Aortic dissection

About 96% of individuals with AD present with severe pain that had a sudden onset. The pain may be described as a tearing, stabbing, or sharp sensation in the chest, back, or abdomen. About 17% of individuals feel the pain migrate as the dissection extends down the aorta. The location of pain is associated with the location of the dissection. Anterior chest pain is associated with dissections involving the ascending aorta, while interscapular (between the scapula bones in the back) back pain is associated with descending aortic dissections. If the pain is pleuritic in nature, it may suggest acute pericarditis caused by bleeding into the sac surrounding the heart. This is particularly dangerous, suggesting that acute pericardial tamponade may be imminent. Pericardial tamponade is the most common cause of death from AD. While the pain may be confused with that of a heart attack, AD is usually not associated with the other suggestive signs, such as heart failure and ECG changes. Less common symptoms that may be seen in the setting of AD include congestive heart failure (7%), fainting (9%), stroke (6%), ischemic peripheral neuropathy, paraplegia, and cardiac arrest. If the individual fainted, about half the time it is due to bleeding into the pericardium, leading to pericardial tamponade. Neurological complications of aortic dissection, such as stroke and paralysis, are due to the involvement of one or more arteries supplying portions of the brain. If the AD involves the abdominal aorta, compromise of one or both renal arteries occurs in 5–8% of cases which may cause kidney damage, while ischemia of the intestines occurs about 3% of the time. Blood pressure People with AD often have a history of high blood pressure. The blood pressure is quite variable at presentation with acute AD. It tends to be higher in individuals with a distal dissection. In individuals with a proximal AD, 36% present with hypertension, while 25% present with hypotension. Proximal AD tends to be associated with weakening of the vascular wall due to cystic medial degeneration. In those who present with distal (Stanford type B) AD, 60–70% present with high blood pressure, while 2–3% present with low blood pressure. Severe hypotension at presentation is a grave prognostic indicator. It is usually associated with pericardial tamponade, severe aortic insufficiency, or rupture of the aorta. Aortic insufficiency Aortic insufficiency (AI) occurs in half to two-thirds of ascending AD, and the diastolic heart murmur of aortic insufficiency is audible in about 32% of proximal dissections. The intensity (loudness) of the murmur depends on the blood pressure and may be inaudible in the event of low blood pressure. Multiple causes exist for AI in the setting of ascending AD. The dissection may dilate the annulus of the aortic valve, preventing the leaflets of the valve from coapting. The dissection may extend into the aortic root and detach the aortic valve leaflets. Alternatively, following an extensive intimal tear, the intimal flap may prolapse into the left ventricular outflow tract, causing intimal intussusception into the aortic valve, thereby preventing proper valve closure. Myocardial infarction Heart attack occurs in 1–2% of aortic dissections. Infarction is caused by the involvement of the coronary arteries, which supply the heart with oxygenated blood, in the dissection. The right coronary artery is involved more commonly than the left coronary artery. If the myocardial infarction is treated with thrombolytic therapy, the mortality increases to over 70%, mostly due to bleeding into the pericardial sac, causing cardiac tamponade.{{cite journal |url= https://www.ncbi.nlm.nih.gov/books/NBK459269/ |last1= Mechanic |first1= O |last2= Gavin |first2= M | last3= Grossman | first3= S |date= 9 March 2021 | title= Acute Myocardial Infarction == Predisposing factors ==

Aortic dissection is associated with hypertension (high blood pressure) and many connective tissue disorders. Vasculitis (inflammation of an artery) is rarely associated with aortic dissection. It can also be the result of chest trauma. About 72 to 80% of individuals who present with an aortic dissection have a previous history of hypertension. Use of stimulants such as cocaine and methamphetamine is also a modifiable risk factor for AD. It can also be caused by smoking. A bicuspid aortic valve (a type of congenital heart disease involving the aortic valve) is found in 7–14% of individuals who have an aortic dissection. These individuals are prone to dissection in the ascending aorta. The risk of dissection in individuals with bicuspid aortic valves is not associated with the degree of stenosis of the valve. Connective tissue disorders such as Marfan syndrome, Ehlers–Danlos syndrome, and Loeys–Dietz syndrome increase the risk of aortic dissection. Turner syndrome also increases the risk of aortic dissection, by aortic root dilatation. Syphilis only potentially causes aortic dissection in its tertiary stage. ==Pathophysiology==

As with all other arteries, the aorta is made up of three layers, the intima, the media, and the adventitia. The intima is in direct contact with the blood inside the vessel, and mainly consists of a layer of endothelial cells on a basement membrane; the media contains connective and muscle tissue, and the vessel is protected on the outside by the adventitia, comprising connective tissue. This can propagate along the length of the aorta for a variable distance forward or backward. Dissections that propagate towards the iliac bifurcation (with the flow of blood) are called anterograde dissections and those that propagate towards the aortic root (opposite of the flow of blood) are called retrograde dissections. The initial tear is usually within 100 mm of the aortic valve, so a retrograde dissection can easily compromise the pericardium leading to a hemopericardium. Anterograde dissections may propagate all the way to the iliac bifurcation of the aorta, rupture the aortic wall, or recanalize into the intravascular lumen leading to a double-barrel aorta. The double-barrel aorta relieves the pressure of blood flow and reduces the risk of rupture. Rupture leads to hemorrhaging into a body cavity, and prognosis depends on the area of rupture. Retroperitoneal and pericardial ruptures are both possible. The initiating event in aortic dissection is a tear in the intimal lining of the aorta. Due to the high pressures in the aorta, blood enters the media at the point of the tear. The force of the blood entering the space between the intima and media layers causes the tear to extend. It may extend proximally (closer to the heart) or distally (away from the heart) or both. The blood travels through the space, creating a false lumen (the true lumen is the normal conduit of blood in the aorta). Separating the false lumen from the true lumen is a layer of intimal tissue known as the intimal flap. Two-thirds of aortic dissections involve the ascending aorta, the rest involve only the descending aorta (type B aortic dissections). While it is not always clear why an intimal tear may occur, quite often it involves degeneration of the collagen and elastin that make up the media. This is known as cystic medial necrosis and is most commonly associated with Marfan syndrome and is also associated with Ehlers-Danlos syndrome.{{cite web |url= https://omim.org/entry/607086 | title= Aortic Aneurysm, Familial Thoracic 1; AAT1 In about 13% of aortic dissections, no evidence of an intimal tear is found. In these cases, the inciting event is thought to be an intramural hematoma (caused by bleeding within the media). Since no direct connection exists between the true lumen and the false lumen in these cases, diagnosing an aortic dissection by aortography is difficult if the cause is an intramural hematoma. An aortic dissection secondary to an intramural hematoma should be treated the same as one caused by an intimal tear. ==Diagnosis==

Because of the varying symptoms of aortic dissection, the diagnosis is sometimes difficult to make. Concern should be increased in those with low blood pressure, neurological problems, and unequal pulses. The diagnosis of aortic dissection is made by visualization of the intimal flap on a diagnostic imaging test. The gold standard test is CT angiography of the aorta. Other possible tests include a CT scan of the chest, cardiac magnetic resonance (MRI), or echocardiography. D-dimer A measurement of blood D-dimer level may be useful in diagnostic evaluation. A level less than 500 ng/ml may be considered evidence against a diagnosis of aortic dissection, and within 24 hours of symptom onset. The American Heart Association does not advise using this test in making the diagnosis, as evidence is still tentative. Chest X-ray . According to the American Heart Association (AHA) a chest x-ray should not be used to diagnose or assess for aortic dissections. The AHA states that chest x-rays are not adequately sensitive nor specific to accurately diagnose aortic dissections. However, certain findings on a chest x-ray may suggest an aortic dissection. These findings include: widening of the mediastinum, disruption of the normal contour of the aortic knob, the calcium sign (a separation of more than 5 mm of the calcified intimal plaque layer from the aortic wall), a "double density" seen in the aorta, and deviation of the trachea to the right. File:DissectionCT.png|CT with contrast demonstrating aneurysmal dilation and a dissection of the ascending aorta (type A Stanford) File:Descending (Type B Stanford) Aortic Dissection.PNG|Chest CT with descending (type B Stanford) aortic dissection (red circle) File:Dissection2018WithPericardial.jpg|Type A dissection with pericardial effusion as a result. MRI of an aortic dissection {{ordered list Magnetic resonance imaging (MRI) has a sensitivity of 97-100% and specificity of 94-100% in the diagnosis of aortic dissections. File:UOTW 17 - Ultrasound of the Week 2.webm|Type A aortic dissection File:UOTW 55 - Ultrasound of the Week 3.webm|Dissection of both the thoracic and abdominal aorta Aortogram An aortogram involves injection of contrast material while taking X-rays of the aorta. Once commonly used, its use has since been supplanted by less invasive and more sensitive imaging modalities. The American Heart Association does not recommend the use of aortograms as a first line imaging modality in the diagnoses of aortic dissections. They note that aortograms have limited sensitivity and are more invasive than other tests. == Classification ==

Several different classification systems have been used to describe aortic dissections. One such classification is based on chronicity and labels aortic dissections as hyperacute (30 days). The systems commonly in use are based on either the anatomy of the dissection or the duration of onset of symptoms before the presentation. The Stanford system is used more commonly now, as it is more attuned to the management of the patient. DeBakey The DeBakey system, named after cardiothoracic surgeon Michael E. DeBakey, is an anatomical description of the aortic dissection. It categorizes the dissection based on where the original intimal tear is located and the extent of the dissection (localized to either the ascending aorta or descending aorta or involving both the ascending and descending aorta). • Type I – originates in ascending aorta, and 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 the ascending aorta and is confined to it. • Type III – originates in the descending aorta and rarely extends proximally, but will extend distally. It most often occurs in elderly patients with atherosclerosis and hypertension. Stanford The Stanford classification is divided into two groups, A and B, depending on whether the ascending aorta is involved. • A – TAAD involves the ascending aorta and/or aortic arch, and possibly the descending aorta. The tear can originate in the ascending aorta, the aortic arch, or more rarely, in the descending aorta. It includes DeBakey types I and II. • B – TBAD involves the descending aorta or the arch (distal to the left subclavian artery), without the involvement of the ascending aorta. It includes DeBakey type III. The Stanford classification is useful as it follows clinical practice, as type A ascending aortic dissections generally require primary surgical treatment, whereas type B dissections generally are treated medically as initial treatment with surgery reserved for any complications.{{cite web |url= https://www.lecturio.com/concepts/aortic-dissection/ | title= Aortic Dissection The main indication for surgical repair of type A dissections is the prevention of acute hemorrhagic pericardial tamponade due to leakage of blood through the dissected layers of the intrapericardial proximal aorta. A secondary indication is acute aortic valve insufficiency (regurgitation): ascending aortic dissections often involve the aortic valve, which, having lost its suspensory support, telescopes down into the aortic root, resulting in aortic incompetence. The valve must be resuspended to be reseated, as well as to repair or prevent coronary artery injury. Also, the area of dissection is removed and replaced with a Dacron graft to prevent further dissection from occurring. However, type B dissections are not improved, from a mortality point of view, by the operation, unless leaking, rupture, or compromise to other organs, e.g. kidneys, occurs. ==Prevention==

Among the recognized risk factors for aortic dissection, hypertension, abnormally high levels of lipids (such as cholesterol) in the blood, and smoking tobacco are considered preventable risk factors. ==Management==

In an acute dissection, treatment choice depends on its location. For Stanford type A (ascending aortic) dissection, surgical management is superior to medical management. Complicated Stanford type B aortic dissections require surgical intervention after initiation of medical therapy, with endovascular stent-grafting (TEVAR) available as a less invasive alternative to surgery. If the individual has refractory hypertension (persistent hypertension on the maximum doses of three different classes of antihypertensive agents), involvement of the renal arteries in the aortic dissection plane should be considered. Surgical Indications for the surgical treatment of aortic dissection include an acute proximal aortic dissection and an acute distal aortic dissection with one or more complications. Complications include compromise of a vital organ, rupture or impending rupture of the aorta, retrograde dissection into the ascending aorta. These are more common with a history of Marfan syndrome or Ehlers-Danlos syndrome. The objective in the surgical management of aortic dissection is to resect (remove) the most severely damaged segments of the aorta and to obliterate the entry of blood into the false lumen (both at the initial intimal tear and any secondary tears along the vessel). While excision of the intimal tear may be performed, it does not significantly change mortality. The particular treatment used depends on the segment or segments of the aorta involved. Some treatments are: • Open aortic surgery with replacement of the damaged section of the aorta with a tube graft (often made of Dacron) when no damage to the aortic valve is seen • Bentall procedure – replacement of the damaged section of the aorta and replacement of the aortic valve • David procedure – replacement of the damaged section of the aorta and reimplantation of the aortic valve • Thoracic endovascular aortic repair, a minimally invasive surgical procedure usually combined with ongoing medical management • Frozen elephant trunk procedure (FET) is one-stage procedure for the repair of acute aortic dissection that permits concurrent total aortic arch replacement with antegrade delivery of a descending aortic stent-graft which itself functions as a proximal landing zone to facilitate prospective endovascular intervention (TEVAR) to treat residual or de novo disease in the more distal aorta. A number of comorbid conditions increase the surgical risk of repair of an aortic dissection. These conditions include the following: • Prolonged preoperative evaluation (increased length of time prior to surgery) • Advanced age • Comorbid disease states (e.g.: coronary artery disease) • Aneurysm leakage • Cardiac tamponade • Shock - obstructive shock • Past history of myocardial infarction • History of kidney failure (either acute or chronic kidney failure) Follow-up Patients who have suffered aortic dissection are at risk of aortic aneurysm formation at the site of the dissection, thought to be due to weakening of the aortic wall. The risk of this aneurysm degeneration is 10 times higher in individuals who have uncontrolled hypertension, compared to individuals with a systolic pressure below 130 mmHg. Regarding long term mortality after aortic dissection, the risk of death is highest in the first two years after the acute event. About 29% of late deaths following surgery are due to rupture of either a dissecting aneurysm or another aneurysm. The rate of aortic aneurysm formation after dissection in the affected section of aorta is 25-40%. Other studies estimate a 17% to 25% incidence of new aneurysm formation, typically due to dilatation of the residual false lumen. These new aneurysms are more likely to rupture, due to their thinner walls. Serial imaging of the aorta is recommended after dissection to assess for progression of the dissection, durability of the repair, screen for aneurysm formation, and assess aortic remodeling. Repeat imaging of the aorta after type B dissections is recommended at 1, 6, 12 months after diagnosis and then yearly. ==Prognosis==

Of all people with aortic dissection, 40% die immediately and do not reach a hospital in time. 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 surgery is decided to be not appropriate, 75% die within 2 weeks. With aggressive treatment, 30-day survival for thoracic dissections may be as high as 90%. ==Epidemiology==

Establishing the incidence of aortic dissection has been difficult because many cases are only diagnosed after death (which may have been attributed to another cause), and is often initially misdiagnosed. Aortic dissection affects an estimated 2.0–3.5 people per 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. Dissection occurs in about 0.0004% of pregnancies. ==History==

The earliest fully documented case of aortic dissection is attributed to Frank Nicholls in his autopsy report of King George II of Great Britain, who had been found dead on 25 October 1760; the report describes a dissection of the aortic arch and into the pericardium. The term "aortic dissection" was introduced by the French physician J. P. Maunoir in 1802, and René Laennec labeled the condition "dissecting aneurysm". London cardiologist Thomas Bevill Peacock contributed to the understanding of the condition by publishing two series of the cases described in the literature so far: 19 cases in an 1843 review, and 80 in 1863. The characteristic symptom of tearing pain in the chest was recognized in 1855 when a case was diagnosed in life. Surgery for aortic dissection was first introduced and developed by Michael E. DeBakey, Denton Cooley, and Oscar Creech, cardiac surgeons associated with the Baylor College of Medicine, Houston, Texas, in 1954. DeBakey developed aortic dissection himself at age 97 in 2005, and underwent surgery in 2006. Endovascular treatment of aortic dissection was developed in the 1990s. ==Notable cases==

• Muhammad, founder of Islam, reportedly died from his aorta being severed. • Lucille Ball was diagnosed with a dissecting aortic aneurysm and underwent a seven hour surgery to repair her aorta and replace her aortic valve. She died after surgery due to an unrelated abdominal aortic aneurysm that had burst. • Playwright Jonathan Larson, best known for the musical Rent, died in 1996 of an aortic dissection believed to be due to undiagnosed Marfan syndrome. • Actor John Ritter died from a thoracic aortic dissection in 2003 after he was misdiagnosed and mistakenly treated for a heart attack. The Ritter Rules were developed to help people spot the early signs of an aortic dissection. • Days of Our Lives and Babylon 5 actor Richard Biggs died in 2004, at the age of 44 due to complications from aortic dissection. • Lux Interior of The Cramps died at the age of 62 following an aortic dissection. • Alan Thicke died in 2016 of type-A aortic dissection at the age of 69. • Japanese actress Hiromi Tsuru died in her car from aortic dissection in 2017 at the age of 57. • Taiwanese entertainer Alien Huang died of an aortic dissection in 2020 at the age of 36. • Kentaro Miura, writer and artist of the manga Berserk, died from aortic dissection in 2021 at the age of 54. • In August 2021, New Zealand cricketer Chris Cairns was paralyzed from the waist down after an aortic dissection. • Guitarist Richie Faulkner of the English heavy metal band Judas Priest suffered an aortic dissection whilst performing at a concert. • In 2022, keyboardist Andy Fletcher (a founding member of the UK band Depeche Mode) died unexpectedly from an aortic dissection at the age of 60. ==See also==