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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.
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.
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.
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.
Sir Magdi Habib Yacoub is an Egyptian-British retired professor of cardiothoracic surgery at Imperial College London, best known for his early work in repairing heart valves with surgeon Donald Ross, adapting the Ross procedure, where the diseased aortic valve is replaced with the person's own pulmonary valve, devising the arterial switch operation (ASO) in transposition of the great arteries, and establishing the heart transplantation centre at Harefield Hospital in 1980 with a heart transplant for Derrick Morris, who at the time of his death was Europe's longest-surviving heart transplant recipient. Yacoub subsequently performed the UK's first combined heart and lung transplant in 1983.
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".
Valvular heart disease is any cardiovascular disease process involving one or more of the four valves of the heart. These conditions occur largely as a consequence of aging, but may also be the result of congenital (inborn) abnormalities or specific disease or physiologic processes including rheumatic heart disease and pregnancy.
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.
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.
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.
Duke E. Cameron is an American cardiac surgeon. Formerly Chief of Cardiac Surgery at Johns Hopkins University School of Medicine; the James T. Dresher Sr. Professor of Surgery; Director of Pediatric Cardiac Surgery; and Director of The Dana and Albert "Cubby" Broccoli Center for Aortic Diseases , at the Johns Hopkins Hospital, he returned to Hopkins in 2023. His clinical interests include:
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.
Heart valve repair is a cardiac surgery procedure, carried out to repair one or more faulty heart valves. In some valvular heart diseases repair where possible is preferable to valve replacement. A mechanical heart valve is a replacement valve that is not itself subject to repair.
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.
Giuseppe Di Benedetto is an Italian cardiac surgeon born in Eboli on 8 January 1946, He holds the Italian national record for the use of carbon dioxide laser to perform revascularization trans-myocardial otherwise inoperable; specialized in congenital diseases of cardiovascular system, is one of the few surgeons in the world that practice successfully the surgery of aortic arch.
References
- ↑ "The Modified David's Reimplantation Procedure". clevelandclinic.org. Retrieved 15 July 2014.
- ↑ Marfan Syndrome: A Primer For Clinicians And Scientists. Peter Nicholas Robinson, Maurice Godfrey eds. Chapter 5: Duke E. Cameron and Vincent L. Gott. Surgical Management of the Marfan Patient at The Johns Hopkins Hospital.
- ↑ David, TE; David, CM; Ouzounian, M; Feindel, CM; Lafreniere-Roula, M (March 2021). "A progress report on reimplantation of the aortic valve". The Journal of Thoracic and Cardiovascular Surgery. 161 (3): 890–899.e1. doi: 10.1016/j.jtcvs.2020.07.121 . PMID 33008570. S2CID 222170984.
- ↑ Sef, D; Bahrami, T; Raja, SG; Klokocovnik, T (June 2022). "Current trends in minimally invasive valve-sparing aortic root replacement-Best available evidence". Journal of Cardiac Surgery. 37 (6): 1684–1690. doi:10.1111/jocs.16453. PMID 35348237. S2CID 247777604.
- ↑ David T, Feindel C (1992). "An aortic valve-sparing operation for patients with aortic incompetence and aneurysm of the ascending aorta". J Thorac Cardiovasc Surg. 103 (4): 617–21, discussion 622. doi: 10.1016/S0022-5223(19)34942-6 . PMID 1532219.
