Diagnosis and Management of Acute Aortic Syndromes: Dissection, Intramural Hematoma, and Penetrating Aortic Ulcer

Abstract

Acute aortic syndromes constitute a spectrum of conditions characterized by disruptions in the integrity of the aortic wall that may lead to potentially catastrophic outcomes. They include classic aortic dissection, intramural hematoma, and penetrating aortic ulcer. Although imaging studies are sensitive and specific, timely diagnosis can be delayed because of variability in presenting symptoms and the relatively low frequency with which acute aortic syndromes are seen in the emergency setting. Traditional classification systems, such as the Stanford system, facilitate early treatment decision-making through recognition of the high risk of death and major complications associated with involvement of the ascending aorta (type A). These patients are treated surgically unless intractable and severe co-morbidities are present. Outcomes with dissections that do not involve the ascending aorta (type B) depend on the presence of acute complications (e.g., malperfusion, early aneurysm formation, leakage), the patency and size of the false lumen, and patient co-morbidities. Patients with uncomplicated type B dissections are initially treated medically. Endovascular techniques have emerged as an alternative to surgery for the management of complicated type B dissections when intervention is necessary. Patients with acute aortic syndromes require aggressive medical care, risk stratification for additional complications and targeted genetic assessment as well as careful long-term monitoring to assess for evolving complications. The optimal care of patients with acute aortic syndrome requires the cooperation of members of an experienced multidisciplinary team both in the acute and chronic setting.

Sudden Cardiac Death

“…These syndromes include aortic dissection, aneurysm expansion, trauma, penetrating ulcer, and intramural hematoma. 12 Patient risk factors include age and hypertension. Loss of tensile strength due to Marfan syndrome, Ehlers-Danlos syndrome, familial thoracic aortic aneurysm disease, and aortopathy associated with congenital bicuspid aortic valve also increase the associated risk.…”

Precipitous Resolution of Type-A Intramural Hematoma with Medical Management in a Patient with Metastatic Stage 4 Renal Cell Carcinoma

“…Acute type-A IMH is treated surgically, whereas type-B IMH in the absence of complications is treated with anti-impulse therapy and strict followup. 6 Conversely, this doctrine has been challenged by Asian studies that have shown good outcomes and resolution in medically treated patients with type-A IMH. 2,13 Moizumi et al 14 found that among the patients in their study, more than 50% were safely treated with medical therapy alone without the development of an overt complication.…”

“…Type-A IMH/PAU are managed surgically whereas type-B IMH/PAU are medically managed with conversion to surgery in the event of rapid aortic dilatation, impending rupture, and frank rupture. 6,7 Nevertheless, this approach has been questioned by studies from Eastern countries. An unknown "Asian factor" has been invoked to explain the improved outcomes seen with medical management of type-A IMH in Japanese and Korean series.…”

New Paradigms in the Treatment of Acute Complicated and Uncomplicated Type B Aortic Dissection

“…Acute aortic syndrome actually refers to a spectrum of diseases ranging from aortic rupture at one extreme to penetrating aortic ulcer with or without expansion at the other. 38 Intramural hematoma, for example, occurs when microscopic tears in the media allows blood to escape into the medial layer of the aorta without flowing back into the true lumen. Although a thorough discussion of acute aortic syndromes other than dissection are beyond the scope of this review, it is clear that with the advent of TEVAR, new treatment paradigms are being investigated in regard to these entities as well.…”