The article discusses the problems of bicuspid aortic valve and associated valvulo-aortopathy, including the principles of separate classifications, mechanisms and risk factors of aortic dilatation formation, indications for surgical treatment and type of surgery. A clinical case of the patient with L-R variant of the 1st type of this defect is presented. The valvular pathology she was diagnosed at the age of 24 and aortic valve replacement operation was performed at the age of 34 due to its severe insufficiency. Before the operation, the diameter of the ascending aorta was 4,8 cm, and after 7 years, control echocardiography showed pronounced dilatation of the ascending aorta with signs of dissection. This confirms the opinion about different mechanisms of the formation of this defect and related aortopathy and proves that the latter is caused not only by hemodynamic factors, as it progresses significantly after isolated aortic valve replacement, requiring repeated surgical intervention. An important predictor of aortic diameter growth is pronounced aortic insufficiency at baseline, and the risk factors are hypertension and smoking in past.
The problem of timely diagnosis and proper management of patients with cystic fibrosis is crucial not only in our country, but throughout the world. Experts of the Union of Pediatricians of Russia have considered various issues of etiology, pathogenesis, epidemiology, diagnosis, and treatment of this genetic disease in a modern light. Particular attention was paid to screening methods for early diagnosis of cystic fibrosis. The principles of complex therapy were justified, including rational use of antibacterial and mucolytic drugs and enzyme replacement therapy that significantly determine the disease prognosis.
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