Aortic aneurysm | |
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Figure A shows a normal aorta. Figure B shows a thoracic aortic aneurysm (which is located behind the heart). Figure C shows an abdominal aortic aneurysm located below the arteries that supply blood to the kidneys. | |
Specialty | Cardiology, Vascular surgery |
Symptoms | abdominal pain and back pain |
Complications | Hemorrhaging |
Diagnostic method | ultrasound |
An aortic aneurysm is an enlargement (dilatation) of the aorta to greater than 1.5 times normal size. [1] Typically, there are no symptoms except when the aneurysm dissects or ruptures, which causes sudden, severe pain in the abdomen and lower back. [2] [3]
The etiology remains an area of active research. Known causes include trauma, infection, and inflammatory disorders. Risk factors include cigarette smoking, extreme alcoholism, advanced age, dyslipidemia, hypertension, and coronary artery disease. [4] The pathophysiology of the disease is related to an initial arterial insult causing a cascade of inflammation and extracellular matrix protein breakdown by proteinases leading to arterial wall weakening. [5] They are most commonly located in the abdominal aorta, but can also be located in the thoracic aorta.
Aortic aneurysms result from a weakness in the wall of the aorta and increase the risk of aortic rupture. When rupture occurs, massive internal bleeding results and, unless treated immediately, shock and death can occur. One review stated that up to 81% of people having abdominal aortic aneurysm rupture will die, with 32% dying before reaching a hospital. [6]
According to a review of global data through 2019, the prevalence of abdominal aortic aneurysm worldwide was about 0.9% in people under age 79 years, and is about four times higher in men than in women at any age. [4] Death occurs in about 55-64% of people having rupture of the AAA. [4]
Screening with ultrasound is indicated in those at high risk. Prevention is by decreasing risk factors, such as smoking, and treatment is either by open or endovascular surgery. Aortic aneurysms resulted in about 152,000 deaths worldwide in 2013, up from 100,000 in 1990. [7]
Aortic aneurysms are classified by their location on the aorta.
Most intact aortic aneurysms do not produce symptoms. As they enlarge, symptoms such as abdominal pain and back pain may develop. Compression of nerve roots may cause leg pain or numbness. Untreated, aneurysms tend to become progressively larger, although the rate of enlargement is unpredictable for any individual. Rarely, clotted blood which lines most aortic aneurysms can break off and result in an embolus. [8]
Aneurysms cannot be found on physical examination. Medical imaging is necessary to confirm the diagnosis and to determine the anatomic extent of the aneurysm. In people presenting with aneurysm of the arch of the aorta, a common sign is a hoarse voice from stretching of the left recurrent laryngeal nerve, a branch of the vagus nerve that winds around the aortic arch to supply the muscles of the larynx. [10]
Abdominal aortic aneurysms (AAAs) are more common than their thoracic counterpart. One reason for this is that elastin, the principal load-bearing protein present in the wall of the aorta, is reduced in the abdominal aorta as compared to the thoracic aorta. Another is that the abdominal aorta does not possess vasa vasorum, the nutrient-supplying blood vessels within the wall of the aorta. Most AAA are true aneurysms that involve all three layers (tunica intima, tunica media and tunica adventitia). The prevalence of AAAs increases with age, with an average age of 65–70 at the time of diagnosis. AAAs have been attributed to atherosclerosis, though other factors are involved in their formation. [8] Risk factors for AAA include the male gender, aging, a history of smoking, hypercholesterolemia, and hypertension. [4] [8] [11] Reviews reported estimates for prevalence rates of AAA were 0.9-9% in men and 1–2% in women, where, generally, the incidence of AAA is four times greater in men compared to women at the same age. [4] [8]
The risk of rupture of an AAA is related to its diameter; once the aneurysm reaches about 5 cm, the yearly risk of rupture may exceed the risks of surgical repair for an average-risk patient. Rupture risk is also related to shape; so-called "fusiform" (long) aneurysms are considered less rupture-prone than "saccular" (shorter, bulbous) aneurysms, the latter having more wall tension in a particular location in the aneurysm wall. [12]
The prevalence of AAA worldwide in 2019 was about 0.9% in people under age 79 years, [4] whereas a 2014 review reported a range of 2-12%, occurring in about 8% of men more than 65 years of age. [13] Men are about four times more likely to have AA compared to women at any age, with death occurring in about 55-64% of people having AAA rupture. [4]
Before rupture, an AAA may present as a large, pulsatile mass above the umbilicus. A bruit may be heard from the turbulent flow in the aneurysm. Rupture may be the first sign of AAA. Once an aneurysm has ruptured, it presents with classic symptoms of abdominal pain which is severe, constant, and radiating to the back. [8]
The diagnosis of an abdominal aortic aneurysm can be confirmed by the use of ultrasound. Rupture may be indicated by the presence of free fluid in the abdomen. A contrast-enhanced abdominal CT scan is the best test to diagnose an AAA and guide treatment options. [14]
In 2019, some 170,000 people worldwide died from AAA rupture, with aging, smoking, and hypertension as principal factors. [4] [11] Annual mortality from ruptured aneurysms in the United States is about 15,000. [15]
An aortic aneurysm can rupture from wall weakness. Aortic rupture is a surgical emergency and has a high mortality even with prompt treatment. Weekend admission for a ruptured aortic aneurysm is associated with increased mortality compared with admission on a weekday, and this is likely due to several factors including a delay in prompt surgical intervention. [16]
An aortic aneurysm can occur as a result of trauma, infection, or, most commonly, from an intrinsic abnormality in the elastin and collagen components of the aortic wall. Aortic aneurysm development and progression have been directly associated with a deficiency of elastin as well as a loss of collagen type 1. [19] The elastin-to-collagen ratio is also significantly higher in aneurysmal abdominal aortas compared to healthy abdominal aortas. [19] While definite genetic abnormalities were identified in true genetic syndromes (Marfan, Elher-Danlos and others) associated with aortic aneurysms, both thoracic and abdominal aortic aneurysms demonstrate a strong genetic component in their aetiology. [20]
The risk of aneurysm enlargement may be diminished with attention to the patient's blood pressure, smoking and cholesterol levels. There have been proposals to introduce ultrasound scans as a screening tool for those most at risk: men over the age of 65. [21] [22]
Anacetrapib is a cholesteryl ester transfer protein inhibitor that raises high-density lipoprotein (HDL) cholesterol and reduces low-density lipoprotein (LDL) cholesterol. Anacetrapib reduces progression of atherosclerosis, mainly by reducing non-HDL-cholesterol, improves lesion stability and adds to the beneficial effects of atorvastatin [23] Elevating the amount of HDL cholesterol in the abdominal area of the aortic artery in mice both reduced the size of aneurysms that had already grown and prevented abdominal aortic aneurysms from forming at all. In short, raising HDL cholesterol is beneficial because it induces programmed cell death. The walls of a failing aorta are replaced and strengthened. New lesions should not form at all when using this drug. [24]
Screening for an aortic aneurysm so that it may be detected and treated prior to rupture is the best way to reduce the overall mortality of the disease. The most cost-efficient screening test is an abdominal aortic ultrasound study. Noting the results of several large, population-based screening trials, the US Centers for Medicare and Medicaid Services (CMS) now provides payment for one ultrasound study in all smokers aged 65 years or older ("SAAAVE Act"). [25]
Surgery (open or endovascular) is the definitive treatment of an aortic aneurysm. Medical therapy is typically reserved for smaller aneurysms or for elderly, frail patients where the risks of surgical repair exceed the risks of non-operative therapy (observation alone).
Medical therapy of aortic aneurysms involves strict blood pressure control. This does not treat the aortic aneurysm per se, but control of hypertension within tight blood pressure parameters may decrease the rate of expansion of the aneurysm.
The medical management of patients with aortic aneurysms, reserved for smaller aneurysms or frail patients, involves cessation of smoking, blood pressure control, use of statins and occasionally beta blockers. Ultrasound studies are obtained on a regular basis (i.e. every 6 or 12 months) to follow the size of the aneurysm.
Despite optimal medical therapy, patients with large aneurysms are likely to have continued aneurysm growth and risk of aneurysm rupture without surgical repair. [26]
Decisions about repairing an aortic aneurysm are based on the balance between the risk of aneurysm rupture without treatment versus the risks of the treatment itself. For example, a small aneurysm in an elderly patient with severe cardiovascular disease would not be repaired. The chance of the small aneurysm rupturing is overshadowed by the risk of cardiac complications from the procedure to repair the aneurysm.
The risk of the repair procedure is two-fold. First, there is consideration of the risk of problems occurring during and immediately after the procedure itself ("peri-procedural" complications). Second, the effectiveness of the procedure must be taken into account, namely whether the procedure effectively protects the patient from aneurysm rupture over the long term, and whether the procedure is durable so that secondary procedures, with their attendant risks, are not necessary over the life of the patient. A less invasive procedure (such as endovascular aneurysm repair) may be associated with fewer short-term risks to the patient (fewer peri-procedural complications) but secondary procedures may be necessary over long-term follow-up.
The determination of surgical intervention is determined on a per-case basis. The diameter of the aneurysm, its rate of growth, the presence or absence of Marfan syndrome, Ehlers–Danlos syndromes or similar connective tissue disorders, and other co-morbidities are all important factors in the overall treatment.
A large, rapidly expanding, or symptomatic aneurysm should be repaired, as it has a greater chance of rupture. Slowly expanding aortic aneurysms may be followed by routine diagnostic testing (i.e.: CT scan or ultrasound imaging).
For abdominal aneurysms, the current treatment guidelines for abdominal aortic aneurysms suggest elective surgical repair when the diameter of the aneurysm is greater than 5 cm (2 in). However, recent data on patients aged 60–76 suggest medical management for abdominal aneurysms with a diameter of less than 5.5 cm (2 in). [27]
Open surgery starts with exposure of the dilated portion of the aorta via an incision in the abdomen or abdomen and chest, followed by insertion of a synthetic (Dacron or Gore-Tex) graft (tube) to replace the diseased aorta. The graft is sewn in by hand to the non-diseased portions of the aorta, and the aneurysmal sac is closed around the graft.
The aorta and its branching arteries are cross-clamped during open surgery. This can lead to inadequate blood supply to the spinal cord, resulting in paraplegia. A 2004 systematic review and meta analysis found that cerebrospinal fluid drainage (CFSD), when performed in experienced centers, reduces the risk of ischemic spinal cord injury by increasing the perfusion pressure to the spinal cord. [28] A 2012 Cochrane systematic review noted that further research regarding the effectiveness of CFSD for preventing a spinal cord injury is required. [29] A 2023 systematic review suggested that rates of postoperative spinal cord ischaemia can be kept at low levels after open repair of thoracoabdominal aneurysm with the adequate precautions and perioperative manoeuvres. [30] The majority of the surgeons believe prophylactic lumbar drains are effective in reducing spinal cord ischaemia. [31] Neuromonitoring with motor-evoked potentials (MEP) can provide the surgeon objective criteria to direct selective intercostal reconstruction or other protective anaesthetic and surgical manoeuvres. Simultaneous monitoring of MEP and somatosensory-evoked potentials (SSEP) seems to be the most reliable method. [30]
Endovascular treatment of aortic aneurysms is a minimally invasive alternative to open surgery repair. It involves the placement of an endo-vascular stent through small incisions at the top of each leg into the aorta.
As compared to open surgery, EVAR has a lower risk of death in the short term and a shorter hospital stay but may not always be an option. [2] [32] [33] There does not appear to be a difference in longer term outcomes between the two. [34] After EVAR, repeat procedures are more likely to be needed. [35]
Globally, aortic aneurysms resulted in about ~170,000 deaths in 2017. [4] This figure represents an increase from approximately ~100,000 in 1990. [7]
Aortic dissection (AD) occurs when an injury to the innermost layer of the aorta allows blood to flow between the layers of the aortic wall, forcing the layers apart. In most cases, this is associated with a sudden onset of agonizing chest or back pain, often described as "tearing" in character. Vomiting, sweating, and lightheadedness may also occur. Damage to other organs may result from the decreased blood supply, such as stroke, lower extremity ischemia, or mesenteric ischemia. Aortic dissection can quickly lead to death from insufficient blood flow to the heart or complete rupture of the aorta.
An aneurysm is an outward bulging, likened to a bubble or balloon, caused by a localized, abnormal, weak spot on a blood vessel wall. Aneurysms may be a result of a hereditary condition or an acquired disease. Aneurysms can also be a nidus for clot formation (thrombosis) and embolization. As an aneurysm increases in size, the risk of rupture, which leads to uncontrolled bleeding, increases. Although they may occur in any blood vessel, particularly lethal examples include aneurysms of the circle of Willis in the brain, aortic aneurysms affecting the thoracic aorta, and abdominal aortic aneurysms. Aneurysms can arise in the heart itself following a heart attack, including both ventricular and atrial septal aneurysms. There are congenital atrial septal aneurysms, a rare heart defect.
Interventional radiology (IR) is a medical specialty that performs various minimally-invasive procedures using medical imaging guidance, such as x-ray fluoroscopy, computed tomography, magnetic resonance imaging, or ultrasound. IR performs both diagnostic and therapeutic procedures through very small incisions or body orifices. Diagnostic IR procedures are those intended to help make a diagnosis or guide further medical treatment, and include image-guided biopsy of a tumor or injection of an imaging contrast agent into a hollow structure, such as a blood vessel or a duct. By contrast, therapeutic IR procedures provide direct treatment—they include catheter-based medicine delivery, medical device placement, and angioplasty of narrowed structures.
Vascular surgery is a surgical subspecialty in which vascular diseases involving the arteries, veins, or lymphatic vessels, are managed by medical therapy, minimally-invasive catheter procedures and surgical reconstruction. The specialty evolved from general and cardiovascular surgery where it refined the management of just the vessels, no longer treating the heart or other organs. Modern vascular surgery includes open surgery techniques, endovascular techniques and medical management of vascular diseases - unlike the parent specialities. The vascular surgeon is trained in the diagnosis and management of diseases affecting all parts of the vascular system excluding the coronaries and intracranial vasculature. Vascular surgeons also are called to assist other physicians to carry out surgery near vessels, or to salvage vascular injuries that include hemorrhage control, dissection, occlusion or simply for safe exposure of vascular structures.
Abdominal aortic aneurysm (AAA) is a localized enlargement of the abdominal aorta such that the diameter is greater than 3 cm or more than 50% larger than normal. An AAA usually causes no symptoms, except during rupture. Occasionally, abdominal, back, or leg pain may occur. Large aneurysms can sometimes be felt by pushing on the abdomen. Rupture may result in pain in the abdomen or back, low blood pressure, or loss of consciousness, and often results in death.
A thoracic aortic aneurysm is an aortic aneurysm that presents primarily in the thorax.
Traumatic aortic rupture, also called traumatic aortic disruption or transection, is a condition in which the aorta, the largest artery in the body, is torn or ruptured as a result of trauma to the body. The condition is frequently fatal due to the profuse bleeding that results from the rupture. Since the aorta branches directly from the heart to supply blood to the rest of the body, the pressure within it is very great, and blood may be pumped out of a tear in the blood vessel very rapidly. This can quickly result in shock and death. Thus traumatic aortic rupture is a common killer in automotive accidents and other traumas, with up to 18% of deaths that occur in automobile collisions being related to the injury. In fact, aortic disruption due to blunt chest trauma is the second leading cause of injury death behind traumatic brain injury.
Endovascular aneurysm repair (EVAR) is a type of minimally-invasive endovascular surgery used to treat pathology of the aorta, most commonly an abdominal aortic aneurysm (AAA). When used to treat thoracic aortic disease, the procedure is then specifically termed TEVAR for "thoracic endovascular aortic/aneurysm repair." EVAR involves the placement of an expandable stent graft within the aorta to treat aortic disease without operating directly on the aorta. In 2003, EVAR surpassed open aortic surgery as the most common technique for repair of AAA, and in 2010, EVAR accounted for 78% of all intact AAA repair in the United States.
Aortic rupture is the breakage of all walls of the aorta, the largest artery in the body. Aortic rupture is a rare, extremely dangerous condition that is considered a medical emergency. The most common cause is an abdominal aortic aneurysm that has ruptured spontaneously. Aortic rupture is distinct from aortic dissection, which is a tear through the inner wall of the aorta that can block the flow of blood through the aorta to the heart or abdominal organs.
Peter Lin is an American vascular surgeon, medical researcher, specializing in minimally invasive endovascular treatment of vascular disease. He has published extensively in the area of vascular surgery and endovascular surgery.
Surgical Outcomes Analysis & Research, SOAR, is a research laboratory of the Department of Surgery at Boston University School of Medicine and Boston Medical Center with expertise in outcomes research. SOAR investigates surgical diseases and perioperative outcomes. The group focuses on pancreatic cancer, other gastrointestinal and hepatobiliary malignancies, vascular disease, and transplant surgery. SOAR's goal is to examine quality, delivery, and financing of care in order to have an immediate impact on patient care and system improvements. The group members utilize national health services and administrative databases, as well as institutional databases, to investigate and to address factors contributing to disease outcomes and healthcare disparities.
Familial aortic dissection or FAD refers to the splitting of the wall of the aorta in either the arch, ascending or descending portions. FAD is thought to be passed down as an autosomal dominant disease and once inherited will result in dissection of the aorta, and dissecting aneurysm of the aorta, or rarely aortic or arterial dilation at a young age. Dissection refers to the actual tearing open of the aorta. However, the exact gene(s) involved has not yet been identified. It can occur in the absence of clinical features of Marfan syndrome and of systemic hypertension. Over time this weakness, along with systolic pressure, results in a tear in the aortic intima layer thus allowing blood to enter between the layers of tissue and cause further tearing. Eventually complete rupture of the aorta occurs and the pleural cavity fills with blood. Warning signs include chest pain, ischemia, and hemorrhaging in the chest cavity. This condition, unless found and treated early, usually results in death. Immediate surgery is the best treatment in most cases. FAD is not to be confused with PAU and IMH, both of which present in ways similar to that of familial aortic dissection.
Open aortic surgery (OAS), also known as open aortic repair (OAR), describes a technique whereby an abdominal, thoracic or retroperitoneal surgical incision is used to visualize and control the aorta for purposes of treatment, usually by the replacement of the affected segment with a prosthetic graft. OAS is used to treat aneurysms of the abdominal and thoracic aorta, aortic dissection, acute aortic syndrome, and aortic ruptures. Aortobifemoral bypass is also used to treat atherosclerotic disease of the abdominal aorta below the level of the renal arteries. In 2003, OAS was surpassed by endovascular aneurysm repair (EVAR) as the most common technique for repairing abdominal aortic aneurysms in the United States.
Inflammatory aortic aneurysm (IAA), also known as Inflammatory abdominal aortic aneurysm (IAAA), is a type of abdominal aortic aneurysm (AAA) where the walls of the aneurysm become thick and inflamed. Similar to AAA, IAA occurs in the abdominal region. IAA is closely associated and believed to be a response to and extensive peri-aneurysmal fibrosis, which is the formation of excess fibrous connective tissue in an organ or tissue in a reparative or reactive process IAA accounts for 5-10% of aortic aneurysms. IAA occurs mainly in a population that is on average younger by 10 years than most AAA patients. Some common symptoms of IAA may include back pain, abdominal tenderness, fevers, weight loss or elevated Erythrocyte sedimentation rate (ESR) levels. Corticosteroids and other immunosuppressive drugs have been found to decrease symptoms and the degree of peri-aortic inflammation and fibrosis
Endovascular and hybrid trauma and bleeding management is a new and rapidly evolving concept within medical healthcare and endovascular resuscitation. It involves early multidisciplinary evaluation and management of hemodynamically unstable patients with traumatic injuries as well as being a bridge to definitive treatment. It has recently been shown that the EVTM concept may also be applied to non-traumatic hemodynamically unstable patients.
Resuscitative endovascular balloon occlusion of the aorta (REBOA) is a minimally-invasive procedure performed during resuscitation of critically-injured trauma patients. Originally developed as a less invasive alternative to emergency thoracotomy with aortic cross clamping, REBOA is performed to gain rapid control of non-compressible truncal or junctional hemorrhage. REBOA is performed first by achieving access to the common femoral artery (CFA) and advancing a catheter within the aorta. Upon successful catheter placement, an occluding balloon may be inflated either within the descending thoracic aorta or infrarenal abdominal aorta. REBOA stanches downstream hemorrhage and improves cardiac index, cerebral perfusion, and coronary perfusion. Although REBOA does not eliminate the need for definitive hemorrhage control, it may serve as a temporizing measure during initial resuscitation. Despite the benefits of REBOA, there are significant local and systemic ischemic risks. Establishing standardized REBOA procedural indications and mitigating the risk of ischemic injury are topics of ongoing investigation. Although this technique has been successfully deployed in adult patients, it has not yet been studied in children.
Nicolai Leontievich Volodos, was a Soviet/Ukrainian cardiovascular surgeon and scientist. An innovator, Volodos developed and introduced into clinical practice the world's first endovascular stent graft for the treatment of stenotic and aneurysmal diseases of arterial system. Volodos was described by his colleagues as "a pioneer innovator and a giant in vascular and endovascular surgery" and "a giant of historic proportions in the vascular and endovascular specialties, and the father of endovascular grafting".
Hazim J. Safi, MD, FACS, is a physician and surgeon who is well known for his research in the surgical treatment of aortic disease. Safi and his colleagues at Baylor College of Medicine were the first to identify variables associated with early death and postoperative complications in patients undergoing thoracoabdominal aortic operations. Safi now serves as professor of cardiothoracic surgery, and founding chair at McGovern Medical School at The University of Texas Health Science Center in Houston, TX.
Benjamin Starnes is a vascular surgeon and medical researcher. He holds the Alexander Whitehill Clowes Endowed Chair in Vascular surgery at the University of Washington. He served as a U.S. Army surgeon for 15 years, doing three tours of duty, including in the last M.A.S.H. unit. On the day of the September 11 attacks he was at the Pentagon rendering medical aid to victims, and his experience was later recounted in the book American Phoenix: Heroes of the Pentagon on 9/11. He is among the primary authors of the official guidelines for diagnosis and management of aortic disease adopted by the American College of Cardiology and the American Heart Association.
Jes Sanddal Lindholt is a Danish vascular surgeon, physician, author, and academic. He is a professor of vascular surgery and Head of the Cardiovascular Excellence Center in Region South as well as a research leader for the Department of Cardiothoracic and Vascular Surgery at the University of Southern Denmark and Odense University Hospital. He also holds the position of adjunct professor of vascular epidemiology at Aarhus University.