In recent years, our department has treated a number of patients with severe perivalvular leaks after aortic valve replacement. This is a rare post-operative complication of aortic valve replacement, which can eventually lead to heart failure or even sudden death and necessitate reoperation. Shockingly, the ultrasound examination revealed that this patient’s valve had torn off about two-thirds of the valve, and only a small portion was attached to the annulus, and the valve was drifting up and down with the blood flow, which could lead to valve fly-off and sudden death at any time. This is actually caused by leukoaraiosis, it turns out that leukoaraiosis is an autoimmune disease, that is, our own immune system should play a defensive function to attack their own tissues, resulting in a number of systemic diseases, common recurrent mouth ulcers, iridocyclitis and genital ulcers, a small number of patients may occur cardiovascular system damage, the most common of which is aortic valve closure insufficiency and aortic aneurysms. Once these patients have undergone aortic valve replacement, perivalvular leakage occurs because of recurrent ulceration at the aortic annulus, which mostly occurs 3 to 6 months after surgery, and similar patients reported in the early stages of the disease died during the second or third operation. We were the first in China to systematically study the cardiovascular damage of leukoaraiosis, and we were the first in China to propose the causes and solutions for perivalvular leakage in patients with leukoaraiosis who underwent aortic valve replacement, and we were the first in China to perform successful aortic root replacement (Bentall procedure) in patients with perivalvular leakage after leukoaraiosis surgery. In young people with simple aortic regurgitation or combined aortic aneurysm, the possibility of leukoaraiosis should be considered after excluding wind heart or infective endocarditis, and leukoaraiosis should be considered if there are recurrent oral ulcers, iridocyclitis and genital ulcers, and should be diagnosed at the hospital. For mild to moderate aortic regurgitation, strict immunosuppressive therapy is recommended first to control the leukocoria activity, and the regurgitation may be reduced in some patients; if the regurgitation is severe and the valve must be replaced, the aortic root replacement should be performed after controlling the leukocoria first, and the valve should not be simply replaced, otherwise a perivalvular leak is likely to occur; In addition, immunosuppressive therapy should be continued postoperatively to avoid the formation of pseudoaneurysms due to leukocoria activity.