Human aorta extends from aortic valve at the level of third coastal cartilage to its bifurcation into common

illiac arteries at level of fourth lumbar vertebra The division and respective branches ascending aorta lower portion left and right coronaries upper portion arch innominate, left carotid, left subclavian ateries isthmus descending thoracic pericardial bronchial two on left and one intercostobronchial on right esophageal mediastinal posterior intercoastal 9 pairs phrenic artery abdominal ventral celiac superior mesentric inferior mesentric lateral inferior phrenic middle suprarenal renal gonadal artery dorsal lumbar - 4 pairs median sacral terminal common illiac

Vaso vasorum

small vessels about 350 micro mts in diameter which arise from intercoastal and lumbar arteries , supply the outer two third of media in arteries with more than 29 lamellar units and hence are absent in the infrarenal aorta

For the uniformity of reporting surgical outcomes aorta is divided as follows

zone 0 aotic valve to end of brachiocephalic trunk zone 1 end of brachiocephalic trunk to the end of lt common carotid zone 2 - end of lt common carotid to end of left subclavian zone 3 end of lt subclavian to beginning of vertical descending thoracic aorta zone 4 whole descending thoracic aorta named z 4 z 12 according to interccoastal arteries abd aorta segments are named after lumbar arteries levels from L 1 L 5.

Three layered structure ( 2 mm ) Intima endothelial cells (120 micro mts) subendothelial space internal elastic lamina Media Lamellar units (1200 micro mts) Cells fibroblasts and mast cells ECM elastin , collagen, proteoglycans external elastic lamina Adventitia collagen, fibroblasts, (500 micro mts) nerves, vasa vasorum

Lamellar unit - consistent relationship between no of lamelllar units and hemodynamic stress, except for the infrarenal aorta with fewer lamellae as expected for the hemodynamic load. - ascending aorta has about 55 -60 units which decrease down to 26 at the bifurcation. - the branches of aortic arch and pelvic and leg vessels also show a predominance of elastic elements and tend to become involved more readily than the visceral vessels as the dissection spreads along the aorta Adventitial Collagen governs the tensile strength of aorta - at mechanical strain levels that exceed the extensile capacity of medial elastic fibres, the aortic tensile strength becomes dependent on the collagen fiber meshwork of the media and adventitia. - the dependence on adventitial collagen in acomodating greater hemodynamic stress is an important feature of abd aortic aneurysms and also in surgical endarterectomy, where most of the media is removed without resulting in aneurysmal dilatation

With increasing age, fragmentation of elastin, loss of smooth cells, accumulation of collagen and deposition of basophilic ground substance occurs, resulting in weaking of aortic wall and thus dilatation as well as elongation and uncoiling of the aortic arch.

First reported case King George II in 1760 The first clear pathologic description of dissection was done by Morgagni in 1761. The term dissection was first coined by Maunoir in 1802 Incidence true incidence difficult to calculate as - rapidly fatal - frequently misdiagnosed on presentation - population based studies 2.5 to 3.5 / 100,000 person per year - incidence appears to be increasing as shown by a swedish study by olsson et al 10.7 in 1987 to 16.3/ 100,000 in 2002. Prevalence in a necropsy series, 0.2 - 0.8 %. Mean age at presentation 63 67 yrs Mean age of patients presenting with ascending aortic dissection (61 yrs) was less than those presenting with dissection of descending aorta (66 yrs) in the IRAD study. emales are on an average older at presentation and present later. Male : Female 1.6-1.8

Aortic dissection is a type of acute aortic syndrome, which encompasses three related disorders namely aortic dissection, intramural hematoma and penetrating atherosclerotic ulcer.

Cystic medial necrosis the most common underlying disorder, which describes

degenaration and fragmentation of elastic fibres, smooth muscle cell loss, increase in collagen and replacement with interstitial ground substance. increased expression of aortic collagen types I and III as well as CTGF.

Mutations in the following have been implicated (in familial , non genetic pts)

FBN1, TGFBR2, and TGFBR1 vascular smooth muscle cell (SMC)-specific -myosin (MYH11) and -actin (ACTA2)

Dissection is defined as the process of separating tissue planes along intervening layers of connective tissue. Thus, aortic dissection is defined as the separation of the lamellae of the aortic wall, and resulting in extraluminal blood. There are two main hypotheses explaining the origin of extraluminal blood - primary intimal tear - hemorrhage in the aortic wall with/without subsequent intimal tear probably d/t ruptured vasa vasorum Dissection can propagate, usually in antegrade direcction. Intimal flap can remain localized or may spiral the length of aorta A second intimal tear can be present, producing an exit site In the majority of patients (90%), an intimal disruption is present. This results in a classic dissection with a septum, or flap, between the 2 lumens. While on noninvasive imaging, 15% of patients with aortic dissection syndromes have an apparent IMH without evidence of an intimal tear, autopsy studies show only 4% have no visible intimal tear

The DeBakey classification system categorizes dissections based on the origin of the intimal tear and the extent of the dissection: Type I: Dissection originates in the ascending aorta and propagates distally to include at least the aortic arch and typically the descending aorta Type II: Dissection originates in and is confined to the ascending aorta Type III: Dissection originates in the descending aorta and propagates most often distally. Type IIIa: Limited to the descending thoracic aorta. Type IIIb: Extending below the diaphragm. The Stanford classification system divides dissections into 2 categories, those that involve the ascending aorta and those that do not. Type A: All dissections involving the ascending aorta regardless of the site of origin Type B: All dissections that do not involve the ascending aorta

Type A dissection was seen in 62.3 % pts in IRAD study Intimal tears
65% in asc aorta 30% in desc aorta 10% in aortic arch

1% in abd aorta

Most ascending aorta dissections begin within a few cms of the aortic valve, and in the descending aorta they begin just distal to lt subclavian

Hypertension

IRAD registry- 72.1% An IRAD analysis by Trimarchi et al showed that refractory hypertension appeared as clinical signs associated with increased in-hospital mortality, particularly when managed medically Significantly more common in type b
SBP>150 mm hg in 70% pts of type B and 35% in type A .

Atherosclerotic disease

In 31% pts in IRAD registry Significantly more in type B than A (42%/ 24%) Intima rupture generally occurs at the plaque edge Htn is main risk factor, smoking and hypercholesterolemia are also associated.
Mainly thoracic aortic aneurysm or a family history of the same IRAD Registry 16% pts Risk increases with increasing size IRAD registry in 18% pts

Known aortic aneurysm

Prior cardiac surgery

Persistent abnormalities of aortic wall, untreated or recurrent dissection, operative injuries to aortic wall and intima.

Aortic valve replacement Aortic aneurysm and/or dissection Coronary artery bypass graft surgery Mitral valve surgery

5.4% 9.7% 4..3% 0.7%

Iatrogenic

Cardiac cathiterization 2%/ cardiac surgery 2% Due to intimal injury.

In 4% pts of IRAD

Crack cocaine

Truma

Typical of young african american who are hypertensives and smoke Probably related to sudden surge in BP and tachycardia

High speed motor accidents 15-20 % deaths are related to aortic trauma. Blunt aortic trauma usually leads to localized tears, periaortic hematomas, transections, but rarely frank dissection. Isthmus is most vulnerable part Weight lifting Or other activities with valsalva physiology may ppt aortic dissection due to increased aortic wall stress.

Pregnancy

Typically occur in the third trimester but occasionally in the early post partum period. In addition to hemodynamic factors, hormonal changes in the aortic wall composition have been described. Mutations in FBN1, TGFBR2,TGFBR2,ACTA2,MYH11 should be checked.

Marfans syndrome

The most common cardiovascular features are MVP and dilation of the sinuses of Valsalva. Children tend to be more severely affected by mitral valve disease, whereas aortic problems are progressive and more likely in adolescence and beyond. The sinuses of Valsalva are often dilated at birth, and the rate of progression varies widely among patients in general and also among relatives. Thus, predicting long-term risks of developing aortic regurgitation or suffering aortic dissection or requiring aortic surgery is fraught with uncertainty. Type A dissection is much more common than type B. As shown by Roman et al in their follow up study intial aortic size is the most important predictor of late complication, with pts having intially localized dilation of sinuses having a much lower risk than those with generalized dilation of arch.

Loeys dietz syndrome

Autosomal dominant resulting from mutations in either the transforming growth factor receptor Type I or II (TGFBR1 or TGFBR2) genes and the diagnosis is confirmed through mutational analysis of these genes. Characterized by the triad of arterial tortuousity and aneurysms, hypertelorism and bifid uvula or cleft palate, or a uvula with a wide base or prominent ridge on it. The vascular disease in these patients is particularly aggressive with a mean age of death of 26 years. Most patients have aortic root aneurysms (98%) that lead to AoD.
78

Ehler danlos syndrome type 4, the vascular form

An estimated incidence of 1/5000 birth Autosomal dominant defect in COL3A1 gene located on chromosome 2q31 producing structural defects in type III collagen median survival of only 48 years Most of the fatal complications are caused by arterial rupture, with most deaths attributable to arterial dissections or ruptures involving primarily the thoracic or abdominal arteries, including AoDs and ruptures.

Turners syndrome

Incidence of AoD is 1.4% among individuals with Turner syndrome) Therefore, the risk of AoD is much lower in patients with Turner syndrome compared with patients with Marfan syndrome or Loeys-Dietz syndrome. The majority of dissections in women with Turner syndrome occur in patients with known risk factors for dissection, such as cardiovascular malformations (bicuspid aortic valve or coarctation of the aorta), systemic hypertension, or both. Familial thoracic aortic dissection syndrome. Pts with a family h/o AoD and no genetic syndrome present at a mean age younger than usual, but older than marfans or LDS. Inherited as autosomal dominant , with variable expression and decreased penetrance in females. Identified genes include TGFBR2, MYH11, ACTA2.

Other Genetic Syndromes With Increased Risk for Thoracic Aortic Aneurysms and Dissections

Autosomal dominant polycystic kidney disease, Noonan's syndrome , Alagille syndrome.

Bicuspid aortic valve

Aortic media degeneration due to activated MMP. About 15 % of AoD patients have BAV.

Aberrant right subclavian artery

In most adult patients, the aorta is also abnormal and is prone to aneurysm formation, dissection, and rupture.

Coarctation of aorta

Approximately one quarter of untreated pts will die of AoD or rupture.

Right aortic arch

The aorta with right-sided arches is also abnormal and typically very fragile and prone to AoD, rupture, or aneurysm formation.

Giant cell arteritis

In a population based study by Nuenninghoff et al the incidence the aortic aneurysm or dissection was seen in 18% of pts, with aneurysm formation per se not reducing survival,

Other vasculitis which have been associated more commonly with aortic aneursym, but also dissection are

Takayasu arteritis behcet disease Syphlitic aortitis Infectious vasculitis.

The presenting characteristics of pts in IRAD registry is as follows

Pain as a presenting complaint was seen in 96%

Stroke on presentation is seen in about 6% pts. Syncope as a presenting complaint was seen in 9.4% pts in IRAD registry

The typical pain is severe, often described as tearing, stabbing, ripping, sudden onset, and of maximum intensity right from the start. Migratory pain has been described as characteristic but was noted in only 16% of patients in IRAD. Chest pain was more common in type a, with anterior location of chest pain being more common than posterior location. Proportion of patients presenting with posterior or back pain was more in type b. Abdominal pain as a presenting complaint was more common in type b According to an IRAD analysis by park et al patients with painless AAD in IRAD registry (6.4%) had syncope, congestive heart failure, or stroke. Compared with patients who had painful AAD, patients who had painless AAD had higher mortality, especially when AAD is type B. Patients on steroids and patients with Marfan syndrome , diabetics and pts with prior cardiac surgery, may be more prone to present without pain. Painless aortic dissections present with syncope in one third, heart failure in 20%, and stroke in 11%.

Was much more commonly seen in type a than type b ( 12.7/ 4.1% ) Often indicates the development of dangerous complications such as cardiac tamponade and cerebral hypoperfusion.

Blood pressure

Where as a history of hypertension was present in about 72% pts in IRAD registry, SBP> 150 mm hg was present in 49% of patients, which was much more common in type b as compared to type a. ( 70% vs 35%). Pts of type a dissection on presentation had a much higher incidence of hypotension and shock. ( 25% vs 4 %) due to associated cardiac tamponade, acute aortic rupture, or heart failure related to acute severe aortic regurgitation. Pulse deficits were found in 15% pts in IRAD registry, being twice more common in type a that b ( 19% vs 9%). Presence of limb ischemia is associated with a significant increase in mortality. These pulse phenomena may be transient due to the intimal flaps changing position. In a pooled analysis by klompas et al, of 1500 pts neurological deficits were present in 17% of pts. Up to 50% of dissection-related neurologic symptoms may be transient and as many as one-third of patients with neurologic symptoms present without complaints of chest pain More common in type A dissections TIA is most common, followed by ischemic neuropathy. Paraplegia due to spinal cord ischemia is seen in 1-2 % pts. Seizures, global amnesia are other rare manifestations. Stroke more commonly affects the left hemisphere, as the left sided arch vessels are more susceptible to advancing false lumen. Much more common in type A than B ( 44% vs 12%) in IRAD registry When present in type b dissection usually suggests associated aortic valve disease or aortic root aneurysm.

Pulse deficits

Neurological deficits

Diastolic murmur of AR

On Retrospective analysis of risk score on IRAD database, sensitivity was 95.7%.

Acute aortic regurgitation

Heart Failure and Shock

Most commonly recognized cardiac complication of Type A dissection occurring in 41% to 76% of cases. Clinical manifestations of dissection-related aortic regurgitation span the spectrum from only a hemodynamically insignificant diastolic murmur to congestive heart failure and cardiogenic shock In this setting, heart failure may result from acute aortic insufficiency, acute myocardial ischemia or infarction, or cardiac tamponade. 6% pts in IRAD registry had CHF at presentation A retrospective analysis on IRAD database by januzzi et al showed that
presentation, frequently leading to a delay in diagnosis. more likely to present in shock but were less likely to complain of chest pain

patients with acute AoD complicated by heart failure are often atypical in their

Myocardial Ischemia or Infarction

IRAD

More commonly affects the rt coronary.

Ischemia 15.1 % Infarction 3.2 % ( more common with type a)

Pericardial Effusion and Tamponade

Frequent complication of acute Type A AoD ( seen in 8 10 %) and can occur via 2 distinct mechanisms.
Most commonly, transudation of fluid across the thin wall of a

false lumen into the pericardial space, leads to a hemodynamically insignificant pericardial effusion, present in about one third of patients. Less often, the dissected aorta ruptures directly into the pericardium, leading rapidly to tamponade physiology and hemodynamic compromise.

Occurs in approximately 13% of cases with multiple potential etiologies, including:

.

cardiac (eg, severe aortic regurgitation, ventricular outflow

Regardless of its etiology, syncope in the setting of AoD increases the risk of near-term adverse events.

obstruction, cardiac tamponade), vascular (eg, impaired cerebral blood flow and aortic baroreceptor activation); neurologic (eg, vasovagal in response to pain), and volume-related (eg, false lumen rupture into the pleural space) causes.

Pleural effusion, the most common pulmonary complication is noted in 16% of cases at presentation. Other pulmonary complications of acute AoD include dissection-related compression of the pulmonary artery and development of an aortopulmonary fistula, Hemoptysis, may result from compression of lung parenchyma by an expanding false lumen or via direct aneurysmal rupture into the lung.

Mesenteric ischemia is the most common gastrointestinal complication most common cause of death among those with Type B AoD. Gastrointestinal hemorrhage is a rare but potentially catastrophic complication

Cxr may demonstrate mediastinal widening, an abnormal aortic contour, or a left pleural effusion Sensitivity ( for thoracic Ao disease) : 64 71% Specificity : 86%

Calcium sign - separation of the intimal calcification from the outer aortic soft tissue border by 10 mm. About 12 15 % pts have a normal chest radiograph. inadequately sensitive to definitively exclude the presence of AoD in all except the lowest-risk patients

Technical aspects

Axial sections remain the mainstay of interpretation, using Helical CT scanners of 4 and greater detector rows. 2- and 3-dimensional reconstructions such as maximum intensity projection, multiplanar and curved multiplanar reformations, and volume rendering, may augment interpretation , although it has not been scientifically shown that the use of these tools increases diagnostic accuracy ECG gating is particularly useful for ascending aortic disease If appropriately acquired, an aorta CT and a complete CT coronary angiogram can be obtained in 1 CT acquisition. The sequence for CT performed in the potential setting of acute AoD generally would include a noncontrast study to detect subtle changes of IMH (Figure 7), followed by a contrast study to delineate the presence and extent of the dissection flap, identify regions of potential malperfusion, and demonstrate contrast leak indicating rupture. The scan coverage should start above the aortic arch and extend at least to the aortoiliac bifurcation, and probably the groin.

The diagnosis is based on the demonstration of an intimal flap which separates the true from the false channel. The flap is identified as a low attenuation linear structure in the aortic lumen. Secondary findings include internal displacement of intimal calcifications, delayed enhancement of the false lumen and aortic widening. Features that characterize the false lumen of an acute dissection include the wedge like angle (the beak sign) where the dissection flap meets the aortic wall, the presence of strandlike structures (cobwebs) in the lumen. In IRAD was the initially used modality in 61% pts. Reports of newer-generation multidetector helical CT scanners show sensitivities of up to 95% and specificities of 98% to 99%. Disadvantages include

Inability to evaluate the coronaries and aortic valve reliably. Motion artifacts Streak artifacts related to implanted devices.

Advantages include

Near universal availability The ability to image the entire aorta, including lumen, wall, and periaortic regions; To identify anatomic variants and branch vessel involvement; To distinguish among types of acute aortic syndromes (ie, intramural hematoma [IMH], penetrating atherosclerotic ulcer [PAU], and acute AoD) And the short time required to complete the imaging process and the 3-dimensional data.

Data from IRAD found that MR was the least-used imaging study, used in only 1.8% of patients as the initial diagnostic study. MR has been shown to be very accurate in the diagnosis of thoracic aortic disease, with sensitivities and specificities that are equivalent to or may exceed those for CT and TEE. Advantages of MR include the

Disadvantages Include

Ability to identify anatomic variants of AoD (IMH and PAU), Assess branch artery involvement, and diagnose aortic valve pathology and left ventricular dysfunction without exposing the patient to either radiation or iodinated contrast.

Prolonged duration of imaging acquisition during which the patient is inaccessible to care providers; Inability to use gadolinium contrast in patients with renal insufficiency; contraindication in patients with claustrophobia, metallic implants, or pacemakers; And lack of widespread availability on an emergency basis. Although time-resolved MR techniques are improving, MR often cannot clearly characterize the relationship of an intimal flap and aortic root structures, specifically the coronary arteries.

A comprehensive MR examination of the thoracic aorta may include many components, including
black blood imaging, noncontrast white blood imaging, contrast-enhanced MR angiography using gadolinium based agents and phase-contrast imaging.

MR examinations remain 2 to 4 times longer than CT examinations. Black Blood Imaging

is used to evaluate aortic anatomy and morphology (such as aortic size and shape), and the aortic wall for hematoma or other causes of thickening such as vasculitis. They may be repeated after the administration of gadolinium-based contrast agents to identify wall enhancement. These sequences should use ECG gating at end-diastole

Noncontrast White Blood Imaging

Signal is generated from blood, making it appear white in the absence of contrast.

Contrast-Enhanced Magnetic Resonance Angiography

usually performed with ECG gating. Although this increases acquisition time, it provides motion-free images of the aortic root and ascending aorta. For patients without a contraindicatio to receiving a gadolinium-based contrast agent, contrast-enhanced MR angiography is often the sequence of choice from which most of the diagnostic information is obtained.

Phase Contrast Imaging

These sequences are usually performed to evaluate gradients across an area of stenosis, across an intimal or cardiac valve. Image contrast is produced by differences in velocity. Images are obtained using ECG gating or triggering.

Angiography provides accurate information about the site of dissection, branch artery involvement, and communication of the true and false lumens. Additionally, angiographic and catheter-based techniques allow for evaluation and treatment of coronary artery and aortic branch (visceral and limb artery) disease, as well as assessment of aortic valve and left ventricular function. In IRAD, was used as an initial diagnostic modality in 4.4% pts. Disadvantages of angiography compared with other less invasive modalities include

1) not being universally available because it requires the presence of an experienced physician 2) being an invasive procedure that is time consuming and requires exposure to iodinated contrast; 3) having poor ability to diagnose IMH given a lack of luminal disruption; 4) potentially producing false negative results when a thrombosed false lumen prevents adequate opacification to identify the dissection; and 5) reported sensitivities and specificities of angiography for the evaluation of acute AoD that are slightly lower than those for the other less invasive modalities.

Thus, CT, MR, and TEE have replaced catheter-based angiography as the first-line diagnostic tests to establish the presence of the acute aortic syndrome. Multidetector CT has also largely replaced angiography for the anatomic studies required for treatment planning and monitoring of aortic disease

Doppler TEE is safe and can be performed at the bedside, with a low risk of complications (less than 1% overall, less than 0.03% for esophageal perforation), most of which are related to conscious sedation. TTE has a sensitivity of 77% to 80% and a specificity of 93% to 96% for identification of proximal AoD. For distal AoD, the sensitivity of TTE is lower. With TEE, sensitivity for proximal AoD is 88% to 98% with a specificity of 90% to 95%. Was used as an initial diagnostic modality in 33% pts in IRAD. Major advantages of TEE include

portability (allowing for bedside patient evaluation), rapid imaging time, and lack of intravenous contrast or ionizing radiation. Additionally, dissection-related cardiac complications can be evaluated including aortic regurgitation, proximal coronary artery involvement, and the presence of tamponade physiology.

Disadvantages of TEE include

a potential lack of availability (particularly at small centers and during off hours) and sedation requirements that may include endotracheal intubation. The accuracy of TEE can be quite operator dependent. For the very distal ascending aorta and the proximal aortic arch, TEE may be limited by a blind spot caused by interposition of the trachea and left main bronchus inability to visualize the abdominal aorta; if there is concern of a malperfusion syndrome, an abdominal CT may also be required.

Echocardiographic Criteria for Aortic Dissection

The echocardiographic diagnosis of an AoD requires the identification of a dissection flap separating true and false Lumens. However, one of the major limitations of both TTE and TEE is the frequent appearance of artifacts that mimic a dissection flap. These usually arise from a mirror image or reverberation artifact that appears as a mobile linear echodensity overlying the aortic lumen. It is therefore essential that the echocardiographer make certain to distinguish true dissection flaps from such artifacts.

The first step is to confirm the presence of the dissection flap

from several angles and from several transducer locations. The second step is to confirm that the dissection flap has a motion independent of surrounding structures and that the apparent flap is contained within the aortic lumen (ie, does not pass through the aortic wall in any view). The third step is to use color-flow Doppler to demonstrate differential flow on the 2 sides of the dissection flap.

The true lumen typically exhibits expansion during systole and collapse during diastole. Additional signs of the true lumen include

little or no spontaneous echocardiographic contrast, systolic jets directed away from the lumen, and forward flow during systole. diastolic expansion, evidence of spontaneous contrast, complete or partial thrombosis, and reversed, delayed, or absent blood flow.

The false lumen often exhibits

Associated echocardiographic findings of clinical importance include

identification of a pericardial effusion. information about right and left ventricular function and myocardial ischemia based on assessment of left ventricular segmental wall motion. On TEE, one can identify the ostia of the 2 coronary arteries and detect involvement by the dissection flap. presence of aortic regurgitation and permits grading of its severity.

In patients with hemodynamic instability, requiring close monitoring, bedside TEE is preferred to avoid moving the patient out of the acute care environment. In a meta-analysis by Shiga et al in 2006, sensitivity and specificity were comparable between imaging techniques, and the diagnostic value of each imaging technique was acceptable for confirming or ruling out thoracic aortic dissection. In patients at high risk for thoracic aortic dissection (pretest probability of 50%), MRI yielded the highest values for confirming thoracic aortic dissection. In contrast, helical CT yielded the best values for ruling out thoracic aortic dissection in patients at low risk for thoracic aortic dissection (pretest probability of 5%). However, capabilities of the 3 imaging techniques were found to be equivalent for confirming or excluding thoracic aortic dissection. The diagnosis of acute AoD cannot be excluded definitively based on the results of a single imaging study. Although TEE, CT, and MR are all highly accurate for the evaluation of acute AoD; falsenegative studies can and do occur and if a high clinical suspicion exists for acute AoD but initial aortic imaging is negative, strongly consider obtaining a second imaging study

In IRAD Bio a substudy of IRAD, by suzuki et al,

At a cutoff level of 500 ng/mL the negative predictive value was 95% , sensitivity was 96% and specificity was 61%. At a rule-in cutoff level of 1600 ng/mL identified patients with a high probability/ likelihood of AD.

The efficacy and safety of this strategy have not been tested in a large clinical trial, and the negative likelihood ratio provided by the most sensitive D-dimer assay is not of sufficient magnitude to provide useful information in highrisk individuals and therefore cannot be used to rule out the disease in this group.

Not routinely recommended as

Delays emergency surgery Has not proved to alter survival Coronary artery involvement can be usually identified and rectified at surgery Can be technically difficult, and is contraindicated in tamponade and aortic rupture Possible catheter and guidewire injuries. Other modalities like TEE can evaluate coronary ostia prior to, or during surgery

In IRAD, only 10% of patients underwent coronary angiography

Initial management is directed at

Limiting propagation of the false lumen by controlling aortic shear stress, Relieving pain Managing hypotension

Aortic wall stress is affected by the velocity of ventricular contraction (dP/dt), the rate of ventricular contraction, and blood pressure. Reasonable initial targets are a heart rate less than 60 bpm and a systolic blood pressure between 100 and 120 mm Hg. Intravenous propranolol, metoprolol, labetalol, or esmolol is an excellent choice for initial treatment. In patients who have a potential contraindication to beta blockade (eg, those with asthma, congestive heart failure, or chronic obstructive pulmonary disease), esmolol may be a viable option given its extremely short half-life. Use of labetalol, which is both an alpha- and beta-receptor antagonist, offers the advantage of potent heart rate and blood pressure control from a single agent, In patients who are unable to tolerate beta blockade, nondihydropyridine calcium channel antagonists (verapamil, diltiazem) offer an acceptable, although lessestablished, alternative. In addition to beta blockade, vasodilators may be required to control blood pressure. Intravenous sodium nitroprusside is the most established agent and offers the advantage of being rapidly titratable. Nicardipine, nitroglycerin, fenoldopam, ACE inhibitors are various other intravenous antihypertensive agents appropriate for this situation. Vasodilators without concomitant use of beta blockers not advised, for the possibility of reflex tachycardia.

Pain control To decrease sympathetic mediated increases in heart rate and blood pressure. Preferred drugs - opiates Hypotension Volume administration Vasopressors with caution Cardiac tamponade
Pericardiocentesis for dissection-related hemopericardium has been associated with recurrent pericardial bleeding and associated mortality. For patients with hemopericardium and cardiac tamponade who cannot survive until surgery, pericardiocentesis can be performed by withdrawing just enough fluid to restore perfusion.

Other cardiac complication that may result in hypotension include severe dissection-related aortic regurgitation, true lumen obstruction by a compressing false lumen, and acute MI. Ultimately, hypotension or shock in the acute AoD patient suggests the need for immediate operative management

Mortality in patients with type A dissection not receiving surgery was 58.0%, in contrast to surgically treated patients with type A dissection with 26% mortality. In an IRAD substudy by rampoldi et al the independent predictors of mortality were

Surgical treatment aims to treat or prevent the common and lethal complications such as aortic rupture stroke, visceral ischemia, cardiac tamponade, and circulatory failure. Essentially the surgery involves the following, as introduced by Dr De Bakey 50 yrs ago
Excision of the intimal tear, Obliteration of entry into the false lumen, and Reconstitution of the aorta with interposition of a synthetic graft with or without reimplantation of the coronary arteries. In addition, restoration of aortic valve competence is paramount in patients who develop aortic insufficiency.

If the ascending aortic and aortic root diameters are normal without downstream displacement of the coronary ostia, and with no commissural detachment of the aortic valve leaflets or other acute or chronic pathological changes of the leaflets, a tubular graft is usually anastomosed to the sinotubular ridge. Whenever one or more commissures are detached, the valve needs to be resuspended prior to graft insertion. If valve reconstruction appears unsafe, or if obvious congenital or acquired abnormalities are present, it is generally better to replace the valve before a supracommissural graft is inserted.

An acute type A (type I, II) dissection in a previously ectatic proximal aorta requires a different approach. In such instances this includes most patients with Marfans syndrome implantation of a composite graft (aortic valve plus ascending aortic tube graft) is recommended. In the original method described by Bentall and De Bono, the coronary ostia remain in continuity with the old aorta and are anastomosed directly to the ascending portion of the graft after valve implantation. If the coronary ostia are close to the aortic annulus and if a stiffened aortic wall creates tension on the ostia when they are relocated to the graft, they may be excised in button form before being anastomosed. The distal connection of the tube graft is made to the uninvolved ascending aorta in those relatively rare cases of limited type A (type II) dissection. In all other patients, replacement is carried to the junction of the ascending aorta and arch or beyond that level.

If the dissection has reached either ostium without disrupting the coronary vessel, the ostium can usually be preserved. An ostium completely surrounded by dissected aortic wall may be excised in button form. The dissected layers around the ostium are conjoined, using tissue adhesive and over and over suturing before the anastomosis to a tube graft is accomplished. Bypass grafting of coronary arteries using saphenous vein segments is limited to those instances where a small torn ostium with irreparable damage precludes secure reconstruction.

In IRAD substudy by rampoldi et al the surgical techniques carried out were as follows

Intraoperative factors associated with mortality were hypotension, need for coronary revascularisation, and RV dysfunction. Where as surgery requiring partial arch repair were associated with better outcomes.

Patients with type B dissection treated medically had the lowest mortality (10.7%) in IRAD registry. However, mortality for patients with type B dissection who underwent surgery was 31.4%. Patients with uncomplicated aortic dissections confined to the descending aorta are at present best treated with medical therapy. Medical therapy includes -blockers, other antihypertensives, and adequate analgesia to keep systolic blood pressure 120 mm Hg, or the lowest pressure that maintains end organ perfusion. The indications for interventions in type B dissection are persistent or recurrent pain, aortic expansion, dissection progression, and end-organ malperfusion syndromes.

Surgery to repair the descending thoracic aorta in this setting is associated with substantial mortality and morbidity, ad thus the option of less invasive endovascular repair was explored. A study by patel et al published in 2009, enrolling 69 pts presented a long-term analysis of an endovascular approach characterized by percutaneous intimal fenestrationand/or branchvessel stenting to restore end-organ perfusion. The 30-day mortality was 17.4% with a mean KaplanMeier survival of 7.3 years. Major periprocedural morbidity included stroke (4.3%), paraplegia (2.9%), and renal replacement therapy (15.9%). This paved the way for STABLE Trial.

The Stable trial prospective,nonrandomized, multicenter clinical trial conducted at institutions in the United States, Europe, and Australia. The studys endpoint was 30-day mortality; Other outcomes included clinical utility, adverse events, aortic remodeling, and device performance. Patients with cTBAD that warranted surgical intervention or did not respond well to medical management were included in the study. The 30-day mortality rate was 5% (2 of 40); two deaths occurred after 30 days, leading to a 1-year survival rate of 90%. Morbidity occurring within 30 days included stroke (7.5%), transient ischemic attack (2.5%), paraplegia (2.5%), retrograde progression of dissection (5%), and renal failure (12.5%).

The Device used is Zenith Dissectio Endovascular System (Cook Medical, Bloomington, Ind) is a dual-construct pathologyspecific device that provides proximal sealing of the primar entry tear with a covered stent graft and distal support of the dissected true lumen with bare metal stents. The Zenith Dissection Endovascular System comprises two modular components: the Zenith TX2 thoracic aortic aneurysm Endovascular Graft and the Zenith Dissection Endovascular Stent. Following deployment of the TX2 stent graft component to cover the primary entry tear, deployment of the bare stent component was recommended if branch vesse obstruction or false lumen perfusion persisted. Bare stent placement may not be necessary in patients for whom all of the following were applicable: (1) no signs or symptoms of obstructed/compromised branch vessels (if patient was included in the study for branch vessel obstruction/ compromise); (2) systolic pressure gradient 20 mm Hg between the aortic root and a distal obstructed aortic segment/vessel (if patient was included in the study for branch vessel obstruction/compromise); and (3) no false lumen flow through secondary re-entry tears. There was no limit to the number or the distal extent of dissection stent used; the decision to use the dissection stent and on the device combination was ultimately based on physician clinical judgment. Molding balloon angioplasty within the TX2 stent graft was optional, whereas it was discouraged within the bare dissection stent. Adjunctive stent placement for occluded or obstructed branch vessels was performed as necessary at the discretion of the operating physician.

Traditionally, stable patients are managed with medical treatment (annual survival rate 80%); however, long-term outcomes remain sobering because of aneurysmal expansion of the false lumen and late complications. Since open surgical technique is associated with unacceptable mortality, the last decade has witnessed remarkable advances in minimally invasive approaches to repair of distal aortic dissection using endovascular stentgraft techniques. INSTEAD Trial was the first randomized trial, evaluating the use of an endovascular approach in this setting.

Multicenter, randomized, controlled trial, enrolling 140 pts. Procedural aspects

TALENT stent grafts (Medtronic, Inc, Santa Rosa, Calif) were used both to scaffold 20 cm of dissected aorta and to seal major entries. 24 f Femoral artery sheath was placed to accommodate the 24F stent-graft system . The stent graft was deployed with systolic pressure lowered to 50 mm Hg by sodium nitroprusside or by rapid right ventricular pacing. After deployment, gentle inflation of a latex balloon was performed if proximal wall apposition was incomplete. Intentional coverage of the left subclavian artery was accepted to provide an appropriate landing zone and avoid endoleak; prophylactic surgical revascularization of the left subclavian artery was left to the discretion of the investigator.

The primary end point was all-cause death at 2 years, whereas aortarelated death, progression (with need for conversion or additional endovascular or open surgery), and aortic remodeling were secondary end points. There was no difference in all-cause deaths, with a 2-year cumulative survival rate of 95.62.5% with optimal medical therapy versus 88.93.7% with TEVAR (P0.15); the trial, however, turned out to be underpowered. Moreover, the aorta-related death rate was not different (P0.44), and the risk for the combined end point of aorta-related death (rupture) and progression (including conversion or additional endovascular or open surgery) was similar (P0.65). Three neurological adverse events occurred in the TEVAR group (1 paraplegia, 1 stroke, and 1 transient paraparesis), versus 1 case of paraparesis with medical treatment. Finally, aortic remodeling (with true-lumen recovery and thoracic falselumen thrombosis) occurred in 91.3% of patients with TEVAR versus 19.4% of those who received medical treatment (P0.001), which suggests ongoing aortic remodeling. A larger ADSORB trial, evaluating similar approach in the same patient paopulation is recuiting patients.

IRAD Follow up study by Tsai et el. Included 303 consecutive patients, discharged alive with TA-AAD enrolled in IRAD between 1996 and 2003. 90.1% patients had been managed surgically and 9.9% were managed medically. Survival for patients treated with surgery was 96.1% and 90.5 at 1 and 3 years versus 88.6% and 68.7% without Surgery. Multivariate analysis identified a history of atherosclerosis and previous cardiac as significant, independent predictors of followup mortality.

IRAD follow up study by Tsai et al. Examined 242 consecutive patients discharged alive with acute type B aortic dissection enrolled between 1996 and 2003. Three-year survival for patients treated medically, surgically, or with endovascular therapy was 77.66.6%, 82.818.9%, and 76.225.2%, respectively. Independent predictors of follow-up mortality included female gender, a history of prior aortic aneurysm, a history of atherosclerosis, in-hospital renal failure and pleural effusion on chest radiograph and in-hospital hypotension/shock.

Tenets of medical follow up are

Medical therapy
Antihypertensive therapy Beta blockers ? ARB in marfans syndrome

Screening first degree relatives in genetically triggered dissecctions Serial imaging of the aorta over time
At 1,3,6,12,18 and 24 months. And then yearly.
MRI/CT

Lifestyle modification

Acute aortic dissection, though rare, is the most common life-threatening disorder affecting the aorta. The immediate mortality rate in aortic dissection is as high as 1% per hour over the first several hours, making early diagnosis and treatment critical for survival. About 20-25% pts die of the disease before reaching the hospital. The symptoms of aortic dissection may be highly variable and may mimic much more common conditions. Thus, a high index of suspicion must be maintained, especially when risk factors for dissection are present or signs and symptoms suggest this possibility. Prompt diagnostic imaging, blood pressure optimization and surgical consultation with early intervention can reduce mortality. Endovascular intervention, a promising techniques is in evolutionary stages. With proper management and follow up life expectancy after discharge is close to that of the normal population.