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Valvular Heart Disease and Heart Failure
Published in Andreas P. Kalogeropoulos, Hal A. Skopicki, Javed Butler, Heart Failure, 2023
Kali Polytarchou, Constantina Aggeli
Patients with aortic root or ascending aorta aneurysm should undergo aortic valve repair or replacement, irrespective of the severity of valvular disease. In general, surgery is recommended for patients with aortic dimension ≥55 mm. In Marfan syndrome, surgery is recommended when the ascending aorta dimension is ≥50 mm or ≥45 mm with concomitant risk factors (such as personal or family history of aortic or other vascular dissection, severe aortic or mitral regurgitation, desire for pregnancy, arterial hypertension, or annual increase of aortic diameter ≥3 mm/year). Patients with Loeyz–Dietz syndrome or TGFBR1 or TGFBR2 gene mutations should undergo surgery when aortic dimension is ≥45 mm. Patients with bicuspid aortic valve or coarctation of the aorta and presence of the above risk factors should procced to surgery when aortic dimension is ≥50 mm. For patients with an indication for aortic valve replacement or repair, aortic root and ascending aorta replacement is indicated when dimension is ≥45 mm.21
Diseases of the Aorta
Published in Mary N. Sheppard, Practical Cardiovascular Pathology, 2022
Aneurysms can be defined as external bulges of a blood-containing structure which expand in systole. All aneurysms have the risk of external rupture with fatal consequences. In true aortic aneurysms, the wall is made up of all the constituents of the aortic wall, i.e. intima, media and adventitia. In what are called false aneurysms, the external bulge has a wall consisting of adventitia or periaortic tissue only (Fig. 8.20). If the aneurysm sac bulges out to one side and communicates with the lumen via a narrow defect in the media, it is called a saccular aneurysm. Diffuse aneurysms involve the whole circumference of the aorta and often extend over a long distance. Diffuse aneurysms of the ascending aorta are almost always associated with aortic regurgitation, due to dilatation of the aortic root at the level of the supra-aortic ridge.
Point-of-Care Ultrasound
Published in Mansoor Khan, David Nott, Fundamentals of Frontline Surgery, 2021
Carlos Augusto M. Menegozzo, Bruno M. Pereira
The pipes will be evaluated for aneurysm, dissection, and rupture of the aorta by scanning the sites with the highest incidence of these abnormalities—the suprasternal, parasternal, epigastric, and supraumbilical. The ideal transducer for this assessment is the phased array, and the examiner may identify aortic enlargements, intraluminal flaps, and periaortic hematomas. The aorta is evaluated by placing the probe in at least four regions. The aortic root is visualised by placing the transducer horizontally on the suprasternal area. The physician should then obtain a parasternal long-axis view to evaluate the descending aorta. Further down, the examiner should place the transducer horizontally in the epigastric and periumbilical areas to cover the abdominal aorta. Abdominal compression may be necessary to obtain a clearer image of the aorta by displacing intraluminal gas. Since most of the aortic ruptures and hematomas are in the retroperitoneum, this specific evaluation may be limited with ultrasound. However, the presence of aortic aneurysm in a hypotensive patient should prompt the need for urgent investigation of a possible rupture. The sensitivity of ultrasound to detect aortic aneurysms vary from 93–100%. Aortic dissection may be identified by aortic root enlargement or an intimal flap. The suprasternal, parasternal long axis, epigastric, and periumbilical views may cover the most common areas of dissection. By using the colour Doppler, it may be possible to identify two distinct lumens in some cases.
Double right coronary artery: a plea for a standardized nomenclature
Published in Acta Chirurgica Belgica, 2022
Sotirios D. Moraitis, Apostolos C. Agrafiotis, Panagiotis Strempelas, Georgios Kagialaris, Pantelis Tsipas
Double coronary artery is a rare anomaly with just a few cases reported in the literature [1]. This anomaly started being reported recently (first case reported in 1994) with the wide use of coronary angiography. Before the advent of advanced imaging and catheterization facilities most of the available data came from the work of anatomists. A 42-year-old patient was recently operated in our department due to a severe bicuspid aortic valve stenosis. Preoperative coronary angiography showed two right coronary arteries (RCA). A 37-year-old patient was admitted for surgery because of aortic root and ascending aorta aneurysm, combined with bicuspid aortic valve regurgitation. In both cases, during surgery two ostia were encountered in the right coronary sinus. The aim of this small case series is to address the problem of classification and nomenclature while referring to the term ‘double RCA’ and underline the clinical implications of this anomaly.
Pre-implantation genetic testing for Marfan syndrome using mini-sequencing
Published in Journal of Obstetrics and Gynaecology, 2022
Sirivipa Piyamongkol, Krit Makonkawkeyoon, Vorasuk Shotelersuk, Opas Sreshthaputra, Tawiwan Pantasri, Rekwan Sittiwangkul, Theera Tongsong, Wirawit Piyamongkol
Marfan syndrome (MFS1, OMIM#154700) is the most common connective tissue disorder. MFS1 is inherited in an autosome dominant manner. Its incidence is about 2–3 in 10,000. It was first clinically described in 1896 (Marfan 1896). Major phenotypes include skeletal, ocular and cardiovascular system involvement. Anterior chest and vertebral column deformity, disproportionately tall stature, arachnodactyly and joint laxity are common skeletal manifestations. Ectopia lentis is a key ocular characteristic. Cardiovascular manifestations are the leading cause of the morbidity and mortality associated with Marfan syndrome. Aortopathy, i.e. aortic root dilatation, aortic regurgitation secondary from aortic dilatation, and fatal aortic dissection are the major causes. A family history of aortic dissection is the most important predicting factor for the risk of aortic dissection in affected offspring (Pyeritz 1993).
Contemporary Review of the Ross Procedure
Published in Structural Heart, 2021
Vincent Chauvette, Laurence Lefebvre, Marie-Ève Chamberland, Elbert E. Williams, Ismail El-Hamamsy
The aortic valve is often considered a passive structure, which opens and shuts in response to changes in trans-valvular pressures. Instead, the aortic valve, a component part of the aortic root, is a dynamic and living structure with many important functions which contribute to optimizing coronary flow reserve, reducing left ventricular workload during systole and ensuring perfect hemodynamics across the aortic root both at rest and with exercise.9 In addition, aortic valve cusps are covered by a monolayer of endothelial cells, which produce nitric oxide, thereby inhibiting platelet aggregation.10 Along with interstitial cells in the body of the cusps, they can also mount an inflammatory reaction in response to bacterial organisms in the bloodstream, limiting the risk of infective endocarditis in normal aortic valves.2,11,12 Finally, the cellular components allow the valve to repair itself and reorganize its extracellular matrix over a lifetime without wear (~2.5 billion cardiac cycles in a normal lifetime).12