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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.
The aortic valve is a valve in the heart of humans and most other animals, located between the left ventricle and the aorta. It is one of the four valves of the heart and one of the two semilunar valves, the other being the pulmonary valve. The aortic valve normally has three cusps or leaflets, although in 1–2% of the population it is found to congenitally have two leaflets. The aortic valve is the last structure in the heart the blood travels through before stopping the flow through the systemic circulation.
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.
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.
An aortic aneurysm is an enlargement (dilatation) of the aorta to greater than 1.5 times normal size. Typically, there are no symptoms except when the aneurysm dissects or ruptures, which causes sudden, severe pain in the abdomen and lower back.
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.
A thoracic aortic aneurysm is an aortic aneurysm that presents primarily in the thorax.
Aortic valve replacement is a cardiac surgery procedure whereby a failing aortic valve is replaced with an artificial heart valve. The aortic valve may need to be replaced because of aortic regurgitation, or if the valve is narrowed by stenosis.
Tirone Esperidiao David, is a Brazilian-born Canadian cardiac surgeon and professor of surgery at the University of Toronto. He is an attending cardiac surgeon at the Peter Munk Cardiac Centre, Toronto General Hospital. He is known for his 2007 development of a valve sparing aortic root replacement procedure to preserve the aortic valve in patients with aortic root aneurysms such as in Marfan syndrome; it is now known as the "David Operation".
Aortic valve repair or aortic valve reconstruction is the reconstruction of both form and function of a dysfunctional aortic valve. Most frequently it is used for the treatment of aortic regurgitation. It can also become necessary for the treatment of aortic aneurysm, less frequently for congenital aortic stenosis.
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.
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 Ross procedure, also known as pulmonary autograft, is a heart valve replacement operation to treat severe aortic valve disease, such as in children and young adults with a bicuspid aortic valve. It involves removing the diseased aortic valve, situated at the exit of the left side of the heart, and replacing it with the person's own healthy pulmonary valve (autograft), removed from the exit of the heart's right side. To reconstruct the right sided exit, a pulmonary valve from a cadaver (homograft), or a stentless xenograft, is used to replace the removed pulmonary valve. Compared to a mechanical valve replacement, it avoids the requirement for thinning the blood, has favourable blood flow dynamics, allows growth of the valve with growth of the child and has less risk of endocarditis.
Annuloaortic ectasia is characterized by pure aortic valve regurgitation and aneurysmal dilatation of the ascending aorta. Men are more likely than women to develop idiopathic annuloaortic ectasia, which usually manifests in the fourth or sixth decades of life. Additional factors that contribute to this condition include osteogenesis imperfecta, inflammatory aortic diseases, intrinsic valve disease, Loeys-Dietz syndrome, Marfan syndrome, and operated congenital heart disease.
Fibrillin-1 is a protein that in humans is encoded by the FBN1 gene, located on chromosome 15. It is a large, extracellular matrix glycoprotein that serves as a structural component of 10–12 nm calcium-binding microfibrils. These microfibrils provide force bearing structural support in elastic and nonelastic connective tissue throughout the body. Mutations altering the protein can result in a variety of phenotypic effects differing widely in their severity, including fetal death, developmental problems, Marfan syndrome or in some cases Weill-Marchesani syndrome.
Randall Bertram Griepp was an American cardiothoracic surgeon who collaborated with Norman Shumway in the development of the first successful heart transplant procedures in the U.S. He had an international reputation for contributions to the surgical treatment of aortic aneurysms and aortic dissection and in heart and lung transplantations. He received nearly $8 million in grants from the National Heart, Lung, and Blood Institute.
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.
Hugh Bentall FRCS was a British surgeon who pioneered open-heart surgery.
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.
Hans-Joachim Schäfers is a German surgeon, as well as cardiac, thoracic, and vascular surgeon and university professor. He is director of the department of Thoracic and Cardiovascular Surgery at the Saarland University Medical Center in Homburg/Saar, Germany. He is known for his activities in aortic valve repair, aortic surgery, and pulmonary endarterectomy.
References
- ↑ Bentall H.; De Bono A. (1968). "A technique for complete replacement of the ascending aorta". Thorax. 23 (4): 338–9. doi:10.1136/thx.23.4.338. PMC 471799 . PMID 5664694.
- ↑ Mookhoek, Art; Korteland, Nelleke M.; Arabkhani, Bardia; Di Centa, Isabelle; Lansac, Emmanuel; Bekkers, Jos A.; Bogers, Ad J. J. C.; Takkenberg, Johanna J. M. (2016). "Bentall Procedure: A Systematic Review and Meta-Analysis". Adult Cardiac. 101 (5). The Society of Thoracic Surgeons: 1684–1689. doi: 10.1016/j.athoracsur.2015.10.090 . PMID 26857635.
