection to distinguish dissection involving the ascending aorta from dissection limited to the descending aorta. We reviewed CT of 19 patients who were proven by aortography to have normal ascending aortas. In eight patients the superior extension of the pericardium was visualized and thought to be a potential source of a false-positive diagnosis of type A aortic dissection.

Aortic Dissection↗

Creation of aortic dissection model in swine.

The use of mongrel dogs for experimental purposes was recently restricted and this report presents the experience of creating an aortic dissection model in swine. All the swine in group 1 were anesthetized without pentobarbital and the descending aorta was side-clamped during the creation of the aortic dissection. The false lumen of the completed dissection was patent in the long term despite not having the anchoring suture that the previous canine model required to stabilize the opening of the entry tear. All the swine anesthetized with pentobarbital (ie, group 2) died of heart failure either during cross-clamping of the descending aorta or postoperative aortography. In conclusion, creation of a thoracic aortic dissection is possible in swine, but cross-clamping of the thoracic descending aorta and pentobarbital anesthesia should be avoided.

Aortic Dissection↗

[Abdominal aortic surgery in Stanford type B chronic dissecting aortic aneurysm].

Three cases of a chronic type B dissecting aneurysm which required abdominal aortic reconstruction are presented. Constriction of the iliac artery due to dissection was found in case 1, in case 2 the left subclavian artery originated from a giant false lumen, and arteriosclerosis obliterans was observed in case 3. The left renal artery originated from a false lumen in all cases. Each patient underwent preliminary graft replacement of the abdominal aorta because using the femoral artery for extracorporeal circulation had a higher risk factor. The distal arch and descending aorta were then replaced with woven dacron graft. In a chronic type B dissecting aneurysm, the surgical procedure is sometimes complex because of the narrowing of the true lumen and the enlargement of the false lumen or arteries originating from the false lumen to major organs. It is necessary to consider preliminary abdominal reconstruction in cases of 1) dilatation of abdominal aneurysm, 2) obstruction, stenosis or thrombus below the abdominal aorta, or 3) presence of a giant false lumen.

Aged↗

[Comparative study of transthoracic Doppler echocardiogram, computed tomography and aortography in the diagnosis of thoracic aortic dissection].

PURPOSE: Comparative and prospective evaluation of three methods (transthoracic echo-Doppler (TTE), computerized tomography (CT) and aortography (AORT) utilized for aortic dissection diagnosis. METHODS: The 39 patients with confirmed aortic dissection (surgery or autopsy) underwent, within a few hours of each other, all three methods scrutinized. There were 19 cases of type A and 20 of type B dissection. RESULTS: In type A dissection the methods were equivalent (TTE = 73.7%, CT = 84.2%, AORT = 73.7% p = NS) but for type B, TTE was significantly inferior to the other two methods (TTE = 60%, TC 90%, AORT = 80%, p < 0.05 for TTE, for TC and AORT p = NS). In three occasions, even though all three methods were performed, the diagnosis was not obtained. CONCLUSION: The methods which were evaluated make the diagnosis in the majority of cases. In type A all methods are similar, however, in type B, TC and AORT are superior to TTE. Even performing all three methods in each patient, in three instances the diagnosis was not made.

Adult↗

Postoperative CT follow-up of aortic dissection.

Computed tomography with contrast enhancement permits noninvasive follow-up of patients treated for aortic dissection. We used CT to examine 52 patients with aortic dissections for up to 14 years postoperatively. The mean interval between surgery and CT was 32 months. Computed tomography demonstrated the aortic graft in 45 patients. Persistent patency of a false lumen was observed in 40 cases. In one case, CT demonstrated extension of the dissection. No rupture or thoracic pseudoaneurysm was noted in our series.

Adult↗