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RPC affects the cardiovascular system in 24% of patients, with resulting aortic and mitral valve regurgitation, aortic aneurysm, aortitis, aortic thrombosis, pericarditis, and myocardial infarction.
Patients with RPC aortitis exhibit inflammation in the media of any portion of the aorta, which is accompanied by loss of glycosaminoglycans and elastic tissue. Affected areas, in descending order of frequency, are the ascending aorta, aortic ring, descending thoracic portion, and abdominal aorta. The most common clinical manifestations are aortic arch syndrome, abdominal aortic aneurysm, and aortic regurgitation. Lesions may be multiple. Clinical presentation of aortic regurgitation (due to involvement of the ascending aorta) may include left ventricular failure, but abdominal aortic aneurysm is usually silent and may lead to rupture and death. Aortic regurgitation may be due to damage to the aortic cusps, or it may be due to annular dilatation caused by destruction of supporting tissues.
