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The Diagnosis and Management of Aortic Dissection

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The Diagnosis and Management of Aortic Dissection CLINICAL REVIEW The diagnosis and management Follow the link from the online version of this article to obtain certi ed continuing medical education credits of aortic dissection Sri G Thrumurthy, Alan Karthikesalingam, Benjamin O Patterson, Peter J E Holt, Matt M Thompson Department of Outcomes Research, Aortic dissection is caused by an intimal and medial tear SOURCES AND SELECTION CRITERIA St George’s Vascular Institute, in the aorta with propagation of a false lumen within the We searched the Medline, Embase, Web of Science, and Cochrane London SW17 0QT, UK aortic media. It is part of the “acute aortic syndrome”— databases for “aortic dissection” and used reference lists to Correspondence to: P J E Holt an umbrella term for aortic dissection, intramural hae- identify key studies. Two authors independently performed the [email protected] matoma, and symptomatic aortic ulcer (table).1 Acute searches and mutual consensus was reached. Because of the Cite this as: BMJ 2012;344:d8290 lack of large well designed randomised controlled trials, we gave doi: 10.1136/bmj.d8290 dissection is the most common aortic emergency, with priority to systematic reviews, meta-analyses, and studies from the an annual incidence of 3-4 per 100 000 in the United International Registry of Acute Aortic Dissection. Kingdom and United States, which exceeds that of rup- tured aneurysm.2 w1 w2 The prognosis is grave, with 20% preadmission mortality and 30% in-hospital mortality.2 European Society of Cardiologists’ classification of acute aortic The best treatment depends on the anatomical and syndrome temporal classification of the disease. Aortic dissection is Classification Pathology Classic dissection with true and false lumens separated therefore categorised according to the site of the entry tear Type 1 and the time between the onset of symptoms and diagno- by the dissecting membrane sis. A dissection is considered “acute” when the diagnosis Type 2 Intramural haematoma Discrete dissection with a bulge at the tear site but no Type 3 is made within 14 days of onset, and thereafter it is termed haematoma “chronic.” The location of the entry tear plays a key role Type 4 Penetrating aortic ulcer bmj.com in treatment and outcome, and it is classified by being in Type 5 Traumatic or iatrogenic dissection Previous articles in this the ascending aorta (Stanford type A dissection) or distal series to the origin of the left subclavian artery (Stanford type B DeBakey classication Ж Laparoscopic dissection) (fig 1).3 Type I Type II Type III colorectal surgery Type A dissection carries a far worse prognosis than (BMJ 2011;343:d8029) type B dissection and urgent surgical intervention is often Ж Managing infants who needed. By contrast, acute type B dissection is usually cry excessively in the first managed conservatively if uncomplicated and surgically few months of life if complicated. We review the epidemiology, diagnosis, and man- (BMJ 2011;343:d7772) agement of aortic dissection drawing on evidence from Ж Managing motion population studies, randomised controlled trials, meta- sickness analyses, and published guidelines. (BMJ 2011;343:d7430) Ж Osteoarthritis at the Who is at risk? base of the thumb The causes of aortic dissection are multifactorial, and both Stanford classication Type A Type B BMJ 2011;343:d7122) inherited susceptibility and acquired degenerative disease Ж Inherited have been implicated. Several modifiable and non-modi- DeBakey classication cardiomyopathies fiable risk factors are recognised, the most important of Type I Originates in the ascending aorta; propagates at least (BMJ 2011;343:d6966) which are discussed below. to the aortic arch and oen beyond it distally Type II Originates in and is con ned to the ascending aorta Type III Originates in the descending aorta and extends SUMMARY POINTS distally down the aorta or, rarely, retrograde into the aortic arch and ascending aorta Aortic dissection is diagnosed and managed according to its anatomical extent and chronicity White men aged over 40 years with hypertension, or those under 40 with Marfan’s syndrome Stanford classication or bicuspid aortic valves, are at highest risk Type A All dissections that aect the ascending aorta, regardless of the site of origin Patients often present with acute onset sharp chest pain, sometimes with loss of Type B All dissections that do not aect the ascending aorta consciousness or poor perfusion of end organs Computed tomography aortography is the first line diagnostic investigation, followed by Fig 1 | The Stanford and DeBakey classifications of aortic transoesophageal echocardiography; magnetic resonance angiography is preferred for dissection. The dissection types are mainly differentiated by surveillance whether they affect the ascending aorta (the ascending aorta Manage proximal (type A) dissection surgically if possible is affected in Stanford type A dissections, but not in Stanford Uncomplicated distal (type B) dissection is best managed with intensive drug treatment; type B dissections). Urgent surgical intervention is warranted complicated type B dissection requires surgical intervention when the ascending aorta is affected, and such cases are All patients need lifelong antihypertensive therapy and surveillance imaging associated with higher mortality and morbidity than isolated descending aortic dissection4 BMJ | 14 JANUARY 2012 | VOLUME 344 37 CLINICAL REVIEW Hypertension TIPS FOR NON-SPECIALISTS Systemic hypertension is one of the most important risk Refer patients with confirmed aortic dissection (or symptomatic factors for aortic dissection and is present in 40-75% of 3 high risk patients) to a regional cardiovascular unit for urgent patients presenting with the condition. Systolic hyperten- diagnostic investigation and treatment sion exacerbates the differential haemodynamic forces act- Young patients with a history of connective tissue disease (such ing on the relatively mobile aortic arch and the relatively as Marfan’s disease) or congenital cardiovascular disease (such fixed ascending and descending thoracic aorta. A cohort as bicuspid aortic valves) are at high risk study of 175 patients identified physical exertion or emo- Maintain systolic blood pressure at 100-120 mm Hg in tional stress as the direct predecessor of acute pain in 66% patients with a history of dissection; prescribe antihypertensive of acute dissections, primarily as a result of acute changes drugs (including β blockers) and deal with other modifiable in blood pressure during the event.w3 cardiovascular risk factors Ensure that patients with a history of dissection are enrolled in a Race and sex surveillance programme at a regional cardiovascular unit A cross sectional study of 951 patients by the International Registry of Acute Aortic Dissection, comprising data from been attributed largely to age, sex, aortic valve dysfunc- 12 international referral centres, showed that 68% of all tion, and the hypertension associated with coarctation.w7 patients presenting with the condition were male and 79% Several familial aneurysmal syndromes (such as congeni- were white.5 tal contractural arachnodactyly, familial thoracic aortic aneurysm or Erdheim’s cystic medial necrosis, familial aor- Connective tissue diseases tic dissection, familial ectopia lentis, and familial Marfan- Various connective tissue diseases predispose to the like habitus) also predispose to aortic dissection.6 w8 inherent weakening of the aortic wall and subsequent dissection, and these diseases are especially important in Miscellaneous risk factors patients under 40 years. These include Marfan’s syndrome Prevalence studies have shown that aortic vasculitic with fibrillin defects, which is seen in 15-50% of patients disease,w9 cocaine misuse,w10 and pregnancy7 are risk fac- under 40 years5 w4; Ehlers-Danlos type IV with abnormal tors for aortic dissection. One report on 723 patients found synthesis of type III procollagenw5; and other connective a 5% rate of iatrogenic aortic dissection after cardiac inter- tissue disorders associated with cystic medial necrosis.w6 ventions, including percutaneous revascularisation and coronary artery bypass grafting.w11 Congenital cardiovascular abnormalities Although British national statistics show that dissection A cross sectional study described a fivefold to 18-fold affects all ages (27% of patients aged 17-59 years, 40% increased risk of dissection in 516 patients with bicus- aged 60-74 years,w12 33% aged >75 years), older patients pid aortic valves.5 This increased risk was attributed to a (>40 years) are more likely to have concurrent hyperten- c oinherited developmental defect of the proximal aorta, sion or atherosclerosis, whereas younger patients are more which conferred a predilection towards apoptosis of the likely to have Marfan’s syndrome, a bicuspid aortic valve, cellular components of the aortic media, and subsequent or aortic intervention before presentation.5 medial weakening and aortic dilation. The presence of a bicuspid aortic valve was also associated with dissection in How do patients present? a greater proportion of patients under 40 years (9% under Patients typically present with the abrupt onset of sharp 40 v 1% over 40; P<0.001). A prospective study of 631 tearing or stabbing chest pain, which may improve slightly patients from the adult congenital heart disease database over time, although pain may be absent in 10% of patients.8 showed that the coexistence of coarctation of the aorta Asymptomatic presentation is more common in patients with
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