Acute aortic syndromes include a variety of overlapping clinical and anatomic diseases. Intramural hematoma (IMH), penetrating atherosclerotic ulcer (PAU), and aortic dissection can occur as isolated processes or can be found in association. All these entities are potentially life threatening, so prompt diagnosis and treatment is of paramount importance. IMH and PAU affect patients with atherosclerotic risk factors and are located in the descending aorta in 60-70% of cases. IMH diagnosis can be correctly made in most cases. Aortic ulcer is a morphologic entity which comprises several entities-the differential diagnosis includes PAU, focal intimal disruptions (FID) in the context of IMH evolution and ulcerated atherosclerotic plaque. The pathophysiologic mechanism, evolution and prognosis differ somewhat between these entities. However, most PAU are diagnosed incidentally outside the acute phase. Persistent pain despite medical treatment, hemodynamic instability, maximum aortic diameter (MAD) >55 mm, significant periaortic hemorrhage and FID in acute phase of IMH are predictors of acute-phase mortality. In these cases, TEVAR or open surgery should be considered. In non-complicated IMH or PAU, without significant aortic enlargement, strict control of cardiovascular risk factors and frequent follow-up imaging appears to be a safe management strategy.

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Bicuspid aortic valve (BAV) patients are at high risk of developing progressive aortic valve dysfunction and ascending aorta dilation. However, the progression of the disease is not well defined. We aimed to assess mid-long-term aorta dilation and valve dysfunction progression and their predictors. Patients were referred from cardiac outpatient clinics to the echocardiographic laboratories of 10 tertiary hospitals and followed clinically and by echocardiography for >5 years. Seven hundred and eighteen patients with BAV (median age 47.8 years [IQR 33–62], 69.2% male) were recruited. BAV without raphe was observed in 11.3%. After a median follow-up of 7.2 years [IQR5–8], mean aortic root growth rate was 0.23 ± 0.15 mm/year. On multivariate analysis, rapid aortic root dilation (>0.35 mm/year) was associated with male sex, hypertension, presence of raphe and aortic regurgitation. Annual ascending aorta growth rate was 0.43 ± 0.32 mm/year. Rapid ascending aorta dilation was related only to hypertension. Variables associated with aortic stenosis and regurgitation progression, adjusted by follow-up time, were presence of raphe, hypertension and dyslipidemia and basal valvular dysfunction, respectively. Intrinsic BAV characteristics and cardiovascular risk factors were associated with aorta dilation and valvular dysfunction progression, taking into account the inherent limitations of our study-design. Strict and early control of cardiovascular risk factors is mandatory in BAV patients.

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