1. AORTIC DISSECTION Dr. M. SOFI MD; FRCP (London); FRCPEdin; FRCSEdin

2. AORTIC DISSECTION Aortic dissection is defined as separation of the layers within the aortic wall. The primary event in aortic dissection is a tear in the aortic intima. Degeneration of the aortic media, or cystic medial necrosis, is felt to be a prerequisite for the development of non-traumatic aortic dissection. Blood passes into the aortic media through the tear, separating the intima from the surrounding media and/or adventitia, and creating a false lumen. Mortality is still high despite advances in diagnostic and therapeutic modalities

3. Dissection of the descending part of the aorta (3), which starts from the left subclavian artery and extends to the abdominal aorta (4). The ascending aorta (1) and aortic arch (2) are not involved.

4. Sudden onset of severe chest pain that often has a tearing or ripping quality Chest pain may be mild Anterior chest pain: Usually associated with anterior arch or aortic root dissection Neck or jaw pain: Aortic arch involvement and extension into the great vessels Tearing or ripping intra- scapular pain: indicate dissection involving the descending aorta No pain in about 10% of patients Syncope Signs and symptoms Aortic dissection can be rapidly fatal, with many patients dying before presentation to the emergency department or before diagnosis is made in the ED.

5. CVA symptoms: hemianesthesia, and hemiparesis, hemiplegia) Altered mental status Numbness and tingling, pain, or weakness in the extremities Horner syndrome (ptosis, miosis, anhidrosis) Dyspnea Hemoptysis Dysphagia Flank pain (with renal artery involvement Abdominal pain (with abdominal aorta involvement) Fever Anxiety and premonitions of death Signs and symptoms

6. Hypertension/Hypotension Inter-arm blood pressure differential greater than 20 mm Hg Signs of aortic regurgitation (bounding pulses, wide pulse pressure, diastolic murmurs) Cardiac tamponade (muffled heart sounds, hypotension, pulsus paradoxus, jugular venous distention, Kussmaul sign) Neurologic deficits (e.g., syncope, altered mental status) Peripheral paresthesias Horner syndrome New diastolic murmur Asymmetrical pulses (e.g., carotid, brachial, femoral) Progression or development of bruits Possible physical examination findings include:

7. Leukocytosis: Stress state Decreases in hemoglobin and hematocrit values: Leaking or rupture of the dissection Elevation of the BUN and creatinine levels: Renal artery involvement or prerenal azotemia Elevation of the cardiac enzymes, myoglobin, and troponin I and T levels: Myocardial ischemia from coronary artery involvement LDH: Hemolysis in false lumen Smooth muscle myosin heavy-chain assay: levels in the first 24 hours are 90% sensitive and 97% specific FDP elevation: FDP of 12.6 μg/mL or higher suggests possible aortic dissection with a patent false lumen FDP level of 5.6 μg/mL or higher suggests possibility of dissection with complete thrombosis of false lumen Laboratory findings include the following :

8. Aortic dissection is often associated with hypertension Chest trauma. 72 to 80% of individuals have a previous history of hypertension. A bicuspid aortic valve (a type of congenital heart disease involving the aortic valve) is found in 7–14%. Risk is not associated with the degree of stenosis of the valve. Marfan syndrome is noted in 5–9%. Individuals with Marfan syndrome tend to have aneurysms of the aorta and are more prone to proximal dissections of the aorta. Causes

9. Chest trauma can be divided into two groups based on etiology: – blunt chest trauma seen in car accidents. – Iatrogenic causes include trauma during cardiac catheterization or due to an intra-aortic balloon pump. Aortic dissection may be a late sequela of cardiac surgery. 18% of individuals who present have a history of open heart surgery. Turner’s syndrome also increases the risk of aortic dissection, by aortic root dilatation. Causes

10. Aorta is made up of three layers, the intima, the media, and the adventitia. The intima is in direct contact with blood and consists of a layer of endothelial cells on a basement membrane; Media contains connective and muscle tissue Adventitia, comprising connective tissue outer layer Blood penetrates the intima and enters the media layer. The high pressure rips the tissue of the media apart along the laminated plane splitting the inner 2/3 and the outer 1/3 of the media apart. This can propagate along the length of the aorta for a variable distance forward or backwards Pathophysiology

11. The initiating event is a tear in the intimal lining of the aorta. High pressure blood enters the media at the point of the tear. The force of the blood causes the tear to extend. May extend proximally or distally or both. The blood traveling through the media, creating a false lumen separating from the true lumen is a layer of intimal tissue. This tissue is known as the intimal flap. The majority of dissection are in – ascending aorta (65%) – aortic arch (10%), – descending thoracic aorta (20%). Pathophysiology

12. AUTOPSYHEARTAUTOPSYHEART Dissection media as well outer media

13. Blood penetrates the intima and enters the media layer. Pathophysiology : The aortic dissections originate with an intimal tear in: 1.Ascending aorta (65%) 2.Aortic arch (10%) 3.Descending thoracic aorta (20%) 4.No evidence of tear (13%)

14. An aneurysm is a localized abnormal dilation of a blood vessel. Dissection of blood vessel along the laminar planes of arterial media with the formation of a blood-filled channel. Cystic medial degeneration - weakened arterial wall - hemodynamic factors (H/T) - intimal tear - dissecting aneurysm -reenter the lumen distally to create a second channel, or rupture through the vessel wall. AORTIC DISSECTION

15. Location of thoracic aortic aneurysm Causes associated with aneurysm type Ascending thoracic aneurysm  Cystic medial degeneration (necrosis). This is the breaking down of the tissue of the aortic wall. This is the most common cause of this type of thoracic aortic aneurysm.  Genetic disorders, which affect the connective tissue, such as Marfan syndrome and Ehlers-Danlos syndrome  Family history of thoracic aortic aneurysm with no incidence of Marfan syndrome  Atherosclerosis. This is hardening of the arteries caused by a build-up of plaque in the inner lining of an artery. This is a rare cause of ascending thoracic aortic aneurysm.  Infection, syphilis (rare causes of thoracic aortic aneurysm) Aortic arch thoracic aneurysm  Takayasu's arteritis. A type of vasculitis that causes inflammation of the arteries  Atherosclerosis  Continuation of an ascending and/or descending aortic aneurysm Descending thoracic aortic aneurysm Atherosclerosis is most often associated with descending thoracic aneurysms, and is thought to play an important role in aneurysmal disease, including the risk factors associated with atherosclerosis. Thoracic aortic aneurysm: Thoracic aortic aneurysms may be caused by different disease processes, especially in respect to their location. Examples of different locations of thoracic aortic aneurysms and their causes may include:

16. Diagnosis The diagnosis of acute aortic dissection requires a high index of suspicion and involves the following: History and physical examination Imaging studies Chest X-Ray CT with contrast MRI Aortoraphy Electrocardiography Complete blood count, serum chemistry studies, cardiac marker assays

17. Chest radiography: Initial imaging technique if it is readily available at the bedside Widening of the mediastinum is the classic finding Hemothorax may be evident if the dissection has ruptured CT with contrast: The definitive test in most patients with suspicion of aortic dissection Useful only in hemodynamically stable patients Findings help determine whether hypothermic circulatory arrest is necessary for surgery Imaging studies

18. Echocardiography: Transesophageal echocardiography (TEE) is more accurate than transthoracic echocardiography (TTE) TTE is most useful in ascending aortic dissections TEE is as sensitive and specific as CT scanning and MRI TEE is strongly dependent on operator experience MRI: The most sensitive method for diagnosing aortic dissection Specificity is similar to that of CT scanning Aortography: Has been the diagnostic criterion standard study for aortic dissection Is being replaced by newer, safer imaging modalities Imaging studies

19. Electrocardiogram of a patient presenting to the ED with chest pain; this patient was diagnosed with aortic dissection.

20. Mediastinum widening: CXR has moderate sensitivity in an ascending aortic dissection. Pleural effusions may be seen on CXR. Commonly in descending aortic dissections. Typically in left hemi-thorax. Obliteration of the aortic knob, depression of the left mainstream bronchus, loss of the para-tracheal stripe, and tracheal deviation. About 12 to 20% of individuals presenting with an aortic dissection have a "normal" chest x-ray Chest radiograph demonstrating widened mediastinum in a patient with aortic dissection. Chest X-Ray

21. The calcium sign on CXR suggests aortic dissection. It is the separation of the intimal calcification from the outer aortic soft tissue border by 10 mm Calcium sign: Aortic dissection

22. Chest x-rays. A dissected aorta can be seen in (b) (arrow).

23. CT is a fast non-invasive test that will give an accurate three-dimensional view of the aorta. Contrast is injected and the scan performed using a Bolus Tracking method. This is a type of scan timed to an injection to capture the contrast as it enters the aorta. The scan will then follow the contrast as it flows though the vessel. It has a sensitivity of 96 to 100% and a specificity of 96 to 100%. Disadvantages include the need for iodinated contrast material and the inability to diagnose the site of the intimal tear. CT with contrast demonstrating aneurysmal dilation and a dissection of the ascending aorta (Type A Stanford) Computed Tomography

24. Contrast-enhanced axial CT image demonstrates an intimal flap that separates the two channels in the ascending and descending aorta diagnostic of a Stanford type A dissection Contrast-enhanced CT scan obtained at the level of the aortic arch demonstrates an aortic dissection with almost complete separation of the aortic intima. The slight prolapse may be the beginning of a configuration at risk for intimo-intimo intussusception, a potentially fatal event.

25. An echocardiogram displaying the true lumen and false lumen of an aortic dissection. In the image to the left, the intimal flap can be seen separating the two lumens. In the image to the right, color flow during ventricular systole suggests that the upper lumen is the true lumen The transesophageal echocardiogram (TEE) is a relatively good test in the diagnosis of aortic dissection, with a sensitivity of up to 98% and a specificity of up to 97%. It has become the preferred imaging modality for suspected aortic dissection.

26.  (MRI) is currently the gold standard test, sensitivity of 98% and a specificity of 98%.  An MRI, allowing the physician to determine the:  Location of the intimal tear  Involvement of branch vessels  Locate any secondary tears.  Detect and quantitate the degree of aortic insufficiency. MRI of an aortic dissection 1 Aorta descends with dissection 2 Aorta isthmus

27. Classification Type I – Originates in ascending aorta, propagates at least to the aortic arch and often beyond it distally. It is most often seen in patients less than 65 years of age and is the most lethal form of the disease. Type II – Originates in ascending aorta and is confined to the ascending aorta. Type III – Originates in descending aorta, rarely extends proximally but will extend distally. It most often occurs in elderly patients with atherosclerosis and hypertension. Percentage60%10 -15%25 – 30 % TypeDeBakey IDeBakey IIDeBakey III Stanford A (Proximal)Stanford B Distal

28. Medical treatment includes: Lowering the BP and the shearing forces of myocardial contractility Antihypertensive therapy beta blockers, is the treatment of choice for all stable chronic aortic dissections Pain management: Narcotics and opiates are the preferred agents Emergency surgical correction is the preferred treatment for: Stanford type A (DeBakey type I and II) ascending aortic dissection Complicated Stanford type B (DeBakey type III) aortic dissections with specific clinical or radiologic evidence Management: The management is medical /surgical. In surgical treatment, the area of the aorta with the intimal tear is usually resected and replaced with a Dacron graft. Endovascular repair is emerging as the preferred treatment for descending aortic dissection.

29. In acute dissection treatment choice depends on its location. 1.Stanford type A dissection, surgical management is superior to medical management. 2.Stanford type B dissections medical management is preferred over surgical. The risk of death due to aortic dissection is highest in the first few hours Acute dissection is one in which the individual presents within the first two weeks. About 66% of all dissections present in the acute phase. Those who present two weeks after the onset of the dissection are said to have chronic aortic dissections. Can be treated with medically Management

30. Beta blockers are first line treatment for acute and chronic In acute dissection, rapidly acting, titratable parenteral agents (such as esmolol, or labetalol) Vasodilators such as sodium nitroprusside for ongoing hypertension, but they should never be used alone. Calcium channel blockers can be used if there is a contraindication to the use of beta blockers. The calcium channel blockers typically used are verapamil and diltiazem, for their combined vasodilator and negative inotropic effects. Management

31. Indications for the surgical treatment include: Acute proximal aortic dissection Acute distal aortic dissection with complications. Complications include: Risk of a vital organ damage Rupture of the aorta Retrograde dissection ascending aorta Marfan syndrome Ehlers-Danlos Syndrome The objective in the surgical Rx Resect the most severely damaged segments of the aorta Obliterate blood into false lumen Surgical Treatment

32. The treatment depends on the segment or segments of aorta involved. Some treatments are Open Aortic surgery with replacement of the damaged section of aorta with a dacron tube graft when there is no damage to the aortic valve. Bentall procedure — Replacement of the damaged section of aorta and replacement of the aortic valve. David procedure — Replacement of the damaged section of aorta and reimplantation of the aortic valve. Endovascular repair via stent graft (covered stent), Surgical Treatment

33. It has been difficult to establish because many cases are only diagnosed after death. It is estimated that aortic dissection affects 2–3.5 people out of every 100,000 every year. Studies from Sweden suggest that the incidence of aortic dissection may be rising. Men are more commonly affected than women: 65% of all people with aortic dissection are male. The mean age at diagnosis is 63 years. In females before the age of 40, half of all aortic dissections occur during pregnancy (typically in the 3rd trimester or early post partum period). Epidemiology & Prognosis

34. Of all people with aortic dissection, 40% die almost straight away and do not reach hospital. Of the remainder, 1% die every hour, making prompt diagnosis and treatment a priority. Even after diagnosis, 5– 20% die during surgery or in the immediate postoperative period. In ascending aortic dissection, if there is a decision that surgery is not appropriate, 75% die within 2 weeks. With aggressive treatment 30-day survival for thoracic dissections may be as high as 90% Epidemiology & Prognosis