![]() ![]() Interruption of the aortic arch accounts for just over 1% of cases of so-called critical congenital cardiac disease. There is a reported seasonal incidence, with paucity of males born between April and August but without identification of an exogenous etiologic agent. Coarctation is also found with 22q11.2 deletion, supporting an association with abnormal migration of cells from the neural crest. It is now known that cells migrating from the neural crest populate the aortic arches, and a 22q11.2 deletion is well recognized as being associated with interruption between the left common carotid and subclavian arteries. Inheritance has also been reported as an autosomal dominant trait. The lesion was found in 10% of a large Danish series of patients with Turner syndrome, albeit with a lower incidence in patients with mosaicism or those with structural anomalies of the X chromosome. Coarctation is generally said to show multifactorial inheritance, although genetic factors are clearly important in certain groups. The overall incidence is in the region of 1 in 12,000, with a slightly increased occurrence in males. 45.2 ).Īortic coarctation accounts for 7% of liveborn children with congenitally malformed hearts, with a higher incidence in stillborn infants. 45.1 ), Celoria and Patton classified this lesion into alphabetic subtypes ( Fig. With regard to interruption of the aortic arch, which can be considered as the severest end of the spectrum of aortic coarctation (see Fig. The most careful dissection did not reveal either in the aorta or in its vicinity any cause to which this extraordinary condition could be attributed.” The part of the vessel which was above the constriction was slightly dilated the distal part was of normal calibre. Hence, in taking apart its walls, which had not decreased in this place, there remained only a small lumen. “The part of the aorta which is beyond the arch, between the arterial ligament and the first inferior intercostal, was so greatly narrowed that it had at most the thickness of a goosequill. According to Craigie, Monsieur Paris, Prosector of the Amphitheatre at the Hotel-Dieu, described, in the winter of 1789, the postmortem of “a very emaciated woman about 50 years old.” In addition to recognizing that the thoracic arteries were thicker and more tortuous than normal, he gave the following description. As pointed out by Craigie, a more recognizable description was published in Desault’s Journal de Chirurgie in 1791. At postmortem, she was found to have an aorta that was “so narrow that its diameter was smaller by half than that of the pulmonary artery, which it should have exceeded or at least have equaled in caliber.” Some argue, however, that it was Morgagni who should be given priority. The first description of aortic coarctation is generally attributed to Johann Freidrich Meckel, the Prussian anatomist, who presented the case of an 18-year-old female to the Royal Academy of Sciences of Berlin in 1750. In this respect, interruption can be interpreted as the extreme end of the spectrum of obstruction of the aorta ( Fig. In hemodynamic terms, it includes cases with a fibrous cord between the discontinuous segments. This involves discontinuity between two adjacent segments of the aortic arch. Within this chapter, however, interruption of the aortic arch is considered. Those distal to the thoracic aorta, together with acquired lesions, are beyond the scope of this chapter. These are considered, along with lesions of the aortic valve, in Chapter 45. ![]() Obstructive lesions can be found more proximally, involving the ascending aorta. When used in the context of the congenitally malformed heart, coarctation most usually described an area of narrowing of the thoracic aorta in the region of the insertion of the arterial duct, with or without additional abnormalities of the aortic arch. Accessed April 1, 2022.Coarctation derives from the Latin term coartatio, which translated literally means “a drawing together.” Aortic coarctation, therefore, indicates a narrowing at some point along the course of the aorta. Aortic valve sclerosis and pathogenesis of calcific aortic stenosis. Journal of the American College of Cardiology. 2020 ACC/AHA Guideline for the management of patients with valvular heart disease: A report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Aortic valve stenosis and pathogenesis of calcified aortic stenosis. Natural history, epidemiology, and prognosis of aortic stenosis.
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