For the physical finding “Short stature, webbed neck, low-set ears, and epicanthal folds” select the cardiovascular disorder with which it is most likely to be associated. (SELECT 1 DISORDER)
Correct Answer: Coarctation of the aorta
Description: Myocarditis, aortitis, and pericarditis all have been described in association with Reiter syndrome; the original description included conjunctivitis, urethritis, and arthralgias. Although its cause is unknown, Reiter syndrome is associated with HLA-B27 antigen, as are aortic regurgitation, pericarditis, and ankylosing spondylitis.Short stature, webbed neck, low-set ears, and epicanthal folds are the classic features of patients who have Turner syndrome. Persons affected by the syndrome, which is commonly linked with aortic coarctation, are genotypically XO. However, females and males have been described with normal sex chromosome constitutions (XX, XY) but with the phenotypic abnormalities of Turner syndrome. Additional cardiac lesions associated with Turner syndrome include septal defects, valvular stenosis, and anomalies of the great vessels.The Argyll Robertson pupil, a pupil that constricts with accommodation but not in response to light, is characteristic of central nervous system syphilis and is associated with vascular system manifestations of this disease. Treponema pallidum invades the vasa vasorum and causes an obliterative endarteritis and necrosis. The resulting aortitis gradually weakens the aortic wall and predisposes it to aneurysm formation. Once an aneurysm has formed, the prognosis is grave.Massive isolated tricuspid regurgitation produces a markedly elevated venous pressure, usually manifested by a severely engorged (often pulsating) liver. If the venous pressure is sufficiently elevated, exophthalmos may result. Tricuspid regurgitation of rheumatic origin is almost never an isolated lesion, and the major symptoms of patients who have rheumatic heart disease are usually attributable to concurrent left heart lesions. Bacterial endocarditis from intravenous drug abuse is becoming an increasingly important cause of isolated tricuspid regurgitation.A Quincke pulse, which consists of alternate flushing and paling of the skin or nail beds, is associated with aortic regurgitation. Other characteristic features of the peripheral pulse in aortic regurgitation include the water- hammer pulse (Corrigan pulse, caused by a rapid systolic upstroke) and pulsus bisferiens, which describes a double systolic hump in the pulse contour. The finding of a wide pulse pressure provides an additional diagnostic clue to aortic regurgitation.
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