Familial aortic dissection

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Familial aortic dissection
Other namesCystic medial necrosis of aorta, Annuloaortic ectasia
Aorta Anatomy.jpg
Aorta

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. [1] It can occur in the absence of clinical features of Marfan syndrome and of systemic hypertension. [2] [3] [4] [5] [6] 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. [7] FAD is not to be confused with PAU (penetrating atherosclerotic ulcers) and IMH (intramural hematoma), both of which present in ways similar to that of familial aortic dissection. [8]

Contents

Causes

Inheritance is thought to be rather complex. There is a good amount of evidence that shows the disease is autosomal dominant, with some penetrance. There is also the possibility of age related dependence. It is known that Marfan’s Syndrome and Ehler-Danlos Syndrome lead to an increased risk for development of FAD. Marfan’s Syndrome is not required to have an aortic dissection. [9] One study suggests that the chromosomal locus for the gene is 5q13-14. The same study found that other genes may be linked, and include loci for Marfan and Ehler-Danlos Syndromes, genes for metalloproteinase 3 and 9, and tissue inhibitor of malloproteinase 2 as well as two loci on chromosomes 5q13-14 and lq23.2-24. [2] [3] Still other studies show that mutations in smooth muscle cell-specific isoforms of alpha actin and beta myosin heavy chain may cause FAD. [10] Mutations in the genes TGFBR 1 and 2 are known to cause dissections in aortas with normal diameter size (>4.3 cm) and gene FPN1 mutations typically affect aortas with larger diameters (<4.4 cm). [11]

There are several hypotheses which attempt to explain how the dissection physically occurs. The first states that a tear develops in the intima layer of the aorta which allows blood to flow from the lumen of the aorta into the intima. This event creates a dissection and essentially two lumens. The second hypothesis suggests that the vasa vasorum ruptures and causes a hemorrhage in the wall of the aorta. The hemorrhaging promotes tearing of the intima and eventually aortic dissection. [12]

The major risk factors for FAD include high blood pressure, old age, haematoma, genetic weakening of aortic wall, cocaine use, pregnancy and diseases causing abnormal connective tissue. [7] [12] One study found that the average age(s) for the occurrence of dissection caused by degenerative aneurysm is 65 years and up. Dissections thought to be the result of genetic mutations appear to be more likely to occur between the ages of 40 and 60. Another study found that 20% of patients with FAD have a close relative with a history of thoracic aortic aneurysm or dissection which suggests yet another major risk factor. [13]

Physiology

FAD is normally associated with Marfan syndrome, Ehlers–Danlos syndrome, and various other genetic disorders which affect the connective tissues of the cardiovascular system. There are various mechanisms by which the medial layers of the lumen are stressed and eventually torn. [14] Once torn these areas begin to fill with blood and become susceptible to aneurysm formation. Depending on the location of the tear, FAD normally affects the ascending or descending aorta, where the primary characteristic of a bulge can be seen. This bulge is the result of the creation of a false lumen due to the vast amount of blood seepage from the aortas and surrounding veins. [15] In some cases it is not uncommon to see degeneration in the ascending and descending aorta and the atrioventricular and semilunar valves due to elastolysis or breakdown and loss of elastic fibers. These connective tissue malfunctions are traceable to mutations, and lack of genes encoding for important components such as collagens, and micro-fibril-associated glycoproteins. Breakdown among these connective layers eventually compromises the integrity of the aortic lumen. [14]

Depending on the extent of pooling and damage to the blood vessel, the Svensson system was created to diagnose and describe the five classes of pathological processes that may be visible due to the dissection. Class 1 refers to any dissection with a true and false lumen. Class 2 specifically depends on the presence of hematoma or hemorrhaging at the site of the dissection. Class 3 is a dissection without hematoma. Class 4 is recognized by the presence of an ulcer among the lumen. Class 5 has to do with any sort of traumatic hemorrhaging in the dissection. [16] Class 5, in the most case, is the most severe and life-threatening due to large amounts of rapid blood loss.[ citation needed ]

Those experiencing aortic dissection typically will complain of agonizing pains described with a ripping feeling in the chest that for some may migrate to their backs. [8] Anything that compromises or obstructs the amount of blood flow and the delivery of nutrients and oxygen to the walls of the ascending and descending aorta has a large impact on the viability of the layers of the surrounding lumen. Chronic hypertension, Inflammatory disease, excessive plaque build-up among coronary walls, intimal thickening, and arteriosclerosis are all believed[ by whom? ] to increase the likelihood of FAD occurring in an individual.

Diagnosis

Since the cause of FAD has not been genetically pinpointed, the only way to diagnose FAD is through the examination of phenotypic variations in the aorta. Usually echocardiography is used to take measurements of the aortic root [17] as well as transesophageal echocardiography. [8] Biomarkers lend a quick way to diagnose dissection when time is of the essence. These have the ability to relay the levels of smooth muscle mysosin heavy chain protein present, which is released from damaged aortic tissue. [18]

There are two types of FAD; groups A and B. Normally if any area of the ascending aorta is involved in the dissection this is considered group A. If the dissection occurs within the descending aorta this is classified in group B. [16] These two groups can than be broken down into three classes of FAD: Type 1, Type 2 and Type 3. Group A consists of Types 1 and 2, whereas Group B consists only of Type 3. Type 1 encompasses dissection in the distal ascending aorta closest to the heart, not including the aortic arch. Type 2 refers to dissection of the ascending aorta, closer to and including the aortic arch. Type 3 refers to the descending thoracic and abdominal aorta. [7]

Group A dissections are the more serious of the two due to the location of the dissection in the ascending aorta, which leads to a higher risk of congestive heart failure and pericardium and/or aortic valve rupture. Individuals also tend to be predisposed to type A if they do have Marfans or Elhers-Danlos syndromes. These contribute to a higher fatality rate in group A dissection if immediate surgery is not performed. The most common corrective surgeries are actual aortic valve replacement and coronary artery bypass. The five-year survival rate after surgery is a successful 70.4% due to vigilant monthly physical exams and chest x-rays to monitor progress. Group B dissections typically have a higher surgery mortality rate and are therefore not good candidates. Instead medical management is the common response to treating and keeping dissections of the descending aorta under control. [8]

Treatment

Type 1 and Type 2 FAD call for the same treatment: immediate surgery to replace the aorta. Surgery is required due to the high risk of mortality. Type 3 is less severe and requires the maintenance of blood pressure through diet and exercise. Upon diagnosing someone with FAD intravenous antihypertensive treatment is frequently used. Often intravenous sodium nitroprusside is used for its efficiency in lessening the pulsatile load thus reducing blood pressure. Reducing this force slows the progression of the dissection. Surgical success depends on age, severity of symptoms, postoperative organ dysfunction and stroke. Surgical intervention is always indicated in Type 1 cases. Aortic surgery is palliative, not curative. The goal is to merely to prevent rupture, restore blood flow, and fix any aortic valve dysfunction. [19] Post operative protocols include frequent monitoring of the aorta diameter. Statins and beta blockers are also popular treatments used to reduce future plaque build up and blockage of epinephrine receptors as a way to control heart rate and blood pressure. [18]

Long term treatment should also include regular check ups every 3 to 6 months. A CT scan or MRI is recommended, along with required chest x-rays. Antihypertensive therapy with beta adrenergic antagonists is required regardless of medical versus surgical treatment. Ten to twenty percent of those who choose surgical intervention are re-operated on due to compression, aneurysm development or blood leakage. [19]

Research directions

Currently, there is controversy over whether or not inheritance truly plays a role in FAD, and if so which gene it acts upon. FAD does not come from strictly one predisposing factor, such as hypertension. It is suggested that the combination of environmental factors along with genetics may contribute to causing FAD. Before newer and more effective cures and therapies can be developed, first the specific gene mutation must be identified. Until such a gene is determined, scientists say patient education, and physician awareness is vital. [17] Currently scientists have found animal models to be beneficial in understanding the pathology behind FAD. In the future there is hope to develop drugs that will better support and strengthen the aortic wall. Endovascular methods of treatment are becoming increasingly popular, and scientists hope to use this technique in both acute and chronic cases. [16]

Related Research Articles

<span class="mw-page-title-main">Aorta</span> Largest artery in the human body

The aorta is the main and largest artery in the human body, originating from the left ventricle of the heart, branching upwards immediately after, and extending down to the abdomen, where it splits at the aortic bifurcation into two smaller arteries. The aorta distributes oxygenated blood to all parts of the body through the systemic circulation.

<span class="mw-page-title-main">Marfan syndrome</span> Genetic disorder involving connective tissue

Marfan syndrome (MFS) is a multi-systemic genetic disorder that affects the connective tissue. Those with the condition tend to be tall and thin, with long arms, legs, fingers, and toes. They also typically have exceptionally flexible joints and abnormally curved spines. The most serious complications involve the heart and aorta, with an increased risk of mitral valve prolapse and aortic aneurysm. The lungs, eyes, bones, and the covering of the spinal cord are also commonly affected. The severity of the symptoms is variable.

<span class="mw-page-title-main">Aortic dissection</span> Injury to the innermost layer of the aorta

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 severe 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.

<span class="mw-page-title-main">Aneurysm</span> Bulge in the wall of a blood vessel

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.

<span class="mw-page-title-main">Vascular surgery</span> Medical specialty, operative procedures for the treatment of vascular disorders

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.

<span class="mw-page-title-main">Bicuspid aortic valve</span> Medical condition

Bicuspid aortic valve (BAV) is a form of heart disease in which two of the leaflets of the aortic valve fuse during development in the womb resulting in a two-leaflet (bicuspid) valve instead of the normal three-leaflet (tricuspid) valve. BAV is the most common cause of heart disease present at birth and affects approximately 1.3% of adults. Normally, the mitral valve is the only bicuspid valve and this is situated between the heart's left atrium and left ventricle. Heart valves play a crucial role in ensuring the unidirectional flow of blood from the atrium to the ventricles, or from the ventricle to the aorta or pulmonary trunk. BAV is normally inherited.

<span class="mw-page-title-main">Aortic aneurysm</span> Excessive enlargement of the human aorta

An aortic aneurysm is an enlargement (dilatation) of the aorta to greater than 1.5 times normal size. They usually cause no symptoms except when ruptured. Occasionally, there may be abdominal, back, or leg pain. The prevalence of abdominal aortic aneurysm ("AAA") has been reported to range from 2 to 12% and is found in about 8% of men more than 65 years of age. The mortality rate attributable to AAA is about 15,000 per year in the United States and 6,000 to 8,000 per year in the United Kingdom and Ireland. Between 2001 and 2006, there were approximately 230,000 AAA surgical repairs performed on Medicare patients in the United States.

<span class="mw-page-title-main">Aneurysm of sinus of Valsalva</span> Medical condition

Aneurysm of the aortic sinus, also known as the sinus of Valsalva, is a rare abnormality of the aorta, the largest artery in the body. The aorta normally has three small pouches that sit directly above the aortic valve, and an aneurysm of one of these sinuses is a thin-walled swelling. Aneurysms may affect the right (65–85%), non-coronary (10–30%), or rarely the left coronary sinus. These aneurysms may not cause any symptoms but if large can cause shortness of breath, palpitations or blackouts. Aortic sinus aneurysms can burst or rupture into adjacent cardiac chambers, which can lead to heart failure if untreated.

<span class="mw-page-title-main">Thoracic aortic aneurysm</span> Medical condition

A thoracic aortic aneurysm is an aortic aneurysm that presents primarily in the thorax.

<span class="mw-page-title-main">Loeys–Dietz syndrome</span> Medical condition

Loeys–Dietz syndrome (LDS) is an autosomal dominant genetic connective tissue disorder. It has features similar to Marfan syndrome and Ehlers–Danlos syndrome. The disorder is marked by aneurysms in the aorta, often in children, and the aorta may also undergo sudden dissection in the weakened layers of the wall of the aorta. Aneurysms and dissections also can occur in arteries other than the aorta. Because aneurysms in children tend to rupture early, children are at greater risk for dying if the syndrome is not identified. Surgery to repair aortic aneurysms is essential for treatment.

Interrupted aortic arch is a very rare heart defect in which the aorta is not completely developed. There is a gap between the ascending and descending thoracic aorta. In a sense it is the complete form of a coarctation of the aorta. Almost all patients also have other cardiac anomalies, including a ventricular septal defect (VSD), aorto-pulmonary window, and truncus arteriosus. There are three types of interrupted aortic arch, with type B being the most common. Interrupted aortic arch is often associated with DiGeorge syndrome.

<span class="mw-page-title-main">Syphilitic aortitis</span> Inflammation of the aorta

Syphilitic aortitis is inflammation of the aorta associated with the tertiary stage of syphilis infection. SA begins as inflammation of the outermost layer of the blood vessel, including the blood vessels that supply the aorta itself with blood, the vasa vasorum. As SA worsens, the vasa vasorum undergo hyperplastic thickening of their walls thereby restricting blood flow and causing ischemia of the outer two-thirds of the aortic wall. Starved for oxygen and nutrients, elastic fibers become patchy and smooth muscle cells die. If the disease progresses, syphilitic aortitis leads to an aortic aneurysm. Overall, tertiary syphilis is a rare cause of aortic aneurysms. Syphilitic aortitis has become rare in the developed world with the advent of penicillin treatments after World War II.

Valve-sparing aortic root replacement is a cardiac surgery procedure which is used to treat Aortic aneurysms and to prevent Aortic dissection. It involves replacement of the aortic root without replacement of the aortic valve. Two similar procedures were developed, one by Sir Magdi Yacoub, and another by Tirone David.

The Bentall procedure is a type of cardiac surgery involving composite graft replacement of the aortic valve, aortic root, and ascending aorta, with re-implantation of the coronary arteries into the graft. This operation is used to treat combined disease of the aortic valve and ascending aorta, including lesions associated with Marfan syndrome. The Bentall procedure was first described in 1968 by Hugh Bentall and Antony De Bono. It is considered a standard for individuals who require aortic root replacement, and the vast majority of individuals who undergo the surgery receive mechanical valves.

Annuloaortic ectasia is a dilation of the proximal ascending aorta and aortic annulus. It may cause aortic regurgitation, thoracic aortic dissection, aneurysm and rupture. It is often associated with connective tissue diseases like Marfan syndrome and Ehlers Danlos Syndrome. It can also be a complication due to tertiary syphilis. In tertiary syphilis the aortic root becomes so dilated that the aortic valve becomes incompetent and cor bovinum results.

<span class="mw-page-title-main">Traumatic aortic rupture</span> Medical condition

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.

<span class="mw-page-title-main">Endovascular aneurysm repair</span> Surgery used to treat abdominal aortic aneurysm

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.

<span class="mw-page-title-main">Acute aortic syndrome</span> Medical condition

Acute aortic syndrome (AAS) describes a range of severe, painful, potentially life-threatening abnormalities of the aorta. These include aortic dissection, intramural thrombus, and penetrating atherosclerotic aortic ulcer. AAS can be caused by a lesion on the wall of the aorta that involves the tunica media, often in the descending aorta. It is possible for AAS to lead to acute coronary syndrome. The term was introduced in 2001.

<span class="mw-page-title-main">Open aortic surgery</span> Surgical technique

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.

<span class="mw-page-title-main">Familial thoracic aortic aneurysm and aortic dissection</span> Medical condition

Familial thoracic aortic aneurysm and aortic dissection is a very rare vascular genetic disorder, it's characterized by recurrent thoracic aortic aneurysms and aortic dissections within a family, these mentioned complications affect one or more aortic segments without any other disease being associated with them. People with this disorder have a higher chance of having a potentially fatal aortic rupture. This disorder is the cause of 20% of thoracic aortic aneurysms

References

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