Aortic dissection with diastolic prolapse of intimal flap into left ventricle.

Ascending aortic dissection is often a catastrophic condition. Dissection into the commissures or into an aortic valve leaflet may lead to leaflet avulsion and valvular insufficiency due to a flail valve. We present an image report describing an important and life-threatening complication due to the movement of a partially dehisced intimal aortic flap into the left ventricle causing aortic valve insufficiency in a patient with acute dissection of the ascending aorta.     

Aortic dilatation resulting in chronic aortic regurgitation and complicated by aortic dissection in a patient with Turner's syndrome

A patient with XO Turner's syndrome with a 12-year history of progressive aortic root dilatation resulting in chronic aortic regurgitation is presented. Her case is unique in that it occurred in the absence of coarctation of the aorta, bicuspid aortic valve, or hypertension. Idiopathic dilatation of the aorta may be an additional risk factor to the development of aortic dissection in the setting of Turner's syndrome.     

Aneurysms of the ascending aorta: Diagnostic features and prognosis in patients with marfan's syndrome versus hypertension

Background: In Marfan's syndrome progressive dilation of the sinuses of Valsalva, the supra-aortic ridge and the ascending aorta are well characterized abnormalities likely to set the stage for severe aortic sequelae accounting for 70% of lethal complications. However, the specific anatomical, clinical, and prognostic profiles of aortic pathology are less well characterized in the setting of patients with Marfan's syndrome symptomatic from aortic complications. Hypothesis: The study was designed to characterize the spectrum of anatomical, clinical, and prognostic profiles of thoracic aortic disease in symptomatic patients with Marfan's syndrome compared with patients with arterial hypertension. Noninvasive imaging techniques were used for comprehensive mapping of aortic pathology associated with Marfan's syndrome. Methods: Thirty-five consecutive patients with Marfan's syndrome (16 women, 19 men; mean age 35± 12 years) were imaged by transthoracic (TTE; n = 26) and transesophageal echocardiography (TEE; n = 11), contrast enhanced computed tomography (XCT; n = 16), or magnetic resonance techniques (MRI; n = 14). Diagnostic results were compared with both in-traoperati ve or angiographic findings and also with a group of 85 consecutive patients with aortic pathology associated with arterial hypertension. Results: Aortic pathology was more frequently confined to the ascending aorta (p<0.05) and aortic regurgitation was more prevalent in Marfan's syndrome than in arterial hypertension (p < 0.05). In 23 cases (66%) of dissection and 12 cases (34%) of nondissecting aneurysms, no Marfan-specific aortic macropathology was identified when compared with arterial hypertension. Diagnostic results of TEE, XCT, and MRI were all excellent; however, in contrast to the transesophageal ultrasound approach, transthoracic ultrasound was not useful in the detection of aortic dissection and intramural hemorrhage. Both 30-day (100 vs. 70%; p<0.05) and 5-year survival rates (91 vs. 57%; NS) were higher in patients with Marfan's syndrome than in those with arterial hypertension. Repeat surgery, however, was more frequently required in patients with Marfan's syndrome (25% vs. none in aortic aneurysm; p < 0.05). Conclusions: There are no macropathologic findings of the aorta specific for Marfan's syndrome. In patients with Marfan's syndrome with an inherently high rate of aortic complications, serial noninvasive imaging should be encouraged, preferably utilizing TEE or MRI.     

Severe aortic regurgitation with intimal intussusception secondary to Debakey type I aortic dissection

Aortic regurgitation (AR) with intimal intussusception, secondary to aortic dissection, is relatively rare and the images are interesting findings. We report a typical case of severe AR with intimal intussusception, secondary to DeBakey type I aortic dissection, detected by contrast-enhanced computed tomography (CECT) and transesophageal echocardiography (TEE). Since there are three types of aortic regurgitation with aortic dissection, it is imperative to consider the most appropriate intervention for AR. The combination of CECT, TEE, and surgical findings may play an important role in determining the optimum surgical procedure for AR with aortic dissection.     

Survival and complication free survival in Marfan's syndrome: implications of current guidelines

OBJECTIVE—To evaluate survival and complication free survival in patients with Marfan's syndrome and to assess the possible influence of recently revised guidelines for prophylactic aortic root replacement in these patients.
METHODS—130 patients who had been attending one institution over 14 years were evaluated. Kaplan-Meier analysis was performed in 125 patients who did not present with aortic root dissection as the first sign of Marfan's syndrome, with the end points: death, aortic root dissection, and prophylactic aortic root replacement after diagnosis. In the patients developing aortic root dissection, current guidelines for prophylactic aortic root replacement were retrospectively applied to investigate the number of dissections that could theoretically have been prevented. The guidelines were: (1) aortic root diameter  55 mm, (2) positive family history of aortic dissections and aortic root diameter  50 mm, and (3) aortic root growth  2 mm/year. Outcomes following emergency surgery (15 patients) and prophylactic surgery of the aortic root (30 patients) were compared.
RESULTS—Five and 10 year survival after diagnosis was 95% and 88%, and the five and 10 year complication free survival was 78% and 66%, respectively. Thirteen patients developed dissection, 30 underwent prophylactic repair, and 82 had an uncomplicated course. Eleven dissections could theoretically have been prevented by application of the current guidelines. Five year survival following emergency and prophylactic repair of the aortic root was 51%, and 97%, respectively.
CONCLUSIONS—Survival in the Marfan's syndrome in the past 14 years seems satisfactory; with application of current guidelines, it has probably even improved. However, because of the high fatality rate in Marfan patients developing aortic root dissection, more extensive screening for Marfan's syndrome and a search for additional risk factors are desirable.


Keywords: Marfan's syndrome; aortic root dissection; survival     

应用主动脉根部替换联合象鼻技术治疗复杂主动脉夹层

目的：寻求手术治疗复杂主动脉夹层的妥善方法。方法：在全麻、深低温停循环加选择性脑灌注下行主动脉根部替换和象鼻技术联合治疗２例。结果：临床疗效满意,无神经系统并发症,近期内避免了Ⅱ期手术。结论：该手术操作复杂,技术难度大,有可能避免Ⅱ期手术,采用右锁骨下动脉选择性脑灌注可预防神经系统并发症。     

Bentall operation in a patient with an anomalous left circumflex artery: Case report and review

Anomalous origin of a left circumflex artery from the right coronary sinus represents a technical challenge in patients who require aortic valve/root procedures. This case report describes a patient who presented with bicuspid aortic valve, anomalous origin of the circumflex artery, severe aortic regurgitation, and aneurysm of the ascending aorta as well as aortic root that was safely managed following the Bentall procedure with the combined button technique.     

Massive progression of annuloaortic ectasia in a patient with Takayasu aortitis

A 52-year-old woman with Takayasu aortitis had undergone coronary artery bypass grafting and left subclavian artery reconstruction two years before admission to the Kinki University Medical Hospital (Osaka, Japan). On this admission, marked annuloaortic ectasia with severe aortic regurgitation was noted on echocardiography and aortography. Because of refractory heart failure, a modified Bentall operation was performed. Considering the serious cardiovascular complications of Takayasu aortitis, such as aneurysmal dilation of the aortic root, coronary artery ostial stenosis and, frequently, the need for surgical intervention, patients should be monitored closely.     

Unreliability of aortic size index to predict risk of aortic dissection in a patient with Turner syndrome

Aortic size index(ASI) has been proposed as a reliable criterion to predict risk for aortic dissection in Turner syndrome with significant thresholds of 20-25 mm/m2. We report a case of aortic arch dissection in a patient with Turner syndrome who, from the ASI thresholds proposed, was deemed to be at low risk of aortic dis-section or rupture and was not eligible for prophylactic surgery. This case report strongly supports careful monitoring and surgical evaluation even when the ASI is 20 mm/m2 if other significant risk factors are present.     

Aortic valve perforation in the setting of Cogan's syndrome

Cogan's syndrome is a rare disorder characterized by the coexistence of ocular and audio‐vestibular manifestations. Systemic manifestations are quite unusual with pan‐vasculitis and cardiac involvement reported in the form of aortitis with aortic aneurysm, dissection, or extremely rare aortic valve perforation. Hereby, we report a case of a 56‐year‐old woman presented with ocular, audio‐vestibular, and systemic manifestations with medium‐sized vasculitis in the form of multiple splenic artery aneurysms, superior mesenteric artery thrombosis, and cardiovascular involvement in the form of aortic regurgitation due to noncoronary cusp perforation. To the best of our knowledge, this is the second case to report aortic perforation in the setting of Cogan's syndrome.     

Trends and Outcomes of Elective Thoracic Aortic Repair and Acute Thoracic Aortic Syndromes in the United States

BackgroundThere is a paucity of data on the contemporary outcomes and trends of elective thoracic aortic aneurysm repair and aneurysm-associated acute aortic syndrome.MethodsWe queried the National Inpatient Sample (NIS) database years 2012-2016 to identify hospitalizations for elective thoracic aortic aneurysm repair and aneurysm-associated acute aortic syndrome. The main study outcome was in-hospital mortality.ResultsThe analysis yielded 24,295 hospitalizations for elective thoracic aortic aneurysm repair and 8875 hospitalizations for aneurysm-associated acute aortic syndrome. The number of hospitalizations for elective aortic repair significantly increased from 4375 in 2012 to 5450 in 2016 (P_trend = .01). The number of hospitalizations for acute aortic syndrome numerically increased from 1545 in 2012 to 2340 in 2016 (P_trend = .10). Overall in-hospital mortality for elective aortic repair was 2.4% with no change over time. In-hospital mortality for acute aortic rupture was 39.4% and for acute aortic dissection was 6.2% with no change over time. Hospitalizations for elective aortic repair had lower incidence of complications compared with those for aneurysm-associated acute aortic syndrome, including cardiogenic shock, cardiac arrest, acute stroke, and shorter length of stay. Factors associated with higher mortality among admissions undergoing elective aortic repair included older age, heart failure, valvular disease, and chronic kidney disease. Older age, coagulopathy, and fluid/ electrolytes disorders were associated with increased mortality among those with acute aortic syndrome.ConclusionContemporary elective thoracic aortic aneurysm repair is associated with lower in-hospital mortality and morbidity when compared with a clinical presentation for an aneurysm-associated acute aortic syndrome. This should be taken into account when deciding the timing of elective aortic aneurysm repair and balancing the risks and benefits.     

Onset of Aortic Dissection Complicated with Cushing's Disease: A Case Report and Review of the Literature

Cushing''s syndrome and Cushing''s disease cause various metabolic disorders associated with high cortisol levels. Some reports have shown that Cushing''s syndrome is complicated with dissecting aortic aneurysm and aortic dissection after long-term exposure to high cortisol levels. We herein report a rare case of aortic dissection complicated with Cushing''s disease. Aortic dissection may occur even under relatively short periods of high cortisol conditions. This case suggests that hypercortisolemia should be treated as soon as possible in order to prevent aortic dissection in subjects with Cushing''s disease.     

Fibroma of the left ventricle in a patient with Sotos syndrome

Primary tumors of the heart are rare, with an incidence between 0.001% and 0.33% in autopsy findings, and with fibromas representing about 4% of benign cardiac tumors. We report the case of a cardiac fibroma in a 5-year-old child affected by Sotos syndrome. The mean sign was a ventricular tachycardia.